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Neurology and Psychiatry - MUS
Neurology and Psychiatry - MUS
NEUROLOGY
&
PSYCHIATRY
OLGA GRIGOROVA
GEORGIONCHEV
vp Медицинско издателство
Ь дрсо
'tMo*
PSYCHIATRY
Autor of english edition: Georgi Onchev
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ISBN: 978-619-197-025-4
INTRODUCTION
C L I N IC A L N E U R O L O G Y ..................................................................................................................................................................................................... 65
и. d is e a s e s o f t h e p e r ip h e r a l n e r v o u s s y s t e m ..................!” Z ! Z Z Z ! ! ! ! Z Z Z Z 66
11.1. Neuritis o f nervus facialis (Facial nerve palsy, Bell’s palsy)......................................................................... *...........................................................66
11.2. Trigeminal neuralgia (Neuralgia N. Trigemini)...............................................Z!!!"""]”!!!"!"!!!!!!!™!!"!!!”!"!."”.'””Z ]" !.'Z Z Z Z Z !.Z Z .Z " .6 7
11.3. Glossopharyngeal neuralgia (Neuralgia N. Glossopharyngei)......................... ”!Z"!!!""!!!"""!!"!!”!" " Z Z Z !...............................................68
11.4. Occipital neuralgia and cervicogenic headache...................................................Z Z !" " !!!!!Z Z " !Z Z Z Z ........................................................69
11.5. Pain syndromes in the area of head, face, oral cavity and teeth................ Z Z Z Z " " Z " Z Z Z Z Z * ‘-........................................................69
11.5.1. Facial pain (Prosopalgia, Facialgta). Facial sympathalgia.............. Z Z Z .! ! ! Z Z Z Z .Z . ............................................................... 70
11.5.2. Pain in the oral cavity. ..................................................................... 72
11.6. Ganglionitis (Herper zoster) of the sensory ganglia...................... Z Z Z .Z Z Z .'" .7 Z Z .Z Z Z Z .....................................................................73
11.7. Cervical radiculopathy (Radiculopathia cervicalis)........... Z Z Z Z 7 7 7 Z 7 Z 7 Z 7 7 Z Z Z Z ................................................................ 73
118 Cervicobrachial plexitis/plexopathy (Plexitis/plexopathia cervicobrachialis)........................................................................................................74
11.9. Mononeuropathies of the upper lim b s..................................................................................................................... 76
Н Ю Lumbosacral radiculopathy (Radiculitis lumbosacralis) 7 Z Z 7 7 Z Z ! 7 Z Z Z Z ........................................................................................ 76
1111 Lumbosacral plexitis/plexopathy (Plexitis/plexopathia lumbosacralis) ................................................................................................................78
11.12 Mononeuropathies of the lower limbs...........................................................................................................................................................................78
11.13. Polyneuropathies.................................................................... •......................................................................................................................................... 79
12. n e u r o in f e c t io n s - m e n i n g i t i s a n d e n c e p h a l i t i s ........................................................................................................ 83
12.1 Meningitis..........................................................................................................................................................................................................................83
Contents / V
12 11 Viral meningitis gj
12 12 Bacterial meningitis
12 2 Encephalomyelitis
12.2 1 Poliomyelitis ( Poliomyelitis anterior acuta. Heine-Me Jin disease)
12.2 2 Rabies <Rabies. Lyssa)
12 2 3 ARBO-viralpanencephahtis
12.2.4. Acute disseminated encephalomyelitis (A DEM)
/2.2.5. Herpes simplex viral encephalitis ................
12.2 6 Herpes zoster viral encephalitis
12.2.7. Chronic, slow-viral and prionic infections o f the Nervous system ...............................................................................................................................90
12 3 Ncurosyphilis (Neurolucs).......................................
12 4 Ncuroborrcliosis (Lyme disease) 97
12.5. Mycotic (Fungal) Infections of CNS .................................................................................................................................................................... 98
12.6 Cerebral parasitosis
12.6.1. Brain echinococcus........................................................................................................................................................................................................................... 99
12.6.2. Cerebral cysticercosis .............................................................................................................................................................
12.6.3. Cerebral toxoplasmosis................................................................................................................................................................................................................../00
12.6 4 Tnchmelosis 0 / /
12.7. Cerebral abscess....................................................................................................................................................................................................... 101
13. D E M Y E L 1 N A T IV E D I S E A S E S ............................................................................................................................................................................ 103
13.1. Multiple sclerosis (M S)............................................................................................................................................................................................... 103
14. V A S C U L A R D IS E A S E S O F T H E N E R V O U S S Y S T E M ..........................................................................................................................UK,
14.1. Asymptomic failure of the brain circulation...........................................................................................................................................................108
14.2. Transient ischemic attacks..........................................................................................................................................................................................108
14.3. Ischemic stroke (Cerebral infarction)........................................................................................................................................................................109
14.4 Parenchymal brain hemorrhage (PBH)..................................................................................................................................................................... 112
14.5. Subarachnoid hemorrhage.................................................................................................................................................................................... 113
14.6. Thrombosis of the cerebral veins and dural sinuses.............................................................................................................................................. 115
14.7. Vascular diseases of the spinal cord...........................................................................................................................................................................117
15. T U M O R S O F T H E N E R V O U S S Y S T E M .........................................................................................................................................................119
15.1 Brain tumors ................................................................................................................................................................................................................ 119
15.2. Tumors of the spinal cord and spinal column..........................................................................................................................................................121
15.3. Idiopathic intracranial hypertension (IIH)............................................................................................................................................................... 122
16. T R A U M A S O F T H E N E R V O U S S Y S T E M ......................................................................................................................................................124
16.1. Cerebral concussion (Commotio cerebri)................................................................................................................................................................. 124
16.2. Cerebral contusion (Contusio cerebri).......................................................................................................................................................................124
16.3 Cranial fractures (Fracturae cranii)...........................................................................................................................................................................125
16.4. Early traumatic complications................................................................................................................................................................................... 125
16.4.1. Epidural hematoma ....................................................................................................................................
16.4.2. Subdural hematoma ......................................................................................................................................................................................................................... 126
16.4.3. Traumatic subarachnoid hemorrhage ......................................................................................................................................................................................126
16.4 4. TVaumatic intracerebral hematomas ........................................................................................................................................................................................ 126
16.5. Delayed traumatic complications..............................................................................................................................................................................127
16.6. Spinal cord traumas......................................................................................................................................................................................................127
17. E P I L E P S Y ..........................................................................................................................................................................................................................129
18. H E A D A C H E ......................................................................................................................................................................................................................135
18 1. Migraine.........................................................................................................................................................................................................................135
18.2. Cluster headache........................................................................................................................................................................................................ 137
18.3. Chronic paroxismal hcmicrania................................................................................................................................................................................. 137
18 4. Tension headache..........................................................................................................................................................................................................137
18.5. Chronic daily headache..............................................................................................................................................................................................138
18.6. Secondary headaches ................................................................................................................................................................................................ 138
19. D E G E N E R A T 1 V E D IS O R D E R S O F T H E N E R V O U S S Y S T E M ...................................................................................................139
19.1. Dementias...................................................................................................................................................................................................................... 139
19.1.1. A Izheimer s disease ......................................................................................................................................................................................................................... 140
19.1.2. Frontotemporal dementia ............................................................................................................................................................................................................. 141
19.1.3. Progressive supranuclear palsy ................................................................................................................................................................................................. 141
19.1.4. Corticobasal degeneration (CBD) .................................................................................................................................................
19 I 5. Disease with diffuse Lewy bodies..............................................................................................................................................
19.1.6. Multisystem atrophy (MSA)..........................................................................................................................................................................................................142
19.2 Parkinson’s disease ’42
19.3. Essential tremor .................................................................................................................................
PSYCHIATRY................................................ 163
22. INTRODUCTION IN PSYCHIATRY ... 165
165
Subject 165
Scope o f mental health problem s......................................................
Ethical framework............................................................................................
23. BRIEF HISTORY OF PSYCHIATRY.......................................................................................................................... 166
166
C ontent ...................................................................................................................................................................................................
VI / Neurology & Psychiatry
24. IN T E R V IE W A N D C L I N IC A L A S S E S S M E N T ....................................................................... 167
Interview; essence and r u les............................................... ............................................................................ 167
Good clinical interview has its ru les: ....................................................................................................................... 167
Structure of interview........................................................................................................................................ 168
Content and process........................................................................................................................................... 169
Other sources of information............................................................................................................................ 170
Interview in specific situations .................................................................................................................................... 170
Other assessment and summarization ...................................................................................................................... 170
25. G E N E R A L P S Y C H O P A T H O L O G Y ............................................................................................... 171
Basic characteristics........................................................................................................................................... 171
Assessment by domains..................................................................................................................................... .171
Assessment by syndromes................................................................................................................................. .171
Anxiety................................................................................................................................................................. .172
Depression............................................................................................................................................................ .172
Mania..................................................................................................................................................................... .173
Acute psychosis.................................................................................................................................................. .173
26. D IA G N O S IS A N D C L A S S IF IC A T IO N O F M E N T A L D I S O R D E R S .......................... 176
Essence and aims of psychiatric diagnosis and classification .................................................................. .176
Paradigms about mental disorder..................................................................................................................... .177
Mental and behavioral disorders in ICD-10, Chapter V .............................................................................. .177
Multiaxial assessment........................................................................................................................................ .178
Limitations of psychiatric diagnosis ............................................................................................................... .178
27. S C H I Z O P H R E N I A ..................................................................................................................................... 179
Epidemiology and significance........................................................................................................................ .179
Etiology and pathogenesis................................................................................................................................. .179
Clinical manifestation........................................................................................................................................ .179
Treatment.............................................................................................................................................................. .181
28 . A F F E C T IV E D I S O R D E R S .................................................................................................................. 181
Epidemiology and significance........................................................................................................................ .181
Etiology and pathogenesis................................................................................................................................. .181
Clinical manifestation......................................................................................................................................... .182
Treatment.............................................................................................................................................................. .183
29. D E L U S IO N A L D IS O R D E R ................................................................................................................. 184
A N D O T H E R P S Y C H O S E S ......................................................................................................................... 184
Delusional disorder............................................................................................................................................. .184
Schizoaffective disorder.................................................................................................................................... .184
Acute pasychoses ................................................................................................................................................................ .184
30 . A N X IE T Y D I S O R D E R S ......................................................................................................................... 185
Epidemiology and significance........................................................................................................................ .185
Etiology and pathogenesis................................................................................................................................. .185
Clinical manifestation......................................................................................................................................... .185
31. S O M A T O F O R M A N D D I S S O C I A T I V E D I S O R D E R S ...................................................... 186
Epidemology and significance ..................................................................................................................................... .186
Etiology and pathogenesis ............................................................................................................................................ .186
Treatment............................................................................................................................................................... .186
Clinical manifestation........................................................................................................................................ .187
Treatment.............................................................................................................................................................. .187
32. B E H A V IO U R A L S Y N D R O M E S A S S O C I A T E D W IT H P H Y S IC A L F A C T O R S .188
Eating disorders................................................................................................................................................... .188
Sex disorders........................................................................................................................................................ .189
Mental factors and dentistry............................................................................................................................ .189
33 . P E R S O N A L IT Y D I S O R D E R S ........................................................................................................... ,190
Epidemiology and significance......................................................................................................................... .190
Conceptualization of personality and its pathology..................................................................................... .190
Etiology and pathogenesis...................................................................................................................... ;......... .190
Clinical manifestation....................................................................................................................................... .190
Comorbidity and relationship personality —d ise a se ........................................................................................ .192
Treatment............................................................................................................................................................. .192
34. A L C O H O L A N D D R U G U S E D I S O R D E R S ............................................................................... 193
Epidemiology and significance......................................................................................................................... .193
Etiology and pathogenesis................................................................................................................................. .193
Clinical manifestation........................................................................................................................................ .193
Treatment............................................................................................................................................................. .195
35 . O R G A N IC M E N T A L D I S O R D E R S ................................................................................................. 195
Epidemiology and significance......................................................................................................................... .195
Etiology and pathogenesis ................................................................................................................................ .195
Clinical manifestation......................................................................................................................................... .196
Treatment............................................................................................................................................................. .196
36 . M E N T A L D I S O R D E R S IN C H IL D H O O D A N D A D O L E S C E N C E ........................... 197
Epidemiology and significance........................................................................................................................ .197
Etiology and pathognesis .................................................................................................................................. .197
Clinical manifestation....................................................................................................................................... .197
Treatment............................................................................................................................................................. .198
37. A G G R E S S IO N A N D A U T O -A G G R E S S I O N .............................................................................. 199
Aggression........................................................................................................................................................... .199
A uto-aggression ................................................................................................................................................................. .199
3 8 . T R E A T M E N T A N D R E H A B I L IT A T IO N O F M E N T A L D I S O R D E R S ................... 201
Basic principles ................................................................................................................................................................. . .201
Biological trefitment........................................................................................................................................... .201
Psychotherapy..................................................................................................................................................... .202
Psycho-social rehabilitation.............................................................................................................................. .203
39. G L O S S A R Y O F B A S IC T E R M S IN P S Y C H I A T R Y ............................................................ 204
R E C O M M E N D E D R E A D I N G S ................................................................................................................ 208
NEUROLOGY
OLGA GRIGOROVA
1. Reflexes / 7
1. REFLEXES
Reflex is involuntary stereotype motor response as muscle receptors (the so called muscle spindles) that is
a result of irritation of the receptors by external or in transmitted by the sensory fibers of the femoral nerve
ternal sensory stimuli. The reflex is the basic functional to L2, L3 and L4 segments of the spinal cord. Here the
unit of the nervous activity, as the neuron is the basic impulses reach the anterior horn cells and efferent im
structural unit of the nervous system. Reflexes are the pulses through the motor fibers of femoral nerve cause
simplest form of motor activity, which carry out the contraction of quadriceps femoris muscle and extension
simplest aspect of the adaptation of the organism. Their of the lower part of the leg.
mechanism is implemented also in more complex motor
and autonomous functions such as walking, maintain
ing posture, balance, breathing and heart function.
ange of the middle finger leads to quick palmar flexion 5. Reflexes of brainstem automatism. The tonic re
of the fingers. flexes (labyrinth cervical symmetric and cervical asym
• Troemner’s sign - tapping the distal phalange metric reflexes) are observed normally during the neona
of the middle finger evokes quick palmar flexion of the tal period of life. Later they take part in more complex
fingers. motor mechanisms for maintaining balance. In patholog
4. Reflexes of spinal automatism. These reflexesic conditions the following phenomena are observed:
occur 3-4 weeks after a complete transection of the spi • Phenomenon o f Magnus and Klein. In hemiplegic
nal cord at level above the lumbar enlargement. They patients the passive turning of the head laterally leads to
are result of liberation of archaic intersegment reflex increasing of the extensors’ muscle tone of the arm towards
es below the level of the injury because of interruption which the head is facing and increasing of the flexors’ mus
of regulatory and controlling pyramid and extrapyra cle tone of the other.
mid tracts. Obtaining these reflexes is connected with • Doll’s head phenomenon (oculocephalic reflex). In
patient’s poor prognosis for recovery. In response of comatose patients the passive movement of the head lateral
strong nociceptive irritation (pricking) or propriocep ly or up- and downwards is connected with passive turning
tive irritation (strong flexion of the toes) the paralyzed of the eyes in the opposite direction. The loss of conjugate
and unsensitive lower limb is flexing in the three joints eye and head movements in the horizontal plane is connect
- shortening response. Light touch of the proximal part ed with damage of pons. The loss of conjugate eye and head
of the thigh of preliminary flexed lower limb leads to movements in the vertical direction demonstrates midbrain
extension in the three joints - lengthening response. injury.
Successive irritation of both lower limbs produces au 6. Grasping reflex. The reflex was described by Yani-
tomatism of locomotion, resembling the act of walking shevski (1873-1936), founder of the University clinic of
in the paralyzed legs. The above mentioned reflexes are Neurology at the Medical University of Sofia. It can be ob
also known as defensive reflexes. It is a general consid served in patients with contralateral frontal lobe lesion and
eration that the shortening reflex is reaction of avoiding is connected with liberation of ancient subcortical mecha
the stimulus and lengthening reflex is reaction of repul nisms of grasping. The reflex consists of involuntary grasp
sion of the stimulus. ing of the object that touches the palmer surface of the hand.
Rarely grasping reflex of the foot can be observed.
2. Sensation / 13
2. SENSATION
In CNS continuous information for the changes in poorly localized slow and diffuse pain is carried out by
the external and internal environment of the body en the nonmyelinated C fibers. There are nociceptors in all
ters and assures adaptation and survival of the organ tissues - in the skin, mucous membranes, muscles, in
ism. Perception, transmission and processing of this ternal organs, blood vessels, meninges.
information are realized by the sensory functions of Proprioreceptors has low sensitive threshold and
the CNS. The receptors perceive the irritation. The sen perceive the senses of position, strength, direction,
sory pathways transmit the afferent information from speed and range of the motion. Such are muscle spindles
the receptors to the cerebral cortex. Processing of the (fig.1.3) sensitive to muscle stretching and tendon recep
sensory information is performed at different levels. In tors of Golgi (fig. 1.4) sensitive to strong stretching of
the spinal cord, brainstem and the subcortical structures the tendons. From these receptors the afferent nerve im
an unconscious analysis of the irritation is carried out pulses are transmitted by the thick myelinated Act and
which is the base of elementary unconditioned reflex AP fibers. Musculoskeletal system contains low thresh
es. In the cerebral cortex conscious analysis is realized old mechanoreceptors like corpuscles of Merkel, Ruffini
which determinates the more complex conditioned and and Vater-Pacini. The nerve impulses from these recep
conscious activity of the human. The most elementary tors are transmitted by the well myelinated Ap fibers.
conscious analysis of the qualitative and quantitative The interoreceptors are localized in the internal or
changes in the external and internal environment of gans and blood vessels. The afferent impulses are trans
the body is called feeling. On the base of feelings are mitted by unmyelinated or slightly myelinated nerve
formed perceptions and knowledge of the human - the fibers. The interceptive afferentation is most often un
so called gnosis. The perception of speech by hearing realized but it triggers important for the homeostasis
and vision is termed verbal-auditory and verbal-visual unconditioned spinal and brainstem reflexes. When the
gnosis. The perceived conscious and unconscious sen- internal irritation is stronger senses with pleasant or un
s.ory information determines the simple and more com pleasant emotional character arise.
plex motor reactions and the highest conscious motor 1. Specialized sensation: vision, hearing, smell,
activity in humans - the so called praxis. taste, equilibrium. The receptors are specialized in per
ceiving of a certain type of physical energy (light or
sound waves, soluble or gaseous chemical substances,
2.1. ANATOMY AND PHYSIOLOGY mechanic accelerations) and are localized in specialized
OF SENSITIVE FUNCTIONS organs - eyes, ears, nose, and tongue.
2. General (somatic) sensation:
Receptors perceive and transform certain type of a) Exteroreceptive (superficial, cutaneous) sensation
physical energy into nervous impulse. They are special - for rough, poorly localized sense (touch, pain and tem
ized nerve structures (organelles) or free nerve endings. perature differences)
According to their localization receptors are divided to b) Proprioreceptive (deep) sensation - for position
exteroreceptors (in the skin and mucous membranes), and motion (joint-position sense), pressure, weight, vi
proprioreceptors (in the muscles, tendons and joints) and brations;
interceptors (in the internal organs). Exteroreceptors c) Interoreceptive (autonomous) sensation
are divided to mechanoreceptors, termoreceptors and The afferentations of all somatic sensory and viscer
nociceptors. Mechanoreceptors has low threshold of al sensory receptors are conducted via afferent sensory
irritation and most often are capsulatcd: corpuscles of fibers which are the peripheral branches of the sensory
Meissner for touch, corpuscles of Ruffini for stretching, neurons in the cranial and spinal sensory ganglia. These
discs of Merkel for pressure, corpuscles of Vater-Pacini pseudounipolar neurons are the so called first stage
for vibrations, nerve plexuses around the hair follicles. sensory neurons. Their peripheral branches participate
The precise and finer tactile stimulations are conducted in the composition of the peripheral nerves, while the
by thick myelinated AJ3 fibers, whle the rough tactile central branches form the dorsal roots of the spinal and
stimuli arc carried out by slightly myelinated A5 fibers. cranial nerves and enter the spinal cord and brainstem.
Termoreceptors are free nerve endings, specialized in Here the fibers of the superficial and deep sensation form
perceiving of senses of hot, cold and temperature ditter- two separate ascending sensory pathways (systems):
ences. Pain receptors (nociceptors) has higher threshold 1. Anterolateral (extralemniscal) system. It is phy-
of irritation. They are free nerve endings, specialized logenetically older, conducts impulses with lower veloc
in perceiving of stronger mechanic, temperature and ity via poorly myelinated and unmyelinated fibers with
chemical stimuli. Well localized, fast and acute pain is more synaptic connections. It provides information of
conducted by the slightly myelinated A5 fibers, while mechanoreceptors, temperature and pain receptors and
14 / Neurology
□.occipitalismajor
□.occipitalisminor
n.auricularismagnus
nn.cervicales- ramipost,
□a.supracliviculares
□.axillaris
n.cutaneusbrachiilat.
n.cutaneusbrachiipost,
n.cutaneusbrachiimcd.
n.cutaneusantebrachiilat.
n.cutaneusantebrachiipost,
n.cutaneusantebrachiimed.
ramilumbalespost,
ramisacralespost,
n.radialis
nmedianus
n.ulnaris
n.cutaneusfemorislat.
n.obturatorius
n.cutaneusfemorisant.
n.cutaneusfemorispost,
n.cutaneussuraelat.
- - Iliohypogastric
n.peroneussuperficialis
n.saphenus
n.calcaneus
n.plantarislat.
n.plantarismed.
F ig .2.1. Peripheral nerve z o n e s and nerve root z o n e s - der- F ig .2 .2 . P eripheral n erve z o n e s and nerve root z o n e s - der
m atom es (ventral) (A m in o ff) m atom es (dorsal) (A m in o ff)
causes sensations that are diffuse, poorly lo bands (the nipples are situated at Th4-5 level; umbili
calized and with higher sensory threshold cal zone is situated at level ThlO). The dermatomes of
(protopathic sensation). the limbs are longitudinal bands (the lateral part of the
2. Posterior column (lemniscal) system. It upper limb and thumb are occupied by C5 and C6 der
is phylogenetically younger, transmits with matomes, while the medial part of the upper limb and V
higher speed via thick myelinated fibers using finger - by C8-Thl; L5 dermatome includes the lateral
fewer synapses. Provides information only of aspect of the lower limb and the medial part of the dor
mechanoreceptors and causes exact and pre sal surface of the foot and big toe; the dorsal aspect of
cise sensations (epicritical sensation). the lower limb, the lateral part of the dorsal surface of
The peripheral nerves (sensory-motor the foot and V toe are occupied by SI dermatome) - fig.
or sensory) innervate particular cutaneous 2.1 and fig. 2.2.
zones, typical for each nerve. Among the
innervation zones of adjacent nerves exists
some overlapping. The knowledge of these
peripheral nerve zones is important for rec
ognition of the injured peripheral nerve (fig.
2.1. and fig. 2.2.).
The thick myelinated Aa and A0 fibers of
the deep sensation in the posterior roots are
located medially while poorly myelinated A6
and nonmyelinated C fibers of the superfi
cial sensation are situated laterally - fig. 2.3.
Each posterior root enters into certain seg
ment of the spinal cord and innervates cer
tain cutaneous area, termed dermatome and
certain body area, named somatome. The F ig .2 .3 . Spinal cord segm en t w ith its dorsal root, g a n g lio n c e lls and sen so ry
dermatomes of the face are circular zones organs (W axm an)
around the nose and the mouth and are pro 1.P acinian corp u scle, 2. M u scle sp in d le, 3. G o lg i tendon organ, 4. E n cap su
lated en d in g, 5. Free nerve e n d in g s, 6. S p inal se n so ry g a n g lio n , 7. Lateral
jecting into certain segments of the sensory
d iv isio s (sm all fibers), 8. M ed ial d iv isio n (large fibers), 9. D orsal co lu m n ,
nucleus nucl. tractus spinalis n. trigemini. 10. sacral, 11. c er v ic a l, 12. S p in oth alam ic tract, 13. sacral, 14. C e rv ic a l, 15.
The dermatomes of the trunk are parallel C orticosp in al tract, 16. sacral, 17. c er v ic a l
2. Sensation / 15
It is typical that the cutaneous areas supplied by tic pathway gives pro
the peripheral nerves and roots overlap. The following jections towards the
cutaneous zones took shape: autonomous (innervated hypothalamus and the
only by certain peripheral nerve or dorsal root), mixed limbic system which
(innervated by neighboring nerves and roots) and addi have vegetative and
tional (innervated mainly by the neighboring peripheral emotional effects of
nerves and dorsal roots). When the peripheral nerve or the pain. The inter
dorsal root is damaged the sensory disturbances are most actions between the
pronounced in the autonomous area, less pronounced in interneurons, neuro
the mixed zone and are absent in the additional zone. peptides (endorphins)
During the embryonic stage of development the and descending inhib
spinal cord is shifted upwards and in adults is situat iting pathways from
ed at the level of L1-L2 vertebra. That’s why there is the brainstem in the
inconsistency between the numbers of vertebra, spinal dorsal horn ensure the
segments and dorsal roots - fig. 2.4. Because the dorsal control of ascending
roots pass through the relevant intervertebral openings, transmission of the
the lumbar and sacral roots (L2-S5) form the so called pain impulses - theory
cauda equina. of gate control.
3.Spinotectal tract
starts from laminae I
2.1.2. SUPERFICIAL SENSATION (FIG. and V as does neospi
2.5) nothalamic tract and
The fibers of the first sensory neuron in the spinal terminates in collic
cord terminate in the dorsal horn and via interneurones ulus superior of the
F ig .2 .4 . Schem atic illustration o f
reach the second stage sensory neurons. Their axons midbrain. The somat the relationships betw een the ver
travel 2-3 segments upward at the same side (ipsilater- ic sensory informa tebral colum n, the spinal cord and
ally) then cross the midline in comissura anterior alba tion integrates here spinal nerves.
and form anterior-laterally the spinothalamic tract. It with the visual and
consists of three parts:
• 1. Neospinothalamic tract starts from the transmis
sible neurons in lamina I and V in the dorsal horn. It
transmits by A5 fibers well localized and differentiated
sensation for pain and temperature and poorly localized som atic sensory
sense of touch and ends in thalamus (nucl. ventralis pos- cortex
terolateralis - VPL). The axons in the spinal cord are
thalam us
arranged somatotopically - laterally travel the fibers
from the lower limbs, medially travel the fibers from
the trunk and upper limbs. In the brainstem the spi prim ary
nothalamic tract has dorsal-lateral position. From VPL afferent tertiary afferent
als to the reticular formation (spinal reticular thalamic nerve. Evoked pain can be observed at stretching the
tract) involved in maintenance of wakefulness. The pain peripheral nerves - mode of Lassegue for sciatic nerve,
sensation is perceived at thalamic-cortical level —the mode of Wassermann for femoral nerve, mode of Bon
parietal sensory cortex is involved in localization, de net and Neri for lumbar spinal roots.
termination of the intensity of the stimulation and other The knowledge of the so called referred pain is also
discriminative aspects of the pain sensation. Collaterals important. This pain appears in diseases of internal
towards limbic system and hypothalamus are involved in organs but is located in certain cutaneous zones some
vegetative and emotional-behavioral aspects of the pain. times far from the diseased organ. For example cardiac
In addition to spinal regulation of pain also brainstem pain is located in the left shoulder and left arm. The ex
level of regulation exists performed by the descending planation of this phenomenon is convergence of the vis
serotoninergic and adrenergic pathways. These path ceral and somatic nociceptor afferentations in the same
ways start from the periaqueductal grey matter of the dorsal horn neurons and the common pathway of the so
midbrain and reticular formation. In the dorsal horns matic and visceral afferents in the spinothalamic tract.
and brainstem are discovered opioid receptors and en Pain is divided also to permanent and paroxysmal,
dogenous produced morphine-like substances - the so relapsing.
called (3-endorphins, encephalin and dinorphine. In dor Neuralgia is paroxysmal pain with shooting charac
sal horns these substances connect with the opioid re ter. It spreads in the zone of innervation of the periph
ceptors and block the releasing of substance P from the eral nerve or root and can easily be evoked by pressing
terminals of the primary pain afferents. It is believed or stretching. Typical example is trigeminal neuralgia,
that the analgesic effect of placebo and acupuncture re more seldom glossopharyngeal neuralgia, occipital neu
sults from the activation of the endogenous analgesic ralgia, intercostal neuralgia can be observed.
system and liberation of endorphins. Stress-induced an Allodynia is sensory disorder where pain is provoked
algesia is connected with activation of the descending by non-painful stimuli.
adrenergic system. Serotonin agonists perform their in Causalgia is chronic intensive not well localized
fluence upon chronic pain by activation of the descend heating pain which is provoked by partial damage of pe
ing serotoninergic system. ripheral nerves, rich with vegetative fibers like median,
Pain is divided into two types - somatic (animal) ulnar, sciatic and peroneal nerves. The pain is provoked
and vegetative (visceral). Somatic pain is observed in by mildest irritation and is accompanied by vasomo
diseases of the skin, muscles, bones, joints and con tor and trophic changes. The skin looks pale, cyanotic,
nective tissue structures. Vegetative pain is observed cold and wet and the affected limb - edematous. With
in diseases of internal organs, blood vessels, vegetative time osteoporosis develops. In these cases terms like
ganglia and plexuses. This pain is more diffuse, poorly posttraumatic bone atrophy of Zudeck, algodystrophy,
localized and has colic-like increasing character. algoneurodystrophy, reflex sympathetic dystrophy are
Pain can be divided also to spontaneous and evoked. used. It is believed that partial lesion of the nerve leads
Spontaneous pain is result of irritation of the nocicep to occurrence of false synaptic connection between so
tors and their afferent fibers and structures, especially matic sensory afferent fibers and sympathetic afferent
when peripheral nerves, sensory ganglia, dorsal roots and efferent fibers.
and thalamus are involved in the process. When the pain Phantom pain has character of causalgia and it feels
results of irritation of the sensory fibers of the peripheral in nonexistent amputated limb. It is connected with ir
nerves, it has shooting character and is called neuralgia ritation of the severed nerve by expansion of schwan
- for example trigeminal neuralgia. The pain connected cells (schwanoma) creating permanent excitatory focus
with damage of the dorsal roots is called radiculalgia, in sensory analyzer.
while those connected with plexus damage - plexalgia. Anaesthesia dolorosa is hyperpathic pain that oc
According to the location of pain is used terms like curs in situation of complete absence of sensation. This
cephalgia, dorsalgia, lumbalgia. According to the local type of pain appears in conditions with severe damage
ization of the pathologic process pain is divided to: of peripheral nerves.
Local pain - the localization of the pain matches to The therapy of pain includes etiologic treatment,
the location of the pathologic process; therapy with opioid and non-opioid analgesics, antide
Projection pain - pain projects distally from the pressants, antiepileptic drugs, NSAIs, different physical
place of the pathologic process, for example radicu and neurosurgical methods.
lalgia, trigeminal neuralgia. Projection tooth ache can
be result of trigeminal neuralgia;
Irradiating pain - pain irradiates from one sensory 2.5. SENSORY SYNDROM ES
branch of the nerve to the other or irradiates to the half
of the body when thalamus is engaged in the process; Sensory syndromes have different characteristics
In addition to the spontaneous pain, evoked pain according to the level of damage and the localization of
can also be observed. This pain is provoked by pressing the process in CNS and PNS.
certain points of Valleix, for example examination of Peripheral nerves, (fig.2.1. and 2.2). Because the
trigeminal nerve, occipital nerves, spinal roots, sciatic peripheral nerves are mixed their damage leads not only
2. Sensation / 19
to sensory but also to motor and autonomous symptoms. post-herpetic neuralgia.
In case of partial damage of the peripheral nerve only 4. Dorsal horns. (Fig.2.7. B) They are affected in
sensory symptoms (pain, paresthesias and hyperesthe diseases as syringomyelia, intramedullar tumors, he-
sia) in the territory supplied by the nerve can occur. matomyelia. As the spinothalamic tract starts from the
When the damage of the peripheral nerve is more se dorsal horn cells, the sense of pain and temperature is
vere hypoesthesia can be observed. When the peripheral disturbed but tactile and joint-position sense are pre
nerve is completely interrupted in its autonomous zone served because posterior funiculi are intact. The sen
there is anesthesia and in mixed zone hypoesthesia, par sory disturbances are of segmental, dermatome type
esthesias, pain, hyperpathia and disesthesia. The term and are typical for syringomyelia, that’s why they are
neuritis is used when the nerve is affected by inflam known as syringomyelitic dissociation of sensation.
matory process. The term neuropathy is used when the Most affected are the cervical and upper thoracic seg
nerve is affected by compression, ischemia, and trau ments and the sensory disturbances are in upper limbs
ma. Polyneuritis is connected with diffuse bilateral and trunk. The spinal nucleus of trigeminal nerve can
and symmetric damage of multiple peripheral nerves also be affected (syringobulbia) with sensory changes
by inflammatory process. Polyneuropathy occurs when involving the face.
the damage of the peripheral nerves is of non-inflam- 5. Spinal cord sensory pathways. Complete inter
matory nature (toxic, deficiency, metabolic). The distal ruption of the spinal cord (trauma, transverse myelitis,
type of sensory disorder is typical for polyneuritis and hematomyelia) leads to damage of all sensory pathways
polyneuropathy. The sensory symptoms increase in the with total loss of all sensory types distally of the level
distal parts of the limbs because at first and most are of lesion (conductive type of sensory disorder). In addi
affected the distal parts of the nerves as they are most tion there is paresis distally of the lesion and bowel and
distally situated from the trophic center - the cell nuclei bladder disturbances. In fact the lesion is 1-3 segments
(fig. 2.1.A). In some of the polyneuropathies superficial upper from the sensory conductive level as the fibers of
sensation is more affected. The predominant damage dorsal horns neurons travel 1-3 segments at the same
of the deep sensation in the other polyneuropathies is side before crossing the midline.
connected with sensory ataxia (syndrome of peripheral The lesion o f the spinothalamic tract leads to con
pseudotabes). tralateral anesthesia for pain and temperature with con
2. Dorsal roots. The most common reason for dor ductive level 1-3 segments lower than the level of lesion.
sal root damage is degenerative pathology of the spi The partial damage of the spinothalamic tract leads to
nal column vertebra, extramedullar tumors, etc. They pseudo segmental sensory disorder. The sensory disor
usually are connected with involvement of single dorsal ders connected with extramedullar pathologic processes
root - monoradicular syndrome. Less often generalized (tumors) initially involve laterally situated sensory fib
lesion of dorsal roots can be observed - meningoradic- ers, start from the sacral dermatomes and ascend. Just
ular syndrome in meningitis, acute or chronic demy- the opposite is true for the intramedullar processes - the
elinative polyradicular neuropathy. Radicular sensory sensory disorders start from the affected spinal seg
syndrome leads to dermatome type of sensory changes. ment and descend. The lesion of the spinothalamic tract
Dermatomes are cutaneous zones each innervated by a above medulla oblongata gives sensory syndrome with
single spinal ganglion, respective dorsal root and spinal contralateral face and body hypesthesia.
segment. Dermatomes differ from the peripheral nerves Lesion o f posterior funiculi (multiple sclerosis, sub
innervations zones and their recognition is important acute combined degeneration of the spinal cord con
for the topic diagnosis in Neurology - fig. 2.1 and fig. nected with vitamin B12 deficiency, Friedreich disease)
2.2. Dorsal root pain is strong, has shooting character disturbs proprioceptive senses for position, motion, pre
and is encircling in the area of trunk or irradiates as a cise tactile sense and complex sensation. Sensory ataxia
longitudinal bands in the limbs. It is accompanied by can be observed with ataxic gait especially in the dark.
paresthesia, hyperesthesia or hypoesthesia. Movements, The term tabetic gait is also used because in the past
spurt, cough or pressing the paravertebral pain points sensory ataxia was most often associated with tabes
increase dorsal root pain. Because of overlapping of the dorsalis. The syndrome of posterior funiculi is also as
dermatome zones, the damage of one dorsal root leads sociated with paresthesias and hyperpatia in response of
to hypoesthesia but not anesthesia. When the autono tactile stimulation because the protopathic sensation is
mous fibers of the dorsal roots are involved in the pro preserved. Lesions of the upper cervical spinal cord are
cess the pain projects to the internal organs (for example connected with proprioceptive disafferentation in the
visceral crisis in tabes dorsalis). arms and pseudo athetosis
3. Spinal ganglia. Most often they are affected with 6. Brainstem. Typical are alternating syndromes -
herpes zoster infection when ganglionitis develops in damage of cranial nerves at the side of the lesion and
one or more ganglia. Typical erythema and vesicular contralateral hemihypesthesia for all sensory types in
rush develops in the relevant dermatomes. It is accom cases of lesion of upper brainstem. When the lesion in
panied by strong pain, paresthesias, hyperesthesia at volves medulla oblongata dissociative hemihypesthesia
the beginning and later by hypoesthesia and hyperpa- is observed because the pathways of superficial and deep
thy. Pain can persist longer after the rush recovery - sensation travel separately here. Ischemic stroke involv
20 / Neurology
ing the dorsal lateral part of medulla oblongata (Wal 9. Cerebral cortex. Due to the large somatotopic
lenberg syndrome) is connected with facial hypesthesia presentation of the contralateral part of the body the
for pain and temperature at the side of the lesion (due to cerebral sensory syndromes are of monotype - most
damage of the spinal nucleus of trigeminal nerve) and often upper limb and face are involved as a result of
contralateral body hypesthesia for pain and temperature their larger cortical representations. Complex type of
(due to damage of the spinothalamic tract) Fig.2.7.D. sensation is most disturbed, while the reduction of pain
7. Thalamus. The thalamic syndrome o f Dejer- and temperature senses is mildest. The focal irritation
ine-Roussy results from damage of the ventral posterior of the sensory cortex leads to short lasting seizures with
lateral and ventral posterior medial nuclei of stroke or paresthesias at the contralateral part of the body or in
tumor. All types of sensation are disturbed at the con more limited areas - sensory Jackson (partial sensory
tralateral part of the body, especially proprioceptive, epileptic seizure).
precise tactile and complex senses. Due to disturbed 10. Psychogenic sensory disorders. These are func
sense for position the arm takes flexed at elbow posi tional disorders and organic structural lesion is absent.
tion (thalamic arm) and the fingers perform involun They can be observed in hysteric disorders and do not
tary choreoathetoid movements (dancing fingers). After correspond to any anatomic law and type of innerva
several weeks starts recovery of the sensation but ap tions. Often anesthesia in response of pricking is ob
pears thalamic pain - extremely strong causalgic pain served. Very typical are the sharp borders between the
that irradiates in the contralateral side of the body. The normal and disturbed sensation without transitional
pain can be spontaneous or can be provoked by minor zones of hypesthesia. Hypoesthesia goes exactly close
irritation. It also depends on the emotional state and is to the body midline. Anesthesia takes form of gloves,
accompanied by autonomous symptoms - palpitation, socks, and boots. They are often accompanied by other
erythema, sweating. The treatment of the thalamic pain functional disorders - hemiplegia, paraplegia, hysteric
is very difficult. blindness, deafness, hysteric seizures etc.
8. Internal capsule. It is involved most often in
ischemic stroke. Contralateral hemihypoesthesia and
hemiplegia can be observed.
5. MOTOR ACTIVITY
Neuromuscular motor activity performs the follow tract is divided into separate bundles between the nuclei
ing functions: 1) movements of the body and its parts in of pons and pontocerebellar pathway. The fibers of tr.
the space; 2) maintains body’s posture against the grav corticonuclearis are depleted at the level of pons and
ity; 3) maintains certain tension of the muscles - muscle medulla oblongata. In the ventral part of medulla ob
tone; 4) coordination of the movements. Motor activity longata the pyramidal tract forms longitudinal protru
is divided to somatic motor (performed by the striated sions called pyramids, where from comes the name of
muscles that are innervated by somatic motor fibers) and the whole system. On the border with the spinal cord the
visceral motor (realized by the smooth muscles of the greater part of the fibers (80%) cross the midline (decus-
internal organs that are innervated by autonomous mo satio pyramidum) and continue as crossed pyram idal
tor fibers). The somatic motor activity is a complicated pathway (tr. corticospinalis lateralis). This tract trav
complex of volitional realized motor activity performed els in the lateral column of the spinal cord and reaches
mainly be the pyramidal system and unrealized invol the sacral segments. Smaller part of the fibers which
untary motor activity performed by the extrapyramidal start from Betz’ cells terminate directly in the dorsal
system. lateral group neurons of the spinal cord anterior horns.
These fibers control the finger movements. The greater
part of the fibers of tr. corticospinalis lateralis ends in
5. 1. PYRAM IDAL SYSTEM the dorsal lateral group motor neurons via interneurons.
They innervate the distal muscles of the limbs, face and
Tractus corticomuscularis consists of two motor tongue. The crossed pyramidal tract is larger in size
neurons - upper (central) and lower (peripheral) motor than the uncrossed one. As it has long way from the
neuron. The central motor neuron forms the pyramidal
system that includes tr. corticospinalis and tr. corti-
conuclearis. The peripheral (lower) motor neuron that
starts from the anterior horns of the spinal cord and the
motor nuclei of the cranial nerves performs the direct
command towards muscles. It is called common final
pathway because the descending pyramidal and extrap
yramidal pathways and segment and intersegment affer-
ents converge to it.
The pyram idal pathway (fig.5.1) consists of the
axons of the central, upper motor neuron that is locat
ed in the Vth layer of the primary motor cortex (gyrus
precentralis) - field number 4 according to Brodmann.
In the motor cortex exists somatotopic representation
of the contralateral side of the body and the head (mo
tor homunculus which stays on its head). The largest
representations here have mouth, tongue and fingers. In
the motor cortex are located the giant pyramidal cells
of Betz which represent only 3-4% of the axons in the
pyramidal tract but are the best myelinated fibers and
conduct nerve impulses with greatest speed. The py
ramidal pathway contains also fibers coming from 6,h
field (premotor cortex and supplementary motor field)
and fibers which originate from the parietal somatosen
sory cortex (3rd, 2nd, 1st primary fields and the associa
tive 5,hand 7,h fields). The axons of the pyramidal tract
form corona radiata and pass through the capsula inter
na. Tr. corticonucJearis travels through the knee of the
internal capsule, while tr. corticospinalis occupies the
anterior 2/3 of the posterior limb of the internal capsule.
In midbrain the pyramidal tract passes through crus
cerebri - medially travel tr. corticonuclaris and laterally
- tr. corticospinalis. In the ventral pons the pyramidal
5. Motor activity 27
cortex to all levels of the spinal cord it is vulnerable to Anterior horns contain not only large a-motor neu
different pathologic processes. rons but also small y-motor neurons that innervate the
The uncrossed pyramidal pathway (tr. corticospi- intrafusal muscles of the muscle spindles (fig. 1.3). They
nalis ventralis) travels in the most medial part of the an register the extent of muscle stretching and participate
terior column of the spinal cord and depletes at the level in the regulation of the muscle tone. The degree of
of upper thoracic segments. It provides the head move y-motor neuron excitement determines the readiness for
ments. The pyramidal fibers terminate via interneurons muscle contraction.
in the ventral medial group of motor neurons and perform The damage of tr. corticomuscularis is connected
partial crossing at each level. They innervate the muscles with reduced muscle strength (paresis) or with complete
of the trunk and the proximal muscles of the limbs. absence of active movements - paralysis (plegia). The
Corticonuclear pathway (tr. corticonuclearis, tr. weakness of one limb is called - monoplegia, of body side
corticobulbaris) starts from the representation of the - hemiplegia, of upper or lower limbs - paraplegia (upper
head in the motor cortex, travels through the knee of or lower), of all four limbs - tetraplegia (quadriplegia).
the internal capsule and innervates the brainstem mo The damage of the upper motor neuron (pyramidal tract)
tor nuclei of the cranial nerves (with exception of the results in central paralisis and the damage of lower motor
oculomotor cranial nerves nuclei). The nuclei of V, IX, neuron - in peripheral paralysis. As the pyramidal tract
X and the upper part of the nucleus of VII cranial nerve controls and reduces the activity of the segmental reflex
receive bilateral cortical innervations as a result of par arcs, its damage liberates the segmental reflex activity of
tial crossing of the corticonuclear fibers. These nuclei the spinal cord and brainstem. As a result proprioceptive
are analogous to the ventral medial group of the spinal hyperreflexia and muscle hypertonia are observed. The
motor neurons. The lower part of the nucleus of the VII liberation of archaic motor reactions leads to occurrence
cranial nerve and the nucleus of the XII cranial nerve of pathologic reflexes. Areflexia and hypotonia are ob
receive only contralateral cortical innervations, similar served when the peripheral motor neuron is damaged, as
ly to the dorsal lateral spinal motor neurons - fig.5.2. it is an efferent arm of the segmental clonic and tonic re
The peripheral (lower) motor neuron generates flexes. Atrophy due to interruption of the efferent trophic
action potentials which via neuromuscular synapses impulses occurs in the denervated muscles (table 5.1).
induce muscle contraction. Each a-motor neuron in
nervates group of muscle fibers called motor unit. The T able 5.1. D ifferen ce betw een the central and peripheral pa
large motor units are connected with maintaining the ralysis
posture and the small with more precise movements. Central (spastic) Peripheral (flaccid)
Motor neurons innervating the striated muscles of the paresis paresis
head are situated in the motor nuclei of the cranial 1. Deep tendon reflexes 1. Deep tendon reflexes
nerves. Motor neurons innervating the muscles of the hyperreflexia and abdomi and cutaneous areflexia
body and limbs are situated in the anterior horns - ven nal areflexia
tral medial group innervates the proximal muscles and 2. Pathologic reflexes 2. Absence of pathologic
dorsal lateral group innervates the distal muscles. The reflexes
axons of the motor neurons leave the spinal cord with 3. Spastically increased 3. Muscle hypotonia
the ventral roots. Those who start from the cervical and muscle tone
lumbar enlargement regroup into cervical and lumbar 4. Absence of muscle at 4. Atrophy of the involved
plexus. From these plexuses start the peripheral nerves rophy muscles
that contain motor fibers from different spinal segments 5. Absence of EMG 5. Fasciculations and
and roots. Muscle groups innervating from certain spi changes EMG data for denervation
nal segment and ventral root are called myotom.
Active movements are examined by assessment of
their amplitude, volume, speed and muscle strength of
active motion as flexion-extension, abduction-adduction,
pronation-supination, rotation. Muscle strength is rat
ed as the patient performs particular movement against
resistance or with special devices - ergometers or di-
namometers. In doubt of mild paresis (latent paresis) test
of Mingazzini-Strumpell for upper and lower limbs and
test of Barre for lower limbs is performed. Muscle tone is
examined by palpation and passive movements.
caudatus). The nigrostriatal neurons are dopaminergic. 2. The ventral descending system consists of tr. cor-
The projections that end on D1 dopaminergic receptors ticospinalis ventralis, tr. vestibulospinalis lateralis and
excite the GABA-ergic neurons of the direct pathway. medialis, tr. reticulospinalis lateralis and medialis and
The projections that end on D2 dopaminergic receptors tr. tectospinalis. Most of this tracts travel ipsilaterally
inhibit the GABA-ergic neurons of the indirect path in the ventral bundle of the spinal cord, perform partial
way. The summary effect of the nigrostriatal system is crossing and end bilaterally in the ventromedial group
amplification of the cortical motor activity via the di motor neurons, which innervate the axial extensor mus
rect circuit and inhibition of the cortex via the indirect cles and proximal muscles of the limbs. The medial re
circuit. In Parkinson’s disease (fig. 5.4, fig. 5.5) due to ticulospinal tract starts from pons and terminates in
degeneration of the nigrostriatal neurons and reduction motor neurons that innervate the antigravity extensor
of the dopamine in striatum occurs imbalance between muscles and insure the upright position of the body. The
the direct and indirect pathway with activation of the lateral reticulospinal tract starts from medulla oblon
indirect pathway and inhibition of the cortical motor ac gata and ends in the dorsolateral motor neurons, which
tivity. The striatum contains also cholinergic interneu innervate the flexor muscle groups. The reticulospinal
rons that activate GABA-ergic striatal neurons of the pathways activate the spinal reflex arches and control
indirect circuit, thus inhibiting the thalamocortical pro the vegetative functions. The cerebral cortex and es
jections and cortical motor activity. In Parkinson’s dis pecially the premotor cortex send massive projections
ease the balance between the dopaminergic and cholin towards the reticular nuclei in the brainstem thus form
ergic system in striatum is disturbed with domination ing cortical-reticular-spinal pathways that ensure the
of the cholinergic system. The established biochemical starting position for the particular movements and mod
disturbances in Parkinson’s disease are the main reason ulate the activity of the muscles agonists and antago
for treatment with precursors of dopamine (L-Dopa), nists for smooth performance of the motion. The lateral
dopamine agonists and central cholinolitics. In extrapy- vestibulospinal tract stimulates the extensor antigravi
ramidal hyperkinesia the activity of the direct pathway ty muscles similarly to the medial reticulospinal tract.
is increased - fig. 5.5. The medial vestibulospinal tract ensures the conjugate
The basal ganglia haven’t direct projections to the movements of the head and eyes. The tectospinal tract
spinal motor neurons. They influence upon the ventral ensures the reflex synchronous turning of the head and
horn motor neurons and the motor nuclei of the cranial eyes towards new visual, auditory or tactile irritant.
nerves via two descending motor systems (lateral and The basal ganglia regulate and modulate the activity
medial) that are called common distal pathway. of the operational frontal cortex. They have significant
1. The lateral descending system consists of tr. cor- role in the movements’ control - planning, initiation and
ticospinalis lateralis and tr. rubrospinalis. They are performance of motions; selection of the most appropri
completely crossed, travel in the lateral bundle of the ate motor program; sequence of the different stages of
spinal cord and terminate in the dorsolateral group mo the motor program; regulation of the speed and ampli
tor neurons that innervate mainly the distal flexor mus tude of the motion; switching the different motor pro
cle groups. The pyramidal tract is important motor exit grams. The basal ganglia participate in associative and
for the basal ganglia. The rubrospinal tract is very well limbic functions - activation of the cognitive processes;
developed in some mammals like cats and dogs and is stabilization and maintenance of the routine skills; reg
underdeveloped in primates and human. ulation of the attention and of the emotional motivation-
А Б E
F ig -5 .5 . N eu roch em ical anatom y o f basal g a n g lia - 1) norm al, 2) p ark in son ism , 3) chorea
5. Motor activity 31
al processes. The limbic circuit ensures the motor and ly the distal parts of the limbs and face. The volitional
vegetative expression of the emotions. movements are disorganized of strained synergic mo
tions. They are accompanied by muscle hypotonia - hy
EXTRAPYRAMIDAL DISTURBANCES potonic-hyperkinetic syndrome which is typical for the
rheumatic chorea minor in children and for the chorea
The extrapyramidal disturbances are manifestited of Huntington.
with changes in muscle tone, reduced and delayed motor • Balism. These are clonic quick hyperkinesias
activity (hypokinesia, bradykinesia), involuntary move that involve the proximal parts of the limbs and lead to
ments (hyperkinesia), postural disturbances. The con sharp ticking. Most often only half of the body is affect
nection of certain extrapyramidal syndrome with par ed (hemibalism) in patients with stroke in the area of the
ticular extrapyramidal structure is extremely relatively. contrlateral subthalamic nucleus.
It is accepted currently that the motor disorders are • Athetosis. These hyperkinesias are slow ver
result of disturbed balance between the direct and indi miform twists of the distal parts of the limbs with im
rect motor circuit. Hypokinetic disorders are connected possibility for maintaining certain posture. The face is
with increased activity of the indirect pathway, while also affected of slow tonic grimaces. Athetosis can be
hyperkinetic disorders are result of increased activity of unilateral or bilateral. The muscle tone is changeable
the direct pathway. - spasmus mobilis. It results of birth trauma - children
/. Hypertonic-hypokinetic (Parkinsonian) syn cerebral palsy, Rh incompatibility. Sometimes a combi
drome. It is characterized by reduced motor activity - nation of chorea and athetosis can be observe - chore-
difficult inert initiation of the motion, slowness of the oathetosis.
movements (bradykinesia) and reduced amplitude of the • Dystonia. This disorder is characterized by
movements (oligokinesia). Gradually the usual automat continuous muscle contractions that lead to strange
ic movements disappear - the participation of the upper body postures. Most often cervical dystonia is observed
limbs movements while walking (physiological synki with involuntary rotation of the head - torticolis spasti
nesia), the face becomes expressionless, mask-like, the ca. Blepharospasm results of contractions of m. orbicu
speech is quite, monotonous, the gaze - staring. The laris oculi that at first have clonic character and lead
muscle tone is increased in all muscle groups, more in to frequent blinking and then become tonic and per
flexors, which explains the slightly flexed posture. The manent. Oromandibular dystonia presents with tonic
examination reveals muscle rigidity type “wax plastici- involuntary spasms of the lips, tongue and mandibula.
ty”or type “cogwheel”. The tremor at rest, known also The combination of blepharospasm and oromandibular
as static tremor, has 4-6 Hz frequency and usually starts dystonia is known as syndrome of Meige. Generalized
asymmetrically from the distal parts of the hand and body dystonia is called torsion dystonia. The action dys
can involve the chin, lips, tongue and legs. It can be ob tonia is connected with particular muscle activity - for
served in relaxed state and disappears with movement. example graphospasm. Drug induced dystonia and dys
Sometimes can be observed tremor while maintaining kinesia are connected with neuroleptic treatment (early
particular posture - postural tremor. In the advanced onset - up to the 48 h and late onset) and w ith long last
stages of Parkinson’s disease occurs postural instabili ing treatment with L-Dopa.
ty connected with reduced synergic movements, flexed • Myoclonias are sudden short lasting contrac
posture and development of motor block (freesing). tions of muscles or parts of muscles with or without mo
Very typical are the pulsed phenomena with tendency tor effect. They are divided to rhythmic and arrhythmic,
for falling forward (anteropulsio), backward (retropul- focal or diffuse and are often observed in combination
sio) and laterally (lateropulsio). with epilepsy (myoclonus epilepsy) or with cerebellar
2. Hyperkinetic disorders. They are divided to rhythsymptoms (dissynergia cerebellaris myoclonica).
mic (tremor) and arrhythmic (chorea, atetosis, balism, • Tics are quick stereotype contractions of mus
dystonia, tics, and myoclonus). Hyperkinesia of head, cle groups with synergic action. Most often they involve
face, mouth, tongue may seriously hamper the manipu the face, neck and shoulders. They are subdivided into
lations in the area of mouth. psychogenic and essential (multiple tic of Gilles de la
• The tremor at rest is typical for the Parkin Tourette).
son’s disease. The action tremor is divided to postur
al (when maintaining particular posture), kinetic (with
movement) and intentional (before reaching the target). 5.3. MUSCLE TONE
It results of neocerebellar lesions and its descending
pathways. Extremely strong, with flapping character Muscle tone is the permanent tension of the muscle
is the tremor in Hepatolenticular degeneration. An iso that exists at rest (static, postural tone) and in clonic
lated action tremor can be observed in Minor’s disease muscle contraction (kinetic, dynamic tone). The static
(essential tremor) that has familial character in the hall muscle tone depends primarily on non reflex mecha
of the cases. nisms as the elastic properties of the muscles, connec
• Chorea. The choreic hyperkinesias are arrhyth tive tissue, ligaments, joint capsules and joint friction.
mic quick scattered movements involving predominant It determines the muscle relief and fixes the posture.
32 / Neurology
The dynamic muscle tone depends on the change in the flex arc is under control of suprasegmental, supraspinal
muscle length and the speed of muscle stretching. It as mechanisms. 1) The cerebral cortex (primary motor,
sists and modulates the clonic muscle contraction. The premotor, supplementary motor cortex and postcentral
dynamic (kinetic) muscle tone is determined by the spi cortex) controls muscle tone by extrapyramidal efferent
nal myotatic reflex o f slow muscle stretching which is fibers that travel together with the pyramidal fibers and
activated by slow stretching of the muscle and leads to inhibit muscle tone via the reticular formation and indi
slow tonic muscle contraction. Thus the permanent con rectly via cerebellum and basal ganglia. 2) The brain
traction of the antigravity muscles insures the upright stem controls muscle tone via two systems: ventrome
posture of body and head. The muscle tone depends on dial (vestibulospinal tract, reticulospinal tracts and tec
the spinal reflex mechanisms and the influence of the tospinal tract) and dorsolateral (corticospinal tract and
descending tracts with inhibitory or excitatory function rubrospinal tract). The vestibulospinal tract starts from
(fig. 5.6). the lateral vestibular nucleus, excites the spinal motor
Spinal regulation of the muscle tone. The reflex arc neurons and increases muscle tone of the extensor anti
of the spinal stretch reflex consists of the anulospiral gravity muscles. The vestibulocerebellum inhibits this
receptor of the muscle spindles, afferent neurons of the pathway. The medial reticulospinal tract starts from the
spinal ganglia, the corresponding spinal a-motor neu reticular nuclei in pons and like the vestibulospinal tract
rons in ventral horns, the efferent nerve fibers and the increases the tone of antigravity muscles and ensures
effector muscle (fig.5.6). The stretching of the muscle the upright posture of body and head. The excitatory
lengthens the intrafusal fibers of the muscle spindles and pontine zone is under cortical inhibition and with re
excites the anulospiral receptor. The afferent impulses lease from cortical control the muscle tone is increased.
reach the corresponding a-motor neurons, they become The lateral reticulospinal tract starts from medulla ob
excited and cause muscle contraction. As a result the longata and terminates in spinal interneurons that in
stretching of the muscle spindles and the frequency of crease the tone in flexor muscles and inhibit the tone
the afferent impulses decrease, the excitement and the in extensor muscles. The inhibiting zone of medulla
impulses of a-motor neurons also decrease and the mus oblongata activates from the motor cortex, cerebellum
cle relaxes. This is the so called spinal feedback system and striatum. 3) Cerebellum hasn’t direct projections
for maintenance of the muscle tone. The same reflex arc to the motor neurons. It influences the muscle tone via
realizes the myotatic reflex of quick stretching of the vestibulocerebellum (inhibition) and neocerebellum
muscle with quick, clonic contraction which is demon (activation) by projections towards the vestibulospinal
strated with the examination of the proprioceptive re complex and reticular formation respectively. 4) Basal
flexes. ganglia also haven’t direct projections to the spinal cord
The muscle spindle (fig. 1.3) is mechanoreceptor and control muscle tone via motor cortex and corticos
that consists of capsule and several striated (intrafus pinal tract and via reticular formation. The muscle tone
al) muscle fibers. They are three types: dynamic, static is under the influence also of the excitatory bulbospi
and crawler. The dynamic fibers are the longest, around nal noradrenergic and serotoninergic raphe-spinal tract.
their central part is localized the anulospiral receptor The descending pathways have excitatory or inhibito
from which start the primary thick myelinated IA (Aa) ry effect upon the spinal reflex arc of muscle tone. The
fibers. These fibers react to the speed of the muscle summary effect o f the descending pathways is inhibitory
stretching, their afferentation interrupts with the inter and with their interruption the spinal and brainstem re
ruption of stretching and they define the dynamic
tone. The static fibers are innervated by the sec
ondary nerve endings (IB fibers). They register
the changes of the muscle length and their ac
tivity continues after interruption of stretching.
Gamma motor neurons innervate the endings of
the intrafusal fibers and define the sensitivity of
muscle spindles and the activity of the stretching
reflex. The increased activity of y-motor neurons
is connected with muscle tone increase.
The inhibitory interneurons of the spinal cord
control the activity of motor neurons via: presyn-
aptic inhibition of IA and IB afferent fibers before
they synapse with the motor neurons (by GABA);
postsynaptic inhibition that is divided to reflexive
(by Renshow cells with mediator glycine), auto
genic (by proprioreceptors of Golgi) and recipro
cal (towards the antagonist muscles).
Supraspinal regulation of the muscle tone.
The muscle tone generated by the segmental re
5. Motor activity 33
flex activity and the muscle tone are increased. of the 4 limbs. Extensor spasticity is observed, which in
the past is not precisely termed decerebration rigidity.
Examination of the muscle tone. The lesions below the vestibular nuclei release cer
The patient must be completely relaxed, without vical and labyrinth tonic reflexes that leads to redistri
making any motions. The static muscle tone is exam bution of the muscle tone in flexor and extensor muscles
ined by observation and palpation of the muscles and the in response of change in the position of body and head.
dynamic tone is examined by performing slow passive The spinal cord lesion leads to spasticity in the flex
movements of the joints. The examination starts from ors below the level of damage because the reticulospinal
the neck and proceeds by symmetrical examination of tract is interrupted. When the damage is incomplete the
upper and lower limbs. The disorders of the muscle tone spasticity can be observed only in upright position. The
occur when the spinal and supraspinal regulation mech spinal spasticity is the most severe one. With time occur
anisms are disturbed. muscle fibrosis and changes in tendons, joints and liga
ments with forming of contractures.
Muscle hypotonia. 2) The muscle rigidity is plastically increased resist
The volume of the passive movements is increased; ance of the muscle, which is permanent during the en
the normally persisting lines between the muscle groups tire muscle stretching and can be observed not only with
disappear. The joints are loose, the lifted and left with movement but also in rest. The muscle rigidity is typical
out support limbs drop down. The muscle hypotonia is for the Parkinson’s disease that is connected with de
result of interruption of the reflex arc of the tonic spinal generation of substantia nigra but can also be observed
reflex in its afferent, central or efferent part - for exam in other extrapyramidal disorders. Cogwheel type mus
ple in mononeuropathy and polyneuropathy, lesions of cle rigidity (Negro phenomenon) can be observed not
the ventral and dorsal roots, spinal cord lesions. Muscle only in Parkinson’s disease but in Minor’s disease also
hypotonia can be observed in cerebellar damage (com (Essential tremor).
bined with ataxia) and in neostriatal lesion (combined The muscle rigidity could be localized, for example
with hyperkinesias). Generalized hypotonia occurs in increase in muscle tone of neighboring muscles con
the acute stage of CNS damage (cerebral and spinal nected with pain and inflammatory conditions or could
shock). be generalized - in pyramidal and extrapyramidal le
sions. The increase in tone of masticatory muscles (tris
Muscle hypertonia. mus) can be associated with inflammatory processes of
• When the tone is increased there is certain muscle the oral cavity and teeth and w'ith meningitis.
resistance that could be equal during the whole passive Meningo-radicular rigidity results of irritation of
movement (“lead pipe” muscle rigidity; waxy plasticity) meninges and of the ventral and dorsal roots passing
or there could be periodic interruptions of the passive through them. It can be observed in meningitis, suba
movement (“cogwheel” muscle rigidity). The phenom rachnoid hemorrhage, neoplastic infiltration of menin
enon of “clasping knife” or spastic muscle hypertonia ges, irritation after drug and other substances applica
is observed when the resistance at the beginning of the tion. It is connected with increased activity of the seg
passive movement is strong and then is suddenly elim mental reflex arc. Under examination is revealed neck
inated. rigidity and positive Kernig and Brudzinski signs.
I) The spastic muscle hypertonia results of increase
of kinetic muscle tone. It is typical for the central mo
tor neuron damage and is accompanied by brisk deep 5.4. CEREBELLUM AND COORDINA
tendon reflexes and occurrence of pathologic reflexes. It TION OF MOVEMENTS
can be observed after the cerebral and/or spinal shock
overcoming and after plastic reorganization of the seg Cerebellum ensures coordination of movements
mental reflexes. It is associated with pyramidal tract by controlling and correcting the speed, direction and
damage and more precisely with lesion of the extrap- duration of the motor act and its components - mus
yramidal fibers that travel together with the pyramidal cle strength, muscle tone, maintenance of equilibrium.
tract. The clinical characteristics of the spasticity de It receives preliminary information from the cortex for
pend on the level of damage. the plan of the movement and for the consecutive stages
In decortication the lesion is above nucl. ruber. Thus for its performance. It compares and corrects the plan of
the nucleus is released from cortical control and via the the movement according to the wealth of afferent infor
rubrospinal tract the muscle tone is increased in the mation from the vestibular system and somatosensory
flexors of upper limbs and extensors of lower limbs. The information for the position of the body and limbs and
so called posture type Wernicke-Mann typical for inter performs the motor act (feedback principle) (fig. 5.7).
nal capsule damage in stroke is observed. Macroscopic structure. Cerebellum consists of
In decerebration the level of the damage is lower but transverse folds (folia) and striations (fissure). The deep
above the vestibular nuclei and releases them from cor er striations divide it into three lobes - lobulus anterior
tical control. As a result the lateral vestibulospinal tract (paleocerebellum), lobulus posterior (neocerebellum)
is activated and increases the muscle tone in extensors and lobulus flocculonodularis (archicerebellum). The
34 / Neurology
S p in o cereb ellu m
Ventral descending
system Motor
Lateral descending performance
system
Equilibrium,
synchronous move
Vestibular nuclei ment of the eyes
and head
6. CRANIAL NERVES
The cranial nerves are (fig. 6.1): I cranial nerve (n. the ganglion cells form the optic nerve. It enters into
olphactorius), 11 cranial nerve (n. opticus), III cranial the skull through foramen opticum. In the area of sella
nerve (n. oculomotorius), IV cranial nerve (n. trochle- turcica the visual fibers partially cross (chiasma opti
aris), V cranial nerve (n. trigeminus), VI cranial nerve cum). The fibers that are coming from the lateral halves
(n. abducens), VII cranial nerve (n. facialis), VIII crani of the retina do not cross. Only the fibers coming from
al nerve ( n. statoacusticus), IX cranial nerve (n. glos- the medial halves of the retina are crossed. Both op
sopharyngeus), X cranial nerve (n. vagus), XI cranial tic tracts start from chiasma opticum. Each of them
nerve (n. accessorius), XII cranial nerve (n. hypoglos- receives fibers from homonymous halves of the retina
sus). They have 5 types of nuclei in the brainstem that - the left tract receives fibers from the left halves of
are situated from medially to laterally as follows (fig. both retinas and perceives information from the right
6.2): 1) Somatic efferent nuclei for innervations of the halves of the visual fields. The third neuron ends in the
striated muscles (the motor nuclei of the III, IV, V, thalamic nucleus corpus geniculatum laterale. From this
VI, VII, IX, X, XI and XII cranial nerves); 2) Viscer nucleus starts the IV neuron. It forms tr. geniculo-cal-
al efferent nuclei for parasympathetic innervations of carinus (radiation optica) that terminates in the primary
the smooth muscles and glands (the nucleus of Eding- visual cortex around sulcus calcarinus in the medial oc
er-Westphal, nucl.dorsalis n.vagi, nucl. salivatorius sup. cipital cortex (area 17). The primary visual cortex has
et inf.); 3) Visceral afferent nucleus (nucl. tr. solitarii); associative connections with the secondary visual fields
4) Somatic afferent nuclei (nucl. tr. spinalis n. trigemini, (areas 18 and 19). Macula lutea that is responsible for
nucl. principalis n. trigemini); 5) Specialized afferent the maximal visual acuity has projections to both oc
nuclei (auditory and vestibular nuclei). cipital lobes.
Examination and syndromes of damage. 1) The
visual acuity is examined separately for each eye with
6.1. N. OPTICUS (OPTIC NERVE), II special tables. The decreased visual acuity is called am
CRANIAL NERVE blyopia and complete loss of vision is called amaurosis.
2) The visual fields are examined by perimeter or by
The first three neurons of the visual afferent system computed perimetry. In presence of optic neuritis is ob
are located in retina - photoreceptor cells (rods and served central scotoma (scotoma centralis). The lesions
cones), bipolar cells and ganglion cells. The axons of of chiasma opticum lead to heteronymous hemianop
sia (hemianopsia heteronima)
- fig. 6.3. When the lesion in
volves the crossed fibers of the
Hypophysis
chiasma (pituitary adenoma)
Mammillary bodies
n.olfactorius the medial parts of the retina,
n.opticus respectively the lateral parts of
tractus opticus n. oculomotorius visual fields drop out (bitem
n.trochlearis
poral hemianopsia). The dam
age of the lateral (not crossed)
Midbrain crus fibers of the chiasma are con
cerebri
nected with binasal hemianop
sia which is rare condition. The
gangl.semilunare lesion of retrochiasmal tracts
lead to contralateral homon
Trigeminal ymous hemianopsia - left or
motor root right (hemianopsia homonima
n.glossopbaryngeus
dextra et sinistra). In lesions
cerebellum above corpus geniculatum lat
n.vagus
n.accessorius erale the macular vision and
medulla oblongata
n.hypoglossus pupilary reaction of light are
Accessory nerve - preserved. The excitatory pro
spinal root cesses involving area 17 cause
perception of lucent points and
sparks (photopsia), while those
Fig.6 . 1. Cranial nerves.
6. Cranial nerves 39
involving areas 18 and 19 lead to visual hallucinations. sympathetic fibers that innervate m. sphincter papillae
Bilateral damage of the visual cortex is connected with and m. ciliaris.
cortical blindness (amaurosis corticalis). 3) The color Reflex o f light - when light falls in one of the eyes
vision is examined by pseudoisochromatic tables and the pupils of both eyes are constricting. The reflex arc
color tests. The disturbances of color vision are known includes: retina, n. opticus, tr. opticus, mesencephalon
as dyschromatopsia. Total absence of color vision is (nucl. pretectalis) where via interneuron is performed
called achromatopsia. 4) The ocular fundus is assessed bilateral connection with the parasympathetic nuclei
by ophthalmoscope in terms of papilla of the optic of Edinger-Westphal. The efferent arm of the reflex arc
nerve, retina, and blood vessels. Papilloedema is
observed in patients with increased intracranial
pressure (cerebral tumor, etc).
starts from these nuclei and in the composition of the Bernard-Horner syndrome).
III cranial nerve reaches m. sphincter pupilae - fig. 6.4. Supranuclear centers of gaze (fig.6.6).The ocular
Reflex o f accommodation — when near object is movements are divided to version (parallel) and ver-
focused occurs contraction of m. ciliaris, the lens be gence (convergent-divergent). The version eye move
comes more spherical, the eye accommodates for close ments are quick (saccadic) and slow (smooth pursuit).
watching, the pupil constricts and the eyeballs converge The frontal center o f gaze is situated in area 8 and to
(reaction o f convergence). The reflex arc is similar to gether with the adjacent area 6 turns volitionally the
those of light reflex but in the efferent arm participate gaze and head contralaterally via the descending tracts
corpus geniculatum laterale and the occipital visual cor that reach the pontine center. The pontine center is
tex (area 17). neighboring in pons with the nucleus of n. abducens.
Sympathetic innervation o f the eye (fig. 6.5). The The pontine center deviates the eyes horizontally on the
sympathetic eye center (centrum ciliospinale) is situat same side. The mesencephalic center o f gaze deviates
ed in the lateral horns of the spinal cord (C8-Th2 seg the eyes in the vertical plane. The smooth-pursuit eye
ments). The preganglionic fibers start from this center, movements are predominantly under the occipital cor
travel in truncus sympaticus and end in ganglion cervi tex control.
cal superius. The postganglionic fibers reach m. dilata Exam ination and syndrom es of damage. The width
tors papillae (dilates the pupil) and m. tarsalis superior of the ocular slits and the position of the eyeballs in or
(lifts the upper eyelid and widens the ocular slit) via bits is assessed by observation. The examination may
plexus caroticus internus. The damage of the sympa reveal drooping of the upper eyelid (ptosis), exophthal
thetic fibers on their long way leads to miosis, ptosis mos (protrusion of the eyeball), enophthalmos (sinking
and subjective impression of enophthalmos (Claude of the eyeball) and strabismus. The examination of pu
pils may reveal difference in their width (anisocoria),
narrow pupil (miosis) or wide pupil (mydriasis). The
examination of the active ocular movements in all di
rections is looking to detect double vision (diplopia) and
delay in movement of one of the eyeballs. The reflex of
light is examined for each eye separately by consecutive
lighting of the pupils. The reaction of accommodation
is examined separately for each eye by quick directing
the sight from distant to a close object. The reaction of
convergence is assessed by slowly approaching of the
fixed object. The syndrome of Argyll Robertson is typ
ical for lues of the nervous system and is characterized
by absence of reflex of light and preserved reflexes of
accommodation and convergence.
The damage of n. oculomotorius is connected with
ptosis, lateral deviation of the eyeball (divergent strabis
mus), reduced or absent mobility of the eyeball upward,
medially and downward (external oculomotor ophthal-
moparesis). Internal oculomotor ophthalmoparesis is
observed when only the parasympathetic fibers are in
volved - wide and motionless pupil. Most often total oc
ulomotor ophthalmoparesis is observed due to paresis of
both external and internal ocular muscles. When the III
cranial nerve is damaged in midbrain - ipsilateral lesion
of the nerve and contralateral hemiparesis due to py
ramidal tract involvement is observed (alternating syn
drome of Weber). The pathologic processes in the area
of cavernous sinus and fissure orbitalis superior lead
to damage of all three oculomotor nerves - complete
ophthalmoparesis with facial pain due to involvement of
the I and II branches of trigeminal nerve, exophtalmos,
edema of the eyelids, conjunctival inection.
The lesion of trochlear nerve leads to diplopia when
viewing down and involuntary head position.
The lesions of n. abducens are frequently observed
especially in cases with increased intracranial pressure
due to the long way of the nerve along the cranium ba
Fig.6 .5 . S ym p athetic inervation o f the eye. (no A m in o ff) sis. Convergent strabismus and diplopia are observed.
6. Cranial nerves 41
pressure on chewing. M. pterygoideus lateralis has up
per and lower head. Its bilateral contraction moves man
dibula forward, while the unilateral contraction moves
mandibula to the opposite side. M. mylohyoideus and
the anterior division of m. digastricus are attached to
the sublingual bone and are innervated by n. trigemi
nus. These muscles open the mouth and participate in
chewing. The masticatory muscles in human unlike oth
er mammals are able to perform different mandibular
movements - upward-downward, left-right, ahead-back
and rotator.
The masticatory muscles are innervated by the motor
fibers of n. trigeminus. Its motor nucleus (nucl. motorius
n. trigemini) is placed in pons medially from the prin
cipal sensory nucleus of the nerve. Close to the upper
pole of the motor nucleus is situated nucl. supratrigem-
inalis responsible for the rhythm of mastication. The
motor branch of the nerve exits pons as portio minor,
Fig.6.6. Supranuclear centers of gase. (no Aminoff)
passes close to the sensory ganglion Gasseri, enters
In cases of pontine pathologic processes the nucleus and into the composition of n. mandibularis and emerges of
the horizontal gaze center are involved and inability of the cranium through foramen ovale. N. masticatorius is
eyeball deviation to the side of the lesion is present. The mixed nerve and innervates m. masseter, m. temporalis,
alternating syndrome of Foville is characterized by ipsi- m. pterygoideus lateralis and medialis. N. mylohyoide
lateral lesion of VI and VII cranial nerves and contralat us that is branch of n. alveolaris inferior innervates m.
eral hemiparesis. mylohyoideus and m. digastricus anterior. The motor
The damage of fasciculus longitudinalis superior nucleus receives proprioceptive afferentations from the
that connects the nuclei of the oculomotor nerves and mesencephalic sensory nucleus (nucl. mesencephalicus
the nucleus of the vestibular nerve leads to development n. trigemini) and so the monosynaptic reflex arc of the
of internuclear ophthalmoparesis - disturbed medi mandibular reflex is build. The motor nucleus receives
al deviation of the ipsilateral eye and nistagmus of the bilateral cortical innervation by tr. corticonuclearis as a
contralateral eye. result of partial supranuclear crossing of its fibers. The
volitional control of the chewing is performed by the
premotor cortex placed closely to the facial representa
6.3. N. TRIGEMINUS (TRIGEMINAL tion in the motor cortex.
NERVE), V CRANIAL NERVE Examination and syndromes of damage. By obser-
rons of the auditory sensation. Most of their axons are tion of stapedial muscle in response of loud sound).
crossed and form corpus trapezoideum in pons. There Indicatively the acoustic acuity is examined by per
after the auditory fibers give rise to lemniscus lateralis ception of whisper (from 5-6 m distance) and normal
and reach the primary auditory center in mesenceph speech (from 15-20 m distance).
alon - colliculi inferiors and corpus geniculatum me D isturbances of hearing. The perception of noise
diate in thalamus. Before joining lemniscus lateralis (tinnitus) most often has vascular etiology in cas
some of the fibers terminate in the additional auditory es of damage of the internal ear and acoustic nerve.
nuclei. They relate to localization of the sound in the The facial neuritis is connected with hyperacusis due
space and orientation of the head and eyes to the sound to paresis of m. stapedius. The processes that cause
source. The partial crossing of the auditory fibers in excitation of the auditory cortex lead to auditory hal
the brainstem ensures bilateral auditory afferentation lucinations. The decrease of hearing (hypacusis) and
to the primary auditory centers and cortex. The pri total hearing loss (anacusis) can be of sound-conduct
mary auditory centers realize the reflex of turning of ing or sound-receiving origin. The age-related hearing
the eyes and head towards the source of the sound. The decrease (presbiacusis) is connected most often with
thalamocortical pathway (acoustic radiation) starts middle ear pathology (otosclerosis). In damage of or
from corpus geniculatum mediate, passes through the gan of Corti is observed loud noise, recruitment phe
internal capsule and terminates in gyrus temporalis nomenon and sound-receiving hearing decrease main
superior - area 41. Adjacent is the associative auditory ly for the high frequencies. The damage of the acustic
cortex - areas 22 and 42 (area of Wernicke) that in the nerve leads to sound-receiving hearing impairment
left hemisphere is associated with speech understand and tinnitus. In cases of the acustic pathways and audi
ing. The connections of the auditory nuclei with the tory cortex impairment is observed mild bilateral and
reticular formation ensure the so-called start-reflex slightly more pronounced contralaterally decrease of
(motor reaction to strong sound) and those with nuclei hearing due to the fact that most of the acoustic fibers
of facial and trigeminal nerve - the contractions of m. are crossed. The syndrome of sudden deafness is ob
stapedius and m. tensor tympani that protect cochlea served in cases of impaired blood flow in a. labyrinthi
from acoustic trauma. - branch of a. cerebelli inferior anterior.
Exam ination of hearing. The acoustic acuity
is examined by subjective tonal threshold audiome VESTIBULAR NERVE. The receptor cells are
try by defining the aerial and bone conduction. The situated in the membranous Iabirynth - in the otolithic
sound-conducting auditory disorder affects the aerial organ (utriculus and sacculus) and in the three sem
conduction curve and sound-receiving auditory disor icircular canals. The cilia of the receptor cells in the
der (damage of organ of Corti, acoustic nerve, acous otolithic organ are submerged in a gelatinous mem
tic pathways) both curves are simultaneously lowered. brane containing otoliths (tiny crystals of calcium car
By tonal above-threshold audiometry is diagnosed the bonate) and those of the semicircular canals - in the
recruitment phenomenon - improper rapid increase gelatinous membrane of cupula. The endolymphatic
of the sound strength in the diseased ear. The objec movements excite the receptor cells. The linear ac
tive audiometry includes examination of the auditory celerations that change the position of body and head
evoked potentials and stapedial reflex (reflex contrac against the gravitation are perceived by the otolithic
the affected side. 2. The peripheral, root vestibular ulotemporalis (branch of the trigeminal nerve) reach
syndrome - acoustic nerve is also involved (tinnitus, gl. parotis.
diminished hearing). In processes in the ponto-cere- The specialized sensory taste fib e rs are peripheral
bellar angle facial nerve is involved. 3. Central ves branches of the neurons of ganglion inferior (petro-
tibular syndrome. It is observed in cases of damage of sum). They innervate the posterior 1/3 of the tongue.
vestibular nuclei and their connections in disturbed The central branches of ganglion inferior neurons ter
blood supply of vertebral-basilar system, multiple minate in nucl. tr. solitarii - common gustatory nu
sclerosis, traumas. Spontaneous nystagmus, bilateral cleus of VII, IX and X cranial nerves.
ataxia and dizziness occur, the hearing is preserved, The visceral sensory fib ers for innervation of the
vegetative symptoms are absent, and there are other pharynx, posterior 1/3 of the tongue, tonsils, soft pal
symptoms of CNS engagement. The cortical lesions ate, palate arches,. Eustachian tube, and part of the
lead to perception of spacial deformation and the op internal ear are peripheral branches of the neurons of
tokinetic nystagmus is lost. The vertigo crises can be ganglion inferius. Their central branches terminate
a manifestation of simple partial epileptic seizure. in nucl. tr. solitariia and nucl. tr. spinalis n. trigem
ini. These fibers form ramus sinus caroticus togeth
er with the fibers of n. vagus and sympathetic fibers
6.6. N. GLOSSOPHARYNGEUS (GLOS from ganglion cervical superior. Ramus sinus carot
SOPHARYNGEAL NERVE), IX CR A icus reaches the baroreceptors of sinus caroticus and
NIAL NERVE hemoreceptors of glomus caroticum.
The somatosensory fib e rs fo r pain, temperature
N. glossopharyngeus is a mixed nerve - it con and touch innervate the skin behind the ear pinna and
sists of motor, sensory, specific sensory (taste) and external auditory canal. Their neurons are located in
parasympathetic secretory fibers. The nuclei of the the smaller ganglion superius. The central branches
nerve that are common with those of X and V cranial of these neurons end in nucl. tr. spinalis n. trigemini
nerve are situated in medulla oblongata. The roots of - common for V, VII, IX and X cranial nerves.
the nerve leave the medulla oblongata dorsally from Examination. Usually the IX and X cranial nerve
the oliva and unite in common trunk. It emerges from are examined together because of their common
the skull through foramen jugulare together with X functions. By observation is estim ated the sym m e
and XI cranial nerves. The two sensory ganglia of try of the both halves of the soft palate, the position
the nerve are located in foramen jugulare. Thereafter of the uvula, their m otility during the act of phona-
the nerve travels through the parapharyngeal area be tion (continuous pronouncing of the letter “a”). The
tween the carotid artery and jugulary vein and gives swallowing is examined by drinking water. The ex
branches for the lateral wall of the pharynx and root amination includes also pharyngeal reflex, the sense
of the tongue. of touch of the soft palate, palate arches, and phar
The motor fibers start from nucl. ambiguous that is ynx, the gustatory sensation of the posterior 1/3 of
common with n. vagus. The glossopharyngeal nerve the tongue for bitter and sour, and saliva secretion.
innervates independently only one of the pharyngeal The reflex of sinus caroticus is examined by press
muscles - m. stylopharyngeus. Most of the pharynge ing the sinus that leads to slow heart rate and low
al muscles and muscles of the soft palate are innervat blood pressure.
ed by n. vagus.
The secretory
parasympathetic
preganglionic f i b
ers for innervation
of the parotid gland
start from nucl.
salivatorius inferi
or. They leave the
trunk of the nerve
as n. tympanicus. It
forms plexus tym
panicus in the inter
nal ear and reaches
ganglion oticum as
n. petrosus minor.
From ganglion oti
cum start the post
ganglionic fibers
which via n. auric- Fig-6.11. Bulbar cranial nerves (Fitzgerald) 1. N.glossopharingeus, 2. N.vagus
6. Cranial nerves 47
Excitatory syndromes. 1). Glossopharyngeal neu The next motor branch of n. vagus is n. laryngeus re-
ralgia. It is characterized by strong shooting pain in currens which descends to the thoracic cavity, makes
the root of the tongue, tonsil, and pharynx that some a loop around a. subclavia to the right and around the
times irradiates towards the ear. The pain is provoked aortic arc to the left and ascends to the larynx, travel
by swallowing, chewing, speaking, sneezing, touching ling between the trachea and esophagus. The terminal
the tonsil. 2) Myoclonus o f the soft palate - rhythmic, branch of the nerve is n. laryngeus inferior. It inner
involuntary contractions on the affected side. vates the muscles of larynx and provides the phonation
Negative syndromes. The soft palate stays lower on and breathing. The main trunk of n. vagus descends in
the affected side and is immobile. The uvula is deviated the cervical zone laterally from the carotid artery and
to the healthy side. The pharyngeal reflex is absent. The medially from the jugular vein, enters in the thoracic
sensation of the affected half of the soft palate, phar and abdominal cavity and participates in the formation
ynx, and posterior part of the tongue is diminished. The of the visceralpexuses (pi. cardiacus, pi. pulmonalis, pi.
taste sensation in the posterior part of the tongue is re esophageus, pi. gastricus ant. and post., pi. hepaticus,
duced. The swallowing is disturbed (dysphagia). When pi. celiacus). N. vagus provides the visceromotor and
drinking, the water comes out through the nose because viscerosensory parasympathetic innervation of the or
the soft palate is not able to close the nasopharynx. The gans of the thoracic and abdominal cavity - narrows the
speech is disturbed (dysarthria) due to the paralysis of bronchi, reduces the heart rate, accelerates peristalsis,
the soft palate and pharynx. These disturbances are of increases the secretion of the glands, etc.
ten accompanied by symptoms of damage of the other The somatic motor fibers start from nucl. ambiguus
bulbar nerves (X and XII cranial nerves) and develop - common with n. glossopharyngeus, and innervate the
ment of a bulbar palsy. pharyngeal and laryngeal muscles thus providing the
swallowing, phonation and breathing. The motor nuclei
of X and IX cranial nerve have bilateral cortical inner
6.7. N. VAGUS (VAGUS NERVE), vation and so the supranuclear lesions do not affect the
X CRANIAL NERVE pharynx and larynx.
The visceromotor preganglionic parasympathetic
N. vagus is a mixed nerve. Its territory of innerva fibers start from nucl. dorsalis n. vagi that is placed cau
tion is extensive and exceeds the area of head. The nu dally from nucl. salivatorius superior and nucl. saliva-
clei of the nerve are located in medulla oblongata and torius inferior. These fibers reach the visceral plexuses
some of them are common with other cranial nerves. and ganglia from which start the postganglionic para
The roots of the nerve exit in sulcus lateralis dorsa sympathetic fibers for innervation of the organs in the
lis, caudally from the roots of IX cranial nerve. There thoracic and abdominal cavity.
after they unite in a common trunk that leaves the skull The somatosensory fibers for innervation of the
through foramen jugulare. Immediately below it are pharynx, uvula and glottis represent peripheral branch
situated the sensory ganglia of vagus nerve - gangli es of the pseudounipolar cells of the smaller ganglion
on superius (ganglion jugulare) and ganglion inferius superius (jugulare). They participate also in formation
(ganglion nodosum). Ganglion superius gives two sen of n. auricularis which together with similar fibers of IX
sory branches: ramus meningeus innervates dura ma and VII cranial nerve innervates the skin behind the ear
ter of the posterior cranial fossa and ramus auricularis pinna and external auditory canal. The central branches
that unites with similar branches of IX and VII cranial of the ganglion cells terminate in nucl. tractus spinalis
nerve and innervates the skin of the ear pinna and the n. trigemini.
external auditory canal. Manipulations as irrigation of The viscerosensory fibers are peripheral branches of
the ear or ear examination with speculum can provoke the cells of ganglion inferius (nodosum). They partic
strong irritation of ramus auricularis with consecutive ipate in the innervation of the larynx and esophagus,
reduction of the heart rate or even cardiac arrest. Gan the lower part of pharynx, baroreceptors and hemore-
glion inferius gives rami pharyngei which form plexus ceptors of sinus and glomus caroticus and aorta and the
pharyngeus together with similar branches of IX cra thoracic and abdominal organs. The central branches
nial nerve and sympathetic fibers of ganglion cervical of the neurons terminate in the caudal area of nucl. tr.
superior. Plexus pharyngeus innervates the muscles and solitarii (nucl. cardiorespiratorius). The viscerosensory
mucous membranes of the pharynx, soft palate, and parasympathetic afferentation normally remains unreal
uvula, palate arches (except m. stylopharyngeus and ized. It participates in accomplishment of several auton
m. tensor veli palatini). The motor innervation is real omous reflexes. The visceral pain from internal organs
ized predominantly by the vagus nerve and the sensory is transmitted predominantly by sympathetic fibers.
one - predominantly by the glossopharyngeal nerve. N. vagus contains small amount of taste sensation
Another branch of n. vagus is n. laryngeus superior. It fibers for innervation of epiglottis in adults. It has a
provides the viscerosensory innervation of the mucous greater role in infancy.
membranes of the larynx to the level of the vocal cords Examination. Usually the IX and X cranial nerve
and the motor innervation of the laryngeal muscles - m. are examined together for position and mobility of the
constrictor pharyngis inferior and m. cricothyreoideus. soft palate and uvula, pharyngeal reflexes, for swallow
48 / Neurology
ing and phonation. The carotis sinus reflex is examined Syndromes of damage. The involuntary tonic or
by pressing the carotid sinus that induces bradycardia clonic contraction of the sternocleidomastoid muscle
and arterial hypotonia. A specific examination of the with subsequent hypertrophy (torticolis spastica) is a
vagus nerve is the indirect laryngoscopy which inspects relatively frequent extrapyramidal focal dystonia. Its
the position and mobility of the vocal cords during pho etiology is unknown but could be a result of nerve irrita
nation. tion from pathologic processes. The lesion of the nerve
Excitatory symptoms. The neuralgia o f n. laryn- or of its nucleus lead to palsy and hypotrophy of the in
geus superior is a rare condition connected with strong nervated muscles, reduced mobility of the head, and low
shooting pain involving the lateral cervical aspects and position of the shoulder. Most often the lesions of the
irradiating to the ear, sometimes accompanied by a for nerve occur simultaneously with lesions of the IX and
cible cough. The spasms of esophagus, cardia and lar X cranial nerves in the course of pathologic processes
ynx with hysteric aphonia are also rare conditions. involving foramen jugulare.
Negative symptoms. The unilateral lesions lead to
soft palate falling and absence of a pharyngeal reflex on
the affected side. The uvula is deviated to the healthy 6.9. N. HYPOGLO SSUS (H Y P O G LO S
side, dysphagia and dysphonia are observed. The vocal SAL NERVE), XII C R A N IA L NERVE
cord occupies medial position and stays stationary in pro
nouncing the letter “a”. The isolated lesion of n. recurrens This nerve is a pure motor nerve and innervates the
in the course of surgical interventions in the cervical area muscles of the tongue. Its motor nucleus is localized in
(for example thyreoidectomy) leads to dysphonia without the dorsal medial and caudal aspects of medulla oblon
dysphagia. The bilateral damage of n. vagus leads to a gata beneath the floor of the IV ventricle. The roots of
bulbar palsy - a life threatening condition characterized the nerve emerge ventrolaterally from the brainstem,
by a soft palate and pharyngeal palsy with dysphagia, between the pyramid and olive. The nerve exits the skull
leakage of fluids through the nose, getting food in the through canalis n. hypoglossi. Thereafter the nerve de
windpipe, nasal speech (dysphonia) to aphonia, larynge scends in the cervical area and at the level of the hyoid
al muscles palsy with dyspnea, abnormal heartbeat with bone enters the tongue (fig. 6.11) and innervates m. hy-
tachycardia and arrhythmia, paretic ileus, etc. oglossus (attached to the hyoid bone), m. genioglossus
(attached to the mandible), and m. styloglossus (attached
to processus styloideus). N. hypoglossus innervates also
6.8. N. ACCESSORIUS (ACCESSORY the internal muscles of the tongue. The nerve is tempo
NERVED IX CR ANIAL NERVE rally joined by motor fibers of C1-C3 roots (ansa n. hy
poglossi) for innervation of the sublingual muscles (m.
This nerve is a pure motor nerve and has a spinal thyreohyoideus, m. sternohyoideus, and m. omohyoide-
origin. Its nucleus is situated in the dorsolateral parts us). The proprioceptive sensory innervation, provided
of the anterior horns of the first 5-6 cervical segments by the muscle spindles, is transmitted by the trigeminal
(C1-C5). Its roots exit through the lateral aspects of the nerve to nucl. mesencephalicus n. trigemini. The nucle
spinal cord, unite in a common trunk and enter the skull us of n. hypoglossus receives only contralateral cortical
through foramen magnum. In the skull vagus nerve innervation because of the complete supranuclear cross
fibers coming from the lower part of nucl. ambiguous ing of the corticobulbar tract. The tongue participates in
temporally join the IX cranial nerve. These fibers sub the acts of articulation, swallowing and chewing.
sequently return in the composition of n. vagus. The ac Exam ination. The observation tests the trophic and
cessory nerve exits the skull through foramen jugulare the volume of the tongue, the presence of fasciculations,
together with IX and X cranial nerve. In the cervical protrusion of the tongue out and its movements in dif
region the nerve is joined by somatic sensory fibers for ferent directions.
innervation of the upper cervical muscles. N. accesso Syndromes of damage. The unilateral lesion of
rius innervates m. sternocleidomastoideus (deviation of the nucleus or of the trunk of the nerve leads to flaccid
the head to the same side and turning it to the opposite atrophic palsy - hemiatrophy and homolateral devia
side) and m. trapezius (raises the shoulder, pulls it back tion o f the tongue. When the patient is asked to show
and adducts scapula). The simultaneous contraction of his tongue, it deviates to the side of the lesion because
both mm. sternocleidomastoidei bends the head for the contralateral healthy m. genioglossus pushes it to
ward. The bilateral contraction of m. trapezius bends the opposite side in absence of resistance of the paralyz
the head backward. ed homolateral m. genioglossus. In bilateral peripheral
Examination. By observation the examiner tests the palsy the tongue is completely immobile - glossopa-
trophic and volume of the muscles. The examination of resis. The speech is incomprehensible (dysarhtria and
the muscle tone and active movements includes turning anarthria), the chewing and swallowing is disturbed. In
of the head to the left and to the right, bending forward chronic nuclear processes fascicullations are observed
and backward and raising the shoulders. The examina in addition to the atrophy. The unilateral supranuclear
tion of the muscle strength is performed by applying re damage (corticobulbar tract) leads to contralateral cen
sistance to the active movements. tral palsy o f the tongue - it deviates to the side of the
6. Cranial nerves 49
paralyzed limbs. The stroke in the medial basal part The syndrome of the jugular outlet most often results
of medulla oblongata leads to alternating syndrome of of nasopharyngeal tumors. Its clinical picture contains
Jackson - ipsilateral peripheral lesion of the tongue and partial bulbar palsy, pain behind and in the ear due to an
contralateral hemiparesis. irritation of the auricular branches of IX and X cranial
nerves), headache (due to an irritation of the meninge
al branch of X cranial nerve), Clode Bernard - Horner
6.10. COMBINED D1SODERS OF syndrome (due to involvement of n. caroticus internus)
CHEWING, SWALLOWING AND and damage of the XI cranial nerve.
SPEAKING (BULBAR AND PSEUDOB The bilateral bulbar palsy is a life-threatening con
ULBAR PALSY) dition in cases of acute development or quick progres
sion. It is characterized by anarthria, aphonia, aphagia
The simultaneous involvement of the bulbar group and glossoplegia. A danger of chocking and mechanical
cranial nerves (IX, X and XII) or their nuclei causes obstruction of the airways and larynx exists due to the
bulbar palsy - an analog to the peripheral flaccid palsy. bulbar palsy with total immobility of the tongue, soft
The bilateral damage of the corticobulbar pathway leads palate, pharynx and larynx. The autonomous parasym
to pseudobulbar palsy that corresponds to the central pathetic reflexes connected with cardio circulation ac
palsy. A mandatory condition for occurrence of a pseu tivity and respiration is disturbed. An urgent intubation,
dobulbar palsy is a bilateral pathologic process due to aspiration and artificial pulmonary ventilation are nec
the bilateral cortical innervation of the IX and X cranial essary. The patients with Myasthenia gravis can devel
nerves. op a myasthenic crisis accompanied by weakness of the
1. Bulbar palsy. It can be observed in the bulbar bulbar muscles - dysphagia, dysphonia, dyspnea. The
form of poliomyelitis, rabies, the ascending paralysis of myasthenic crisis can be induced by intake of medicines
Landry in the course of the acute demyelinative inflam that disturb the neuro-muscular transmission.
matory polyneuropathy (syndrome of Guillaine-Barre), 2. Pseudobulbar palsy. It can be observed in vas
diphtheritic polyneuritis, cerebral stroke involving me cular encephalopathy, encephalitis and diffuse cortical
dulla oblongata (syndrome of Wallenberg), basal neo and subcortical processes. In patients with Amyotrophic
plastic or leucosis infiltrates, etc. lateral sclerosis is observed simultaneous involvement
The unilateral bulbar palsy is connected with dis of both central and peripheral motor neurons with de
turbances in speech (dysarthria, dysphonia), swal velopment of a combined bulbar and pseudobulbar pal
lowing (dysphagia) and chewing. The speech is nasal, sy. The clinical picture of the pseudobulbar palsy is
hoarse, poorly articulated due to a palsy of the soft similar but much milder than those of the bulbar palsy
palate, tongue and vocal cords. The swallowing is dif and does not represent a life-threatening condition. It is
ficult, the liquids outflow through the nose due to the characterized by dysphagia, dysphonia, dysarthria and
incomplete closing of the nasopharynx from the paret glossoparesis. The speech is poorly articulated, hoarse,
ic soft palate. Foods and liquids may fall in trachea and and spastic due to the central spastic palsy of the ar
cause aspiration pneumonia. The tongue on the side ticulator muscles. The tongue is spastic, with reduced
of the lesion is atrophic and deviates to the side of the mobility but its trophic is preserved. The swallowing is
lesion. In nuclear damage of the hypoglossal nerve are disturbed due to the spastic paresis of the swallowing
observed contractions of the lingual muscles (fascicu- muscles. The pharyngeal reflex is preserved. The man
lations). The pharyngeal reflex on the side of the lesion dibular reflex is exaggerated and sometimes clonus of
is absent. The affected side of the soft palate droops the mandibula can be observed. The pathologic reflexes
down and do not participate in phonation. The uvula of oral automatism become positive. Unprovoked laugh
deviates to the healthy side. The indirect laryngoscopy ter or crying are often observed due to liberation of the
reveals median position and immobility of the affected subcortical emotional reactions of the cortical control.
vocal cord. The general sensation of the soft palate, The bulbar and pseudobulbar palsy are connected
pharynx and posterior third of the tongue is disturbed. with immobility of the tongue, soft palate and pharynx
The taste sensation of the posterior third of the tongue and may complicate the dental manipulation of the oral
is disturbed also. cavity by liquid aspiration.
50 / Neurology
The Autonomous nervous system (ANS) also re afferent fibers reach the spinal cord where they interact
ferred to as vegetative or visceral nervous system, in with the sensory neurons of posterior horns and with
nervates the internal organs and blood vessels and main the efferent autonomous neurons in the intermediolat-
tains homeostasis. The activity of the ANS is independ eral columns of the grey matter. The second neuron of
ent (autonomous), involuntary and remains unrealized. the visceral sensation is localized in the posterior horns.
It works in close collaboration with the somatic nervous Its axons join the lateral paleospinothalamic tracts and
system and endocrine system. The ANS is subdivided spinoreticular tracts and reach the reticular formation of
to peripheral (sympathetic and parasympathetic part) the brainstem, thalamus, hypothalamus, and limbic mo
and central (reticular formation o f the brainstem, hypo tor cortex. As opposite to the somatic nervous system,
thalamus and limbic system). A separate compartment the efferent visceral impulses do not reach the internal
of the peripheral ANS is the enteric nervous system that organs directly but via two neurons - preganglionic and
innervates the gastrointestinal tract and controls its mo postganglionic.
tility and secretion.
The activity of ANS similarly to those of the somatic
nervous system is based on the principle of the reflex 7.1. SYMPATHETIC NERVOUS SYS
arc. The impulses of the visceral receptors (mechanore- TEM (FIG.7.1)
ceptors, hemoreceptors, osmoreceptors, and pain recep
tors) are transmitted via poor myelinated A6 and non This system is called sympathetic because it acts in
myelinated C fibers of the afferent neurons. Their cell “sympathy” with the emotions. The preganglionic neu
bodies are located in the spinal ganglia. The visceral rons are placed in nucl. intermediolaterlis of the lateral
Fig.7.1. Sympathetic nervous system (left part) and parasympathetic nervous system (right part). (Martin)
7. Autonomous nervous system 51
horns from Thl to L2 segments of the spinal cord. Their starting from the terminal ganglia near the internal or
myelinated axons leave the spinal cord with the ante gans. 1 he cranial parasympathetic portion innervates
rior roots and via rami communicantes albi reach the the pupils, lacrimal and salivary glands.
paravertebral ganglia of the sympathetic trunk (trun- 1) From the parasympathetic nucleus o f West-
cus sympaticus). This is a paired structure situated on phal-Edinger in the midbrain start preganglionic fibers
both sides of the spinal cord. It consists of 3 cervical, that reach ganglion ciliare in the orbit in the composi
11 thoracic, 4 lumbar and 4 sacral ganglia in which are tion of n. oculomotorius. The short postganglionic fibers
located the cell bodies of the postganglionic neurons. innervate m. sphincter pupillae for constriction of the
The postganglionic fibers are non myelinated and via pupil when light falls on it (pupillary light refiex) and
rami communicantes grisei return back in the compo m. ciliaris for accommodation of the eye.
sition of the spinal nerves for segmental innervation of 2) From nucl. salivatorius superior in the pons start
the blood vessels, sweat glands, and pile erector muscles preganglionic fibers that as a part of n. intermedius and
of the hair. Some of the preganglionic fibers pass direct via its branch n. petrosus superficialis reach ganglion
ly through the paravertebral ganglia without stopping pterygopalatinum. From this ganglion start postgangli
there and continue as sympaphetic (splanchnic) nerves. onic fibers that join n. zygomaticus and participate in
They end in the prevertebral ganglia near the internal the innervation of the lacrimal glands, nasal glands, si
organs. The medulla of the suprarenal gland is innervat nuses, soft palate and pharynx. The rest of the parasym
ed by preganglionic fibers. It cells, secreting adrenalin pathetic fibers leave the facial nerve as chorda tympani
and noradrenalin are regarded as an analogous of the and reach ganglion submandibulare. The postganglionic
sympathetic ganglia. The preganglionic sympatheic in fibers starting from this ganglion innervate the sublin
nervation of the head starts from Thl-Th2 segments of gual and submandibular salivary glands.
the spinal cord. The postganglionic one starts from the 3) From nucl. salivatorius inferior start pregangli
superior cervical ganglion. The sympathetic network onic fibers for the parotid gland. They join the glos
surrounding the carotid artery and its branches inner sopharyngeal nerve and via plexus tympanicus and n.
vates the blood vessels, sweat, and lacrimal glands and petrosus minor reach ganglion oticum. From the latest
the eye (m. dilatators papillae and m. tarsalis superior). start postganglionic fibers that terminate in the parotid
The sympathetic innervation of the neck, thorax and gland in the composition of n. auriculotemporalis.
upper limb is provided by Th2-Th5 spinal segments, 4) Preganglionic fibers from nucl. dorsalis n. vagi in
from the inferior cervical and the upper thoracic gan the composition of n. vagus reach the parasympathetic
glia. Ganglion cervical inferior often unites with the up ganglia in the walls of the thoracic and abdominal cav
per thoracic ganglion in ganglion stellatum. The latest ity internal organs.
gives rise to the majority of the sympathetic fibers for The sacral parasympathetic portion starts from
the heart (pi. cardiacus) and for the lungs (pi. pulmona- the lateral horns of S2-S4 spinal segments. The pregan
lis). The sympathetic innervation for the abdomen starts glionic fibers, in the composition of nn. splanchnici pel-
from Th5-Thl0 spinal segments and those for lower vini, reach the ganglia in the distal part of the colon and
limbs - from Thl0-L2. The preganglionic innervation pelvic organs.
is provided by thoracic splanchnic nerves (for the ab The parasympathetic fibers are cholinergic - secret
dominal cavity) and by lumbar and sacral splanchnic ing acetylcholine which binds to the muscarinic recep
nerves (for pelvis). The postganglionic fibers start from tors of the internal organs. The parasympathetic stim
the prevertebral gangl. celiacum, gangl. mesentericum ulation leads to myosis, bradicardia, bronchial spasm,
superius, gangl. mesentericum inferius. and increased motility of the gastrointestinal tract with
The sympathetic fibers are adrenergic - the postgan sphincter relaxation and bladder and rectal emptying,
glionic fibers release noradrenalin which is binding to increased secretion of the lacrimal, salivary, bronchial,
the a- and 0-andrenergic receptors. The stimulation of stomach and intestinal glands, increased insulin secre
the sympathetic nervous system, for example by stress, tion. The parasympathetic effects are more limited and
is connected with generalized physiological reactions long lasting in comparison to the sympathetic. They are
requiring energy expenditure - secretion of adrenalin connected with anabolic, excretory and reproductive
from the suprarenal glands, mydriasis, tachycardia, el functions.
evation of the blood pressure, peripheral vasoconstric The enteric nervous system consists of two plex
tion, bronchodilation, suppression of the intestine peri uses: the myenteric plexus o f Auerbach which controls
stalsis and secretion, hyperhidrosis, hyperglycemia, etc. the motility of the gastrointestinal tract and the submu-
cose plexus o f Meissner which regulates the water and
electrolyte transport via the epithelium membranes. The
7.2. PARASYMPATHETIC NERVOUS sympathetic system inhibits while the parasympathetic
SYSTEM (FIG.7.1) system activates the enteric nervous system.
Most of the internal organs have double innervation
As opposite to the sympathetic nervous system, the - sympathetic and parasympathetic which have con
preganglionic parasympathetic fibers have long way and troversial effects. For example, the sympathetic effect
postganglionic parasympathetic fibers have short way, upon the heart is excitatory, while the parasympathetic
52 / Neurology
effect is inhibitory. All preganglionic neurons (sympa piratory muscles) and pneumotaxic center in pons, that
thetic and parasympathetic) just like the postganglion plays a pacemaker roll for dorsal center control. The
ic parasympathetic neurons are cholinergic - secreting dorsal nucleus controls inspiration and the ventral one -
acetylcholine. The postganglionic sympathetic neurons exhalation. The dorsal center has its own basic rhythm
innervating the sweat glands and blood vessels of the but is under the influence of afferent impulses from the
skeleton muscles are cholinergic also. All the rest post hemoreceptors of glomus caroticus (IX cranial nerve),
ganglionic sympathetic fibers are noradrenergic. The aortic arc (X cranial nerve) and pulmonary receptors.
cholinergic receptors are of two types —nicotinic (in the The cardiovascular control is performed by the pres
vegetative ganglia and skeleton muscles) and muscarin sor sympathetic center that is situated in rostral ven
ic (in the effector organs - smooth muscles, glands). The trolateral RF of medulla oblongata. It sends descending
adrenergic receptors are of two types - a and (i. The tonic impulses to the preganglionic sympathetic spinal
al-adrenergic receptors are with postsynaptic localiza neurons for vasoconstriction and stimulation of the
tion and when activated induce mydriasis, vasoconstric heart activity. The depressor center is localized in the
tion, sphincter contraction. The a2-adrenergic recep caudal part of the ventromedial RF. It sends efferents to
tors are with presynaptic localization and in response the parasympathetic nucl. dorsalis n. vagi for vasodila
to excitation inhibit the liberation of neurotransmitters. tion and slowing of the heart rate. Both centers generate
The postsynaptic pi-adrenergic receptors of the heart constant rhythmic activity that is influenced by the ba-
increase the heart rate and contractility, and those of the roreceptors and hemoreceptors of the carotid sinus, aor
kidneys - the secretion of rennin. The latest increases tic arc, vena cava and heart atrium. These afferentations
the vasoconstrictor effect of angiotensin II. The post reach nucl. tr. solitarius via IX and X cranial nerves. In
synaptic p2-receptors in excitation lead to bronchodi- response to increase in blood pressure, nucl. tr. solitari
lation and liberation of glycogen from the hepatocytes. us activates the depressor center and inhibits the pressor
one. This is the mechanism of the baroreceptor reflex.
reactions. The limbic structures are strongly epilepto sympathetic (sympathetic-adrenal) crises are observed
genic. Clinically are observed simple partial seizures in neurotic disorders, panic attacks, stress. They are
(olfactory, gustatory, vegetative, psychic), complex par characterized by blood pressure elevation, increased
tial seizures with variety of psychomotor automatisms heart rate, tightness in the heart area, prickling of the
and psychosensory reactions, and secondary general skin, pallor, sweating, mydriasis, agitation, vital fear.
ized seizures. The bilateral anterior temporal lobotomy The parasympathetic crises are characterized by de
leads to syndrome of Kluver-Bucy, characterized by hy creased blood pressure, slow hart rate, decreased res
persexuality, hyperphagia, absence of reactions of fear. piratory rate, sense of suffocation, vertigo, hypersaliva
tion, nausea, vomiting, diarrhea, myosis.
The brainstem autonomous syndromes are observed
7.6. METHODS OF EXAMINATION OF in patients in coma and result of involvement of the
THE AUTONOMOUS NERVOUS SYS cardial and respiratory centers in medulla oblongata.
TEM They are characterized by pathologic types of breath
ing - Cheine-Stokes type, Kussmaul type, Biot type, by
The neurovegetologic anamnesis and examination irregular heart rate and variations in the blood pressure.
define the type and localization of the autonomous dis The following syndromes are accepted as local: 1) oph
orders. The following vegetative reflexes are examined: thalmoplegia oculomotoria interna (mydriasis and sta
1) pupil reactions of light and accommodation; 2) reflex tionary pupil) as a result of a damage of the parasympa
es of bowel and bladder; 3) pilomotor reflex - prickling thetic nucleus and parasympathetic fibers in the oculo-
of the skin in response of irritation with needle or with mor nerve; 2) disturbed lachrymal and saliva secretion
cold object; 4) perspiration reflex - iodine-starch meth as a result of involvement of the parasympathetic nuclei
od of Minor. The pilomotor and perspiration reflexes are and fibers in VII and IX cranial nerves; 3) the lesion of
sympathetic segmental reflexes. The reactivity of the the parasympathetic nucleus of n. vagus leads to dis
ANS is tested by: 1) Sinus caroticus reflex (the press turbed parasympathetic innervation of the thoracic and
ing of the carotid sinus normally leads to slowing of the abdominal organs.
heart rate by 5-10 beats/min and decrease of the blood The spinal segmental sympathetic syndromes due to
pressure by 10 mmHg); 2) Test of Valsalva - after deep lesions of Thl-L2 segments are characterized by vaso
inspiration and expiration against resistance the blood motor, pilomotor and thermoregulatory disturbances in
pressure decreases and the heart rate increases, followed the affected dermatomes. The syndrome of Horner (pto
by elevation of the blood pressure and slowing of the sis, myosis and subjective impression of enophthalmus)
heart rate; 3) Respiratory sinus arrhythmia - increasing is observed in lesion of centrum ciliospinalis (C8-Th2
of the heart rate during inspiration and slowing during spinal segments), superior cervical sympathetic gangli
expiration; 4) Orthostatic test of Shellong - when get on, sympathetic plexus surrounding the carotid artery,
ting up from supine position normally the blood pres descending hypothalamic-spinal sympathetic pathway.
sure elevates by 20 mmHg and the heart rate increase The sympathetic excitation is accompanied by mydria
by 30 beats/min; 5) Cold pressor test - the immersion sis, wider eye slit, and subjective impression of exoph-
of the hand in cold water leads to vasoconstriction with thalmus (syndrome of Pourfour du Petit).
increasing of the blood pressure; 6) Sympathetic skin The peripheral vegetative syndromes are observed
response - changes in the skin resistance in response in damage of the autonomous fibers in the roots, plex
of activation of the sweat glands; 7) Examination of the uses, peripheral nerves and tr. sympaticus. Usually they
lachrymal secretion with the test of Shirmer; 8) Phar are accompanied by causalgic pain that spreads outside
macological tests for testing sympathetic and parasym the innervation area of the affected peripheral nerve and
pathetic nervous system reactivity; 9) Hormonal testing has heating and diffuse character. Vasomotor, secretory
- pituitary hormones, catecholamines, rennin, aldoster- and trophic changes are also observed. The syndrome o f
on, etc.; 10) Doppler sonography of the cerebral blood Raynaud is characterized by painful pallor of the fingers
vessels and blood vessels of the limbs and testing of the in exposure to cold or emotional stress. The increased
cerebral blood vessel reactivity; 11) Infrared thermom sweating (hyperhydrosis) can be localized (hands and
etry. feet) or generalized. The gastrointestinal dysfunctions
(hypomotility, hypermotility, colics, constipation, and
diarrhea) are observed in diabetic and other polyneu
7.7. NEUROVEGETATIVE SYN ropathies. The orthostatic hypotonia is diagnosed when
DROMES the systolic blood pressure is decreased by more than 30
mmHg when getting up from supine position. As a re
The neurovegetative syndromes are subdivided to sult of the cerebral hypoperfusion is observed general
sympathetic, parasympathetic and combined; to local ized weakness, dizziness, sinking. It can be observed in
ized and generalized; and according their course - to older patients, in some of the polyneuropathies, intake
paroxysmal and permanent. The generalized paroxys of vasodilators, damage of the sympathetic structures
mal disorders are also termed vegetative crises. The in multiple systemic atrophy, Parkinson’s disease, etc.
7. Autonomous nervous system 55
7.8. AUTONOMOUS INNERVATION level above the lumbar intumescense interrupts the voli
OF THE PELVIC ORGANS tional innervation of bladder but the autonomous is pre
served. Spastically increased muscle tone of the external
The sympathetic center for bladder control is situat sphincter leads to urinary retention. In overfilling of the
ed in the lateral horns of spinal segments Thl0-L2. The bladder is observed periodic reflex involuntary bladder
sympathetic nn. splanchnici lumbales perform the act empting (incontinentio urinae intermittens). The lesions
of urine retention by relaxation of the detrussor muscle below LI level interrupt both volitional and autonomous
and contraction of the internal sphincter. The parasym innervations - the patient does not feel the urge to uri
pathetic center in S2-S4 segments of conus medularis nate, the sphincters and detrrussor muscle are relaxed,
performs the bladder empting by constriction of the de there is overfilling of the bladder and permanent urine
trussor muscle and relaxation of the internal sphincter. incontinence (incontinentio urinae vera). The innerva
The somatic conscious sensation for filling of the blad tion of rectum is similar to those of bladder. The sympa
der with urge for urination is performed by nn. puden- thetic center in Thl2-L2 spinal segments constricts the
dales, S2-S4 spinal segments, somatosensory pathways, external anal sphincter and retains defecation. The par
and primary sensory cortex in lobules paracentralis. The asympathetic center in S2-S4 spinal segments relaxes
cortical motor center for volitional urination is located the sphincter and initiates defecation. The autonomous
further ahead of the sensory center in lobules paracen innervation of the genitals is both parasympathetic (for
tralis. In increased activity of the detrussor muscle as a erection) and sympathetic (for ejaculation). Impotentio
result of pyramidal lesion is observed urinary urgency generandi means lost fertility and impotentio coeundi -
and incontinence. The transection of the spinal cord on inability for copulation performance.
56 / General neurology
The cerebral cortex is the youngest and most compli read and to understand written speech. The patient can
cated structure which realizes the supreme regulation of write under dictation and spontaneously but is unable
the motor and sensory functions. The cortical areas that to understand the read - alexia without agraphia (pure
receive afferent projections of the general and special verbal blindness). It results of lesion in the border occip
ized sensation and the areas that send direct projections ital-temporal area.
to the motor neurons are termed primary (projection) The auditory agnosias are subdivided to nonverbal
areas. In these areas the sensory and motor functions and verbal. Nonverbal agnosias are: 1) object auditory
have somatotopic presentation. The lesions of these ar agnosia (failure in recognition of the objects by the spe
eas lead to localized in space hypoesthesia and anes cial sounds produced by them); 2) agnosia for human
thesia, paresis and plegia. The secondary cortical zones voices (phonoagnosia); 3) agnosia for music tones (amu-
are neighboring the primary areas. They perform more sia); 4) agnosia for rhythms. The speech auditory (ver
complicated processing of the sensory information (per bal) agnosia results of disturbance of the acquired abil
ception or gnosis) and programming of the motor activ ity for identification of the speech sounds (phonemes).
ities (praxis). Their lesions lead to disturbed cognitive The patient is unable to understand the verbal speech, to
sensory perception (agnosia) and destruction of the de repeat heard words and to write under dictation but he
veloped motor skills and habits (apraxia). The tertiary can speak, write and read (pure verbal deafness). The
(associative) cortical areas include the so-called “area lesion is in the left temporal associative auditory cortex.
of the three lobes” (the border area of the parietal, oc The tactile agnosia (stereoagnosia) is characterized
cipital and temporal lobes) and prefrontal cortex (the by inability for recognition of the objects by palpation.
anterior frontal cortex located in front of the premotor It results of lesion of the parietal associative cortex.
cortex). In lesions of the tertiary areas are observed The visual-spatial gnosis is function of the right
more global behavioral and intellectual disturbances. parietal lobe with participation of the visual, vestibular
The involvement of the secondary and tertiary fields of and somatosensory systems. In visual-spatial agnosia
the left hemisphere is connected with speech agnosia - the patient is unable to orient in space, by map, to find
disturbance of the sensory perception of speech sounds his way back home, he is unable to recognize the arrows
(phonemes) and of speech written signs (graphemes). of the clock. In simultanagnosia the perception of more
The motor production of phonemes and graphemes is than one object in the space and the understanding of
disturbed because of involvement of the articulatory and the situation are impossible. It is a part of the Balint’s
written praxis - speech apraxias. The disturbances of syndrome - absence of attention to the objects in the
the semantic and grammatical rules of the language are peripheral visual field. In the unilateral visual-spatial
termed aphasias. The higher cortical functions (gnosis, ignoring the patient is unable to read the left half of
praxis, speech) are built by the processes of education a certain text, to draw in the left part of the sheet. He
and imitation in the early childhood. ignores the tactile irritations in the left half of the body
due to right parietal lesions.
The disturbances of the body perceptions and the
8.1. GNOSIS AND AGNOSIAS picture of someone’s own body (somatoagnosia) are di
vided to: 1) hemiasomatognosia - ignoring one of the
Gnosis (perception) is acquired via education abili body’s halves, most often the left one; 2) autotopagno-
ty for recognition and identification of objects and phe sia - the patient is unable to show parts of his body or
nomena perceived by the senses and compared with the he does not recognize them as his own; 3) anosognosia
past experience. Agnosia is disturbed gnosis, inability - selective absence of perception of someone’s own de
for recognition and identification of the sensory stimuli fect (hemiplegia, hemianopsia). The disturbances of the
while the elementary sensory functions are preserved gnosis of someone’s one body are result of lesions of the
and in absence of psychic disorders. According to the right upper parietal lobe. The syndrome o f Gerstman is
disturbed sensory modality the agnosias are divided to bilateral somatoagnosia - finger agnosia, left-right dis
visual, auditory and tactile. orientation, acalculia, sometimes alexia and homony
The visual agnosias are subdivided to: \) object mous hemianopsia. It is a result of left parietal lesions.
agnosia - inability to recognize the objects via visual
canal; the recognition is realized by the other senses;
2) color agnosia (in preserved color vision); 3) prosop 8.2. PRAXIS AND APRAXIA S
agnosia (inability to recognize the faces of popular or
close people; the recognition is auditory by voice); 4) Praxis is a complex of complicated automatic motor
speech visual agnosia (alexia) - the patient is not able habits and skills acquired via education and training.
to recognize letters and words by vision, he is unable to Apraxia is disturbance of the smooth and automatic per
8. Higher cortical functions 57
formance of the built during the individual life automat con) and rules for their combining (syntax). The small
ic skills in absence of palsy, ataxia, sensory and psychic est verbal units are the phonemes that haven’t meaning
disabilities. According to the scheme of Liepmann the but by their combining are obtained parts of the words
apraxias are divided to motor, ideomotor and ideation. (morphemes) or whole words (lexemes) that have par
The motor (melokinetic) apraxia results of damage ticular meaning. The basic language processes are lan
of the left premotor cortex. It corresponds to the mo guage encoding (generation of language message) and
tor aphasia and often accompanies it. The overall motor language decoding (understanding and comprehension
plan is preserved but its performance is clumsy and with of the linguistic message). Via complicated, learned
impaired automation. The patient looks like a person during the individual life motor programs are produced
who is trained for a first time to a new motor skill. The verbal sounds (phonemes) and verbal written characters
precise movements are extremely difficult. (graphemes). These processes are executed by the ver
The ideation apraxia results of larger cortical lesions bal articulator and verbal written praxis. In response to
and is just the opposite to the motor apraxia. It corre verbal message from someone else received by auditory
sponds to the sensory aphasia of Wernicke. The over or visual canal the signals at the beginning are recog
all plan for the movement is destroyed but its separate nized via the verbal auditory or visual gnosis. Thereaf
components of the movement are not much disturbed. ter a semantic and grammatical analysis of the perceived
The movements under verbal command, in imitation, sentences is performed, followed by language decod
with objects support and symbolic actions are impossi ing. The differentiation between language and speech
ble. Classic examples are those with lighting a cigarette is very important. The language is a system of signs
and the apraxia of dressing. The left-sided apraxia is re formed by education that keeps in brain the semantic
sulting of lesion of the anterior part of corpus callosum meanings and grammatical rules for performance of the
that disturbes the connection between the left and right language encoding and decoding. The speech includes
premotor areas. So the motor plan information from the the processes of production of the verbal signals (verbal
left premotor zone is inaccessible. praxis) and perceiving of the verbal signals (verbal gno
Ideomotor apraxia is observed in damage of lobu- sis). The speech is divided to articulator (spoken) and
lus parietalis inferior of the left hemisphere (areas 39 written. Other existing forms for language communica
and 40) or of the supplementary motor field and its con tions are Braille lettering, Morse code, typewriting, etc.
nections with tha basal ganglia. There is disconnection Aphasias are disturbances in the processes of lan
between the center for motor ideas and the performing guage encoding (genesis, creation of the language mes
motor center (similarly to conduction aphasia). The pa sages) and decoding (understanding, comprehension of
tient is unable to perform common actions under in the perceived language messages). Aphasias are subdi
struction by gestures in absence of real objects, for ex vided into two main types - sensory and motor. Motor
ample pouring water into a glass from imaginary kettle, aphasia results of anterior, pre-Rolandic lesions. The
speaking by phone, cutting with scissors, etc. He is una language encoding is disturbed, but language decod
ble to perform certain gestures under instruction - blow ing is relatively preserved and the patient realizes the
kiss, saluting, etc. and to show by mimics and gestures existing defect. The production of spontaneous speech
particular emotion - fear, unpleasant smell, etc. might be impossible or difficult, poor, with interrup
The constructive apraxia is observed in lesions in tions (non-fluent speech). Sensory aphasia results of
volving the right parietal-occipital-temporal area com posterior, post-Rolandic lesions. Both language encod
bined with disturbed visual-spatial gnosis. Typical are ing and language decoding are disturbed and the patient
the difficulties in sorting, drawing, constructing. It does not realize the defect. The production of sponta
might be accompanied by ignoring of the left side of neous speech is free and fluent, sometimes verbose and
the space and of the own body, by left hemianopsia uncontrolled (fluent speech). Nowadays the preferred
and apraxia of dressing. The finger apraxia and spatial terms are expressive (instead of motor aphasia) and im
apraxia of the posture are also referred here (inability pressive, receptive aphasia (instead sensory aphasia)
to perform by imitation particular postures with fingers because the existing problems are not on motor or sen
and postures of the arm to the face). sory level but in production and understanding of the
According to the affected actiones are dilterentiat- linguistic stimuli.
ed the following apraxias: 1) orofacial apraxia; 2) ar Classical scheme for explanation of aphasias is the
ticulator (verbal) apraxia; 3) apraxia of the writing; 4) “house model” of Lichthaim-Wernicke according to
apraxia of the gaze; 5) apraxia of walking; 6) apraxia which there exist: 1) motor center, containing the motor
of dressing. images of the verbal units; 2) sensory center, keeping
their sensory images; 3) ideation center, keeping the
semantic meanings of the verbal signs. Among these
8.3. LANGUAGE, SPEECH AND APHA centers exist associative connections, motor exit from
the motor center and sensory input to the sensory center
SIAS
- fig. 8.1. The lesions of the motor and sensory centers
The language is system of signs, sounds and sym lead respectively to cortical motor and cortical sensory
bols for communication, consisting of dictionary (lexi aphasia. The interruption of the connections between
58 / General neurology
the motor or sensory centers with the ideation center (echolalia). The understanding of the oral and written
leads to transcortical aphasias - motor or sensory. The speech is completely preserved. Repetition of speech,
interruption of the motor exit from the motor center the writing under dictation and the automatic speech
leads to subcortical motor aphasia. The lesion of the (counting, enumeration of months) are preserved. This
sensory input from the primary auditory cortex to the aphasia is often part of the frontal syndrome, manifest
sensory center of speech leads to subcortical sensory ed by absence of spontaneity, lack of initiative, abulia,
aphasia. According to the contemporary understanding disturbed attention and executive functions.
the subcortical aphasias are actually not aphasias be Cortical sensory aphasia of Wernicke. The lesion
cause the language functions are preserved and only the is in the posterior part of gyrus temporalis superior
verbal praxis and verbal gnosis are disturbed. The clas (area 42 according to Brodman) in the left hemisphere.
sification of A. R. Luria in Bulgaria is popular mostly It results of verbal-auditory agnosia and disturbed lan
among speech therapists. guage decoding (understanding) and encoding with in
Cortical motor aphasia of Broca. The lesion is in ability for differentiation of the phonemes in the word,
the posterior part of the inferior frontal gyrus (area 44 to be spoken and written. The spontaneous speech is
according to Brodman) in the left premotor cortex. Later fluent, without efforts and inhibitions (the verbal praxis
was established that the motor center of speech includes is preserved) but completely incomprehensible (absence
a larger area - frontal-parietal operculum and insula. of control upon the expressive speech from the poste
This aphasia is connected with disturbed verbal articu rior speech area) - fluent aphasia. Often the patients
lator praxis and the language encoding. The disturbanc speak much, without hearing the interlocutor (logor-
es in articulator praxis lead to inability for articulation rhea), there is plenty of phonemic substitutions (literal
(mutism) or to stereotype repeating of particular sylla paraphasias) and word substitutions (verbal, semantic
ble or word (speech embolism, perseveration). In mild paraphasias) or meaningless combinations of phonemes
er cases the oral speech is non-fluent, with difficulties (neologism) and words - jargonophasia. The paragram-
in pronunciation of word, interruptions, intermissions atism is typical - disturbed grammatically and syntac
and phonemic substitutions (literal paraphasias). The tic structuring of the language. The understanding of
language encoding (generation of verbal messages) is the heard and read speech is disturbed, the repetition
disturbed, the speech consists of nouns only, and verbs, of speech is impossible, impossible are the spontaneous
adjectives and prepositions are absent (agrammatism, writing and writing under dictation and the nomination.
“telegraphic style”. The repetition of speech, automat Transcortical sensory aphasia. The lesion involves
ic speech and reading aloud are disturbed similarly to most often the middle-temporal areas of the left hemi
the spontaneous speech. The understanding of the heard sphere that interrupts the connections between the sen
and read speech is preserved. sory and ideation centers. Only the linguistic level is
Transcortical motor aphasia. The lesion is in the disturbed, the verbal gnosis and praxis are preserved.
dorsolateral premotor and prefrontal cortex and in the The language disturbance impedes not only the under
supplementary motor field of the left hemisphere. The standing of the verbal messages of the others but also
language encoding is disturbed but the verbal articu the generation of own verbal messages. There is severe
lator praxis is preserved. The spontaneous speech is disorder of the semantic filling of the words (“aliena
non-fluent, laconic, with simplified grammar and vo tion of the meaning of the words”) and of the grammar
cabulary scarce, the speech initiative is also absent. The rules. The spontaneous speech is with fluent articulation
spontaneous writing is disturbed in similar way. The without phonemic defects, but with verbal paraphasias
monolog speech when the patient is asked to retell or (substitution of words), echolalia and almost completely
write something on a given topic or picture is the most not understandable. The nomination is disturbed (ano-
difficult task. The dialog speech is also scarce; the pa mia). The understanding of the verbal messages (heard
tient repeats words and phrases of the questions asked and read speech) is severe disturbed. The repetition of
speech is preserved but the repeating is
mechanic and without understanding.
The patient is able to read whole phrases
without understanding their meaning.
Conduction aphasia. It results of
interruption of the connections between
the sensory and motor centers of speech
- damage of fasciculus arcuatus - a
massive bundle of fibers travelling be
low gyrus supramarginalis and parietal
operculum and connecting the frontal
and temporal lobes. The most impres
sive defect is the disturbed repetition
of speech while the spontaneous speech
is relatively preserved with infrequent
8. Higher cortical functions 59
literal (phonemic) paraphasias. The perceiving of the Alexias are divided to agnostic, peripheral (impaired
heard and read speech is preserved but the patient is un analysis in the visual system) and aphasic, central (im
able to repeat newly heard word or phrase. In both writ paired analysis at linguistic level).
ten and oral speech are observed literal (graphemic) par 1. The peripheral alexias are subdivided to:
aphasias. The perception of the heard and read speech • Alexia with neglect syndrome. Results of ina
is preserved but the patient is unable to repeat newly bility for processing the visual information in one half
heard words and phrases. There are literal (graphemic) of the visual field, most often the left one. The ignoring
paraphasias in both written and oral speech. of the beginning of the row or of the word in the left half
Anomic aphasia (amnesic aphasia, anomia). It is of the visual field is typical.
characterized by disturbance in the expressive speech • Pure alexia (alexia without agraphia, pure ver
as the patient is unable to recall the necessary word, bal blindness). The ability to identify the letters (graph
especially names of objects. He compensates the forgot emic gnosis) and reading are impaired. The patient un
ten name by a descriptive explanation of the object (for derstands auditory perceived speech, speaks without ar
example when displaying a pencil he says “the object ticulator and linguistic mistakes, writes spontaneously
we wrote with”). The suggestion of the first syllable of and under dictation but doesn’t understand the text he
the word is often helpful. This expressive aphasia is op is reading or even the text he just wrote. He is unable
posing to the telegraph style, where the speech of the to retype texts and reproduces the letters with all their
patient consists only of nouns without using verbs. The details (“slavish coping”). In pure alexia there is simul
style in anomic aphasia is verbose and circumstantial. taneous impairment of the primary visual cortex in the
The spontaneous writing is disturbed similarly to the left hemisphere and the posterior part of corpus callo
spontaneous speech. The understanding of the read sum with disconnection between the incoming visual
and heard speech, the repetition of speech, the reading information and the linguistic zone.
aloud and the writing under dictation are preserved. The • Attentive alexia. The identification of the let
lesion is located in the posterior temporal parts of the ters is preserved. Disturbed are the spatial encoding of
left hemisphere and can involve the occipital-temporal the letters and their grouping in words.
fields. Anomia is observed as a separate disorder or as a 2. The central alexias result of linguistic impairment
part of other aphasic syndromes. and are observed as a part of the aphasic syndromes.
Mixed transcortical aphasia. It is observed in They are subdivided to:
diffuse lesions of the anterior and posterior associa • Superficial alexia is inability to read aloud un
tive fields of the left hemisphere with preservation of usual written words due to difficulties in accession to
the centers of Broca and Wernicke. The encoding and the semantic image of the letters.
decoding speech functions are deeply disturbed, but • Phonologic alexia is inability to read aloud
the verbal gnosis and verbal praxis are preserved. The new, unfamiliar words when the reading and under
spontaneous speech and spontaneous writing consist standing of familiar words is preserved.
of single stereotype and meaningless phrases. The un • Deep alexia is inability to read both unfamiliar
derstanding of the heard and read speech is severely and familiar words
impaired. The repetition of speech is completely pre Agraphia is impairment of the acquired via educa
served. The patient repeats in echolalic manner the tion and training writing ability. Similarly to alexia it is
heard phrases without understanding them. divided to peripheral (non-aphasic) and central (apha
Global aphasia. It results of extensive lesions in sic).
volving the anterior and posterior linguistic zones, in l.The peripheral agraphia is subdivided to:
cluding centers of Broca and Wernicke. The verbal gno • Alexia with agraphia. This is acquired im
sis and praxis and the language encoding and decoding pairment of the ability of reading and writing, possi
are both disturbed. The spontaneous speech is almost ble resulting of impairment of the working memory.
impossible to degree of mutism or the speech consists The difficulties in identification of the letters lead to
of particular verbal stereotypes (speech embolus). The disturbed reading and writing. The reading is slowly,
repetition of speech and nomination are also impossible. letter by letter, the meaning of the read often remains
The understanding of the heard or read speech is severe not understood. The writing, irrespective handwriting
ly impaired or impossible. In the process of recovery or typewriting, is also disturbed. The lesion is in the left
the global aphasia slowly transforms in cortical motor gyrus angularis.
aphasia. • Pure agraphia. Results of impairment in the
Disorders of the written speech. Alexias and selection of the appropriate words and their proper ar
agraphias. ranging. Both spontaneous writing and writing under
The reading and writing develop lately in school age dictation are disturbed, literal paraphasias are present.
on the basis of the developed speech via education and There is reversal and adding of letters, combinations of
training. The alexias and agraphias are acquired dis small and capital letters. The writing is fluent and skill
orders in the understanding of a written text and in the ful and the written letters are very well shaped.
ability of writing. Dislexia and disgraphia are disorders • Apraxic agraphia (apraxia o f writing). Results
in the development of the ability of reading and writing. of impaired writing of the letters that are deformed and
60 / General neurology
unrecognizable. The writing is slowly, clumsy, uncer The examination of the patients with aphasia con
tainly. It results of disconnection between the left fron tains the following steps:
tal and parietal cortical centers or between the left fron 1. Spontaneous oral speech: fluency of the artic
tal cortex and the basal ganglia. ulation, phrases length, presence of literal and verbal
2. The central agraphias are connected with linguisparaphasias, neologism, perseverations, agrammatism,
tic impairments and are associated with the aphasic syn paragrammatism.
dromes. They are subdivided to: 2. Repetition of speech - of single words, short and
• Superficial agraphia (lexical agraphia). The long sentences, meaningful and meaningless phrases
ability to write familiar words by unusual orthography 3. Automated speech - counting the days of the
is impaired. week, the months of the year
• Phonologic agraphia. The ability of writing 4. Nomination -.o f objects, parts of the body, colors,
of meaningless combinations of sounds under dictation figures
is disturbed due to difficulties in transforming of pho 5. Assessment of the understanding: identification of
nemes into graphemes. the heard words, understanding of questions, executing
• Deep agraphia. There is severe impairment of of commands
the writing under dictation of familiar and unfamiliar 6. Writing - spontaneous, under dictation, retyping
words due to impairment in the phonemic-graphemic 7. Reading - reading aloud, understanding of the
transforming. read words and sentences
9. Impairments of consciousness / 61
9. IMPAIRMENTS OF CONSCIOUSNESS
Consciousness is the highest function of the human Table 9.1 G lasgow -L iege com a scale
brain and results of the physiologic activity of the cer Examined functions Points
ebral cortex. The wakefulness, clarity of consciousness Eyes opening
and the degree of active attention are defined by the ac • Spontaneously 4
tivity of upper, mesencephalic-dyencephalic parts of the • To verbal command 3
reticular formation - the so-called ascending reticular • To painful stimulation 2
activating system (ARAS). ARAS receives collaterals • Absence of response 1
from all ascending sensory systems that after shifting Verbal response
in the non-specific thalamic nuclei send diffuse projec • Oriented 5
tions to the cerebral cortex. • Confused 4
The impairments of the consciousness are divided • Inadequate 3
into two types: qualitative and quantitative. There is im • Incomprehensible 2
pairment in the clarity of consciousness with psychotic • Absence of response 1
production in qualitative impairments o f consciousness Motor response
like delirium and amentia. The patients with delirium • Executes commands 6
have impairment of consciousness combined with psy • Localized response - the limbs per- 5
chomotor agitation, anxiety, incoherent thinking, hallu form purposeful movements to avoid
cinations, and illusions. The patients are with preserved harmful stimuli
awareness of their own personality (autopsychic aware • Withdrawal from painful stimuli 4
ness), but the awareness for time and place (allopsychic • Abnormal flexion 3
awareness) is disturbed. The quantitative impairments • Abnormal extension 2
o f consciousness are divided according to the clarity of • Absence of response 1
consciousness into: obnubilation, somnolence, sopor Brainstem reflexes
and coma. In obnubilation the verbal contact with the • Frontal-orbicular 5
patient is preserved. The patient looks overtyred, the • Vertical oculocephalic 4
concentration and attention are impaired, and sometimes • Pupillary reflex to light 3
there is disorientation for time and place. In somnolence • Horizontal oculocephalic 2
the verbal contact is severely impaired. The patient is • Oculocardial 1
disorientated - for himself and for time and place. In • Absence of response 0
absence of external stimulation the patient falls asleep.
The verbal contact with patient in sopor is impossible. Wakeful coma (coma vigile). It results of diffuse
In response of strong irritation the patient is opening anoxic-ischemic lesions of the cerebral cortex and mid
his eyes, but is unable to execute any commands. Coma brain - state following clinical death, poisoning, drown
is characterized by total unconsciousness. The patient ing, cranial traumatic injury. All motor functions are
cannot be awaken by any kind of stimulation, includ suppressed, including speech, gestures, mimic. Despite
ing painful. He doesn’t give motor and verbal respons of a certain degree of wakefulness, replaced by a state
es. Three stages of coma are differentiated: superficial, of sleep, there is complete absence of psychic and mo
deep and coma depasse. For quantitative assessment of tor initiative. The patient does not perform volitional
the depth of coma is used Glasgow coma scale. It as movements, doesn’t speak, and does not react to sensory
sess the eye opening, motor and verbal responses. In stimulation. He is opening his eyes spontaneously and
the combined scale of Glasgow-Liege assessment ol the in milder cases looks as if he is following with eyes the
brainstem reflexes is added - table 9.1. The maximal moving objects. The defensive reflexes are absent; there
number of points is 20. The critical prognostic thresh are some primitive reflexes like chewing, swallowing,
old is 8 points. When the number of points is below 8, grasping reflex.
the prognosis for recovery is poor. The examination ot Apallic syndrome. It is a synonymous to wakeful
the brain bioelectric activity (EEG) is of particular im coma. It is connected with diffuse lesion of the cerebral
portance for assessment of the depth of coma and the cortex with suspension of her functions. Most often it
prognosis of recovery. The normal a-wave activity, typ is observed in severe cranial traumatic injury and after
ical for the state of wakefulness, in comatose patients recovery from comatose state. Similarly to the wakeful
is replaced by high-voltage slow-wave activity. With a coma the patient is with opened eyes, sometimes he is
deepening of coma the amplitudes of the waves slowly following the objects with his eyes, but the contact with
decrease to the stage of bioelectrical silence in the state him is completely impossible, he does not respond to any
kind of commands, the emotional reactions are absent.
of brain death.
62 / General neurology
The muscle tone is increased, sometimes to the stage of with irreversible brain damage and suspension the brain
decorticated rigidity. In response to painful stimulation functions, including the functions of the brainstem, but
primitive defensive movements can be observed. artificial maintenance of respiratory and cardiac func
Minimally conscious state. The patient demon tions is possible. According to the modern understand
strates signs of relative perception of himself and the ing the death of the individual is equated with brain
environment. He is able to perform elementary com death and the time of death - with complete suspension
mands and to respond by mimic and gestures for “yes” of the brain functions but not with the cardiac arrest that
and “no”. He is able to perform simple purposeful ac occurs later. The establishment of brain death is carried
tions and reacts to stimulation. out by:
Akinetic mutism (abulia). It represents a variant of 1. Absence of cortical functions - spontaneous
the minimally conscious state. Akinetic mutism results movements and response to visual, auditory and senso
of bilateral frontal-orbital lesions and lesions of ARAS ry stimulation.
projections to the frontal lobe - for example bilateral 2. Complete loss of brainstem functions - eye move
infarction in the basin of the anterior cerebral artery, bi ments, corneal, pharyngeal, vestibular reflexes; the pu
lateral thalamic infarction, tumors of the third ventricle. pils are fixed and nonreactive.
The patient appears vigil. He is with opened eyes and 3. Lack of restoration of the spontaneous respiratory
can fixate and follow objects with eyes, but he doesn’t functions after repeated administration of oxygen and
show any volitional activity. The spontaneous speech is carbon dioxide.
absent except in response of strong external stimuli. 4. Persisting hypothermia (below 25°)
Locked-in syndrome. The syndrome is connected 5. Isoelectric line of EEG.
with lesion of the basal parts of pons, where the motor 6. Exclusion of reversible reasons for apnoic coma.
pathways are travelling. The clinical presentation of the
syndrome is with central quadriplegia and pseudobul
bar palsy. The consciousness is preserved, as well as
the sensory and cognitive functions because the pontine
tegmentum (where the sensory pathways and ARAS
are situated) is undamaged. The patient is able to see,
hear and understand everything, but he can’t speak and
perform any kind of movement with his head and body.
The only possible movements are the vertical eye move
ments and sometimes the eyes closure and thus the pa
tient communicates with the others.
Psychogenic coma. The psychogenic coma is ob
served in conversion-dissociative disorders (hysteria)
and results of psychic trauma. Most often an episodic
clouding of the consciousness is observed than psycho
genic coma. The patient seems unconscious; he lies mo
tionless with eyes closed and does not react to external
stimuli. Sometimes convulsions are present but they do
not have the typical sequence of epileptic seizures. The
patient is not cyanotic; he does not bit his tongue; the
pupil reaction to light and vital functions are preserved.
The patient resists to the attempts to open his eyes and
actively clinches his eyelids.
Brain death. The biologic death is characterized
by irreversible arrest of respiratory and cardiac activi
ty, complete loss of all CNS functions with subsequent Fig.9.1. Flow chart for the clinical diagnosis of brain death
death of the organism. The brain death is connected (Wiebers)
10. Methods of examination of the nervous system / 63
pramaximal nerve stimulation with low frequency (2-5 trast-filled spinal canal. Computed tomography of brain
Hz) and is used for identification of Myasthenia gravis. and spinal cord defines the density of the soft tissue
The high frequency stimulation (to 50 Hz) is used for structures by X-ray beam with slice thickness 10 mm. It
registration of facilitation in presynaptic block (Ea- registers hyperintense (bright), hypointense (dark) and
ton-Lambert syndrome). For anterior horn damage are isointense (confluent with the brain parenchyma) imag
typical “giant” MAPs and for primary muscle damage es. The examination is used for diagnosis of tumors, in
- polyphasic slow-amplitude MAP. farction, hemorrhage, contusion, congenital anomalies.
Sometimes CT-assisted myelography is used. Spiral CT
gives three dimensional reconstructive images of the
10.5. ULTRASOUND EXAMINATIONS brain blood vessels.
Magnetic resonance tomography is based on prin
They are used for noninvasive examination of the ciple of strong magnetic field application after which
extra- and intracranial blood vessels. Doppler sonogra the relaxation times of protons are registered. In T1
phy registers the ultrasound wave frequencies that are sequences there is strong signal from the brain paren
changing proportionally to the velocity of the erythro chyma and low signal from the CSF, in T2 sequences
cytes in the blood flow. Thus are evaluated the veloc just the opposite relations exist. MRI gives a very good
ity and direction of the blood flow in the extracranial imaging of the demyelinative lesions, tumors, infarc
arteries and are found stenoses and thromboses of the tions, traumas, brain edema. MR-angiography without
arteries. Transcranial Doppler sonography evaluates the contrast matter application displays the arterial and
collateral circulation in circle of Willis, the vasospasm venous blood vessels. Positron emission tomography
after subarachnoid hemorrhage, presence of arterio (PET) uses radioligands emitting positrons and is ap
venous malformations, sinocarotid fistulas, etc. Duplex plied for quantitative evaluation of the brain blood flow
scanning evaluates the structure changes in the blood and metabolism of oxygen, carbon dioxide, glucose, etc.
vessel walls (plaques, stenoses, and calcification) and Single proton emission computed tomography (SPECT)
the hemodynamic of the blood flow (direction, turbu uses radioligands emitting single photons. It is applied
lence, velocity). for diagnostic of Parkinson’s disease, Essential tremor,
Parkinson-plus syndromes, early changes in the region
al blood flow.
10.6. NEUROIMAGING
Peripheral nervous system (PNS) includes the cra 1-2 days with development of facial palsy that is often
nial nerves and spinal nerves. The classification of PNS preceded by retroauricular pain. The mimic muscles
disorders is based predominantly on the localization of palsy is characterized by smoothing of the skin folds of
the pathologic processes. It can involve different parts of the forehead, wider eye slit (lagophthalm), drooping of
the PNS - anterior or posterior roots (radiculitis), spinal the mouth angle and smoothing of the nasolabial fold.
and cranial ganglia (ganglionitis), plexuses (plexitis), The facial asymmetry is more obvious in lifting of the
peripheral nerves (neuritis), symmetrical involvement eyebrow and closing of the eye when the eyeball is turn
of many peripheral nerves (polyneuritis), involvement ing upwards and the weakness of m. orbicularis ocu-
at a different time of many peripheral nerves (multiple li makes visible wide area of sclera (sign o f Bell). The
mononeuritis). These terms are used when the disor movement of the eyeball upwards is a normal synergic
der of the peripheral nerves has inflammatory nature motion when closing the eye. When the patient is asked
- acute and chronic inflammatory demyelinating poly to show his teeth the mouth angle stays lower. The pa
radiculoneuritis, diphtheric polyneuritis, and Herpes tient is unable to whistle and to inflate cheeks. As the
zoster ganglionitis. In most of the disorders the etiology patient is eating the liquids leak out from the involved
is non-inflammatory (toxic, drug-induced, metabolic, mouth corner and the food is retained between the teeth
deficiency-associated, ischemic, traumatic, and neo and paralyzed cheek.
plastic). The more precise terms for these disorders are: The damage of the nerve in the bone canal above
radiculopathy, plexopathy, neuropathy, polyneuropathy. chorda tympani leads to gustatory impairment (ageu
When the disorder is manifested by pain symptoms only sia) in the anterior two-thirds of the tongue and reduced
that has relapsing character and negative symptoms are saliva secretion as chorda tympani contains fibers for
absent, the term neuralgia is used. saliva secretion and taste. The lesion of the nerve above
The patomorphologic changes of the peripher the division of n. stapedius adds increased perception of
al nerves are of different types: axonal degeneration sounds (hyperacusis) in the affected ear. When gangli
(primary involvement of the axon), segmental demy- on geniculi is affected (by Herpes zoster infection) pain
elination (primary damage of the myelin shelf), and behind the ear and herpetic rash of the external audito
combined axonal and demyelinating disorder. Clini ry canal are observed (syndrome o f Ramsey-Hunt). The
cal symptoms of damage of the peripheral and cranial damage of the nerve above the division of n. petrosus
nerves include positive and negative sensory symptoms, superficialis major leads to reduced lacrimation of the
motor and autonomous disturbances, as the peripheral affected eye. The lesions of the nerve in meatus acusti-
nerves are mixed and contain sensory, motor and auton cus internus are connected with simultaneous involve
omous fibers. ment of n. statoacusticus (tinnitus, reduces hearing,
vertigo). The lesions in ponto-cerebellar angle lead to
involvement of n. trigeminus and more seldom of IX and
11.1. NEURITIS OF NERVUS FACIA X cranial nerves.
LIS (FACIAL NERVE PALSY, BELL’S Diagnosis. Diagnostic confirmation is obtained
PALSY) from the neurological examination. It reveals damage of
the facial nerve and absence of corneal and conjuncti
The most common reason for facial palsy is the neu val reflexes on the affected side. Signs of other cranial
ritis of n. facialis. The annual morbidity is 10-40/100 nerve involvement are absent and otherwise the neuro
000. It is more frequent during the cold seasons, in logical examination is normal. In suspicion of symp
pregnant women and in people suffering from diabe tomatic neuropathy the following examinations must
tes. The etiology of facial nerve palsy is not completely be performed: CSF, sedimentation rate, examination
clear. Recently the viral etiology is accepted. This theo for Lyme disease, for Diabetes, MR angiography. The
ry is supported by the isolation of Herpes simplex type diagnosis and the stage of the lesion are confirmed the
I genome in endoneurial liquid and in geniculate gangli EMG - presence of denervation after the 10 day is typi
on. The greater morbidity after head exposure to cold cal for axonal degeneration and is prognostic for slower
impact is connected with development of inflammatory recovery.
edema and compressive ischemic changes in the narrow Differential diagnosis. Neuritis of the facial nerve
and long facial nerve canal. must be differentiated by nuclear lesion that is also with
Clinical features. The onset is relatively acute in peripheral character but its presentation is with motor
11. Diseases of the peripheral nervous system / 67
signs only. The nuclear type facial palsy, especially in examinations. The highly sensitive MRT and MR an
children, is always suspicious for pontine form of polio giography may reveal presence of the so-called "vas-
myelitis. The peripheral facial palsy must be differenti cular-nervous conflict ” caused by compression of the
ated from central palsy. The later is observed most often nerve in close proximity to brainstem by atherosclerotic
in patients with ischemic infarctions and involves only changed blood vessels (cerebellar arteries, elongation of
the contralateral lower facial half combined with hemi the basilar artery) or parapontine veins. Different rea
plegia and paresis of tongue on the opposite side. The sons may lead to symptomatic neuralgia - tumors of the
facial nerve may be affected from different pathologic ponto-cerebellar angle (neurinoma of n. statoacusticus,
processes along its way - tumors of the parotid gland, meningioma, angioma), aneurism of basilar artery, de
sarcoidosis, traumatic and intraoperative injuries, otitis, myelinating plaques in pons, focal infections of dental
mastoiditis and other inflammatory processes, neoplas origin, inflammatory processes involving sinuses, nasal
tic invasion, fractures of the temporal bone, tumors of and oral cavities, and traumas.
ponto-cerebellar angle (neurinomas of n. statoacusticus, The pathogenesis is still not fully understood. It
meningeomas), vascular malformations, aneurism, neo is accepted that the focal demyelination caused by the
plastic infiltration of menings, pontine gliomas, demye- nerve compression creates an opportunity to occur ab
linative plaques in Multiple sclerosis. Bilateral neuritis normal ectopic impulses and transmission of the im
of the facial nerve is observed in the course of acute pulses from tactile to pain conducting nerve fibers. This
demyelinating inflammatory polyneuropathy, Lyme dis explains the provoking of pain by non-painful stimula
ease, basal meningitis. The relapsing facial palsy com tion of the trigger zones. Not only peripheral but central
bined with facial edema, involving especially the upper mechanisms participate also in the appearance of the
lip, and lingua plicata is typical presentation of the syn neuropathic pain. This fact explains the positive effect
drome of Melkersson-Rosenthal. of anticonvulsants and the lack of efficacy from risot-
The prognosis of recovery is very good in 80% of omy of the nerve root and after nerve decompression.
the cases. Complete or partial recovery is observed in Clinical features. Typical are the relapses of neu
1-6 months. Early complication is keratitis due to in ralgic pain, coming suddenly without harbingers, last
complete eye closure. Sometimes contractures of the ing few seconds or minute and repeated many times.
paretic facial muscles are observed with narrowing of The pain is extremely strong and may cause reflex ton
the eye slit, the mouth corner is elevated and the na- ic or clonic contraction of the facial muscles (Tic dou
so-labial fold is deeper. Due to improper regeneration of loureux, described by N. Andre in 1756). The relapses
the nerve fibers occur pathological synkinesias - the eye are coming spontaneously or are provoked by emotional
closure leads the elevation of the mouth corner and the excitement, mimics, speaking, chewing, swallowing,
teeth showing leads to closing of the eye. Rarely patho and touch of certain facial area (trigger zone). With the
logical gusto-lacrimal reflex is observed - lacrimation end of the relapse occurs refractory period lasting sev
during eating (“crocodile tears syndrome”). It results of eral minutes during which the provoking factors can’t
improper regeneration of saliva secretion fibers towards evoke neuralgic pain. As the time passes the relapses
n. petrosus superficialis major. become more frequent and may involve the other nerve
Treatment starts with steroids (1 mg/kg Methyl- branches.
prednisolon for 5 days with consecutive dose tapering). Herpes zoster ophthalmicus results from Herpes
During the acute period anti-edema treatment with 10% zoster ganglionitis. The disorder is connected with la
Mannitol is recommended. Sometimes Acyclovir 2000 tent virus reactivation in patients with immunosuppres
mg for 10 days is prescribed. Artificial tears and oph sion. The neuralgic pain is in the area supplied by the
thalmic unguents are often useful. Massage and gym ophthalmic branch and is accompanied by herpetic rash.
nastic of the affected mimic muscles is recommended. When the conjunctiva is involved a possibility for devel
Surgical decompression of the facial nerve canal doesn’t opment of ulcerous keratitis and decreased vision exist.
prove its efficacy. The diagnosis of trigeminal neuralgia is based on
the typical clinical presentation - series of pain par
oxysms with clear onset and end, distribution of pain
11.2. TRIGEMINAL NEURALGIA in the areas innervated by the trigeminal nerve - most
(NEURALGIA N. TRIGEMINI) often its II and III branches. The examination reveals
hyperesthesia and pain in the points of Valleix for the
It is characterized by presence of strong relapsing involved nerve branch - foramen supraorbitale, fora
shooting and piercing pain in the area of innervation of men infraorbitale, and foramen mentale. The younger
the trigeminal nerve and mainly in the areas supplied by age, the presence of pain in the area of I nerve division
its maxillar and mandibular branches. Trigeminal neu accompanied by hypesthesia, absent corneal and con
ralgia is a frequent disorder with morbidity 5-6/100 000. junctival reflexes and weakness of the masticatory mus
It is observed in adults and elderly people and is more cles is often associated with symptomatic neuralgia and
frequent in women, aged over 50. trigeminal neuropathy. For diagnosis establishment is
Etiology. Most often the etiology remains un recommended MR angiography, exclusion of neoplastic
clear (essential neuralgia) prior to the neuroimaging or inflammatory involvement of the nerve, cranial basis,
68 / Clinical Neurology
(vascular pain), stomatalgia (oral cavity pain), dental- for innervation of the lacrimal glands and the glands
gia, glossalgia, etc. of the nasal cavity and soft palate and with vasodilator
The headache generally is divided to primary and fibers; 2) sympathetic fibers coming from the carotid ar
secondary. The primary headache is not induced by an tery via n. petrosus profundus; 3) sensory fibers for the
other disorder, but the secondary one is a symptom of palate and upper jaw (nn. pterygopalatini). The disorder
another disease. The group of the primary headaches is more frequent in women after the age of 40 and with
includes migraine, tension headache and cluster head medical history for long lasting maxillar and sphenoid
ache. The headache has diffuse character and specific sinusitis. The pain starts from the nasal basis and orbit
features in tumors and other space occupying process and spreads towards the upper jaw, palate, pharynx, oc
es, in patients with arterial hypertension, subarachnoid cipital and cervical regions and shoulder. The pain has
hemorrhage, and psychogenic disorders. The pain can burning and continuous character, with relapsing wors
have focal character - in glaucoma, refraction abnor ening. Usually vasomotor symptoms are also observed -
malities, sinusitis, myeloma, etc. In most of the cases the lacrimation, nasal secretion, and saliva hypersecretion.
pain syndromes in the area of head have typical clinical In difference of the trigeminal neuralgia the pain arises
features and are easily diagnosed. In rare cases the pain spontaneously, its intensity grows rapidly and lasts for
has atypical characteristics, hasn’t relapsing-neuralgic several hours. The examination reveals pain in palpation
features and is accompanied by vegetative symptoms. of the internal eye corner and proc. mastoideus; hypes-
thesia and dysesthesia of the palate, gums and cheeks.
11.5.1. Facial pain (Prosopalgia, Facialgia). Differential diagnosis is made with trigeminal neural
Facial sympathalgia gia, nasociliar neuralgia and migraine. The treatment
includes remediation of the infection of the surrounding
The most frequent pain disorders in the facial area tissues, anti-neuralgic drugs, NSAIs, endonasal block
are the so-called facial sympathalgias. They arise as a ade with novocaine, physiotherapy.
result of irritation and damage of the sympathetic fib Neuralgia of n. Vidii. (Syndrome of Weill). The
ers, and sympathetic and parasympathetic ganglia in the syndrome is described in 1929. It results of n. ptery-
area of the head. The diffuse, poorly localized pain is goideus damage caused by sinusitis of the neighboring
a leading symptom in the clinical picture. The pain is sphenoid sinus. The nerve consists of the parasympa
more pronounced during the night and is accompanied thetic n. petrosus major and sympathetic n. petrosus
by vegetative, vascular and trophic changes - facial ede profundus coming from the common carotid artery
ma, excessive lacrimation, rhinorrhea. The localization plexus. N. pterygoideus travels towards gangl. pterygo-
of the pain and numbness does not correspond to the paltinum. The disorder manifests itself clinically with
anatomy of the sensory innervation. The etiology of the relapsing pain in the area of orbit, nose, teeth, ear, face,
sympathalgias is disputable - general or focal inflam neck and shoulder and is accompanied by increased lac
matory processes, neoplastic and other pathologic pro rimation and rhinorrhea.
cesses neighboring the vegetative structures and blood Neuralgia of n. intermedius. The disease is charac
vessels. These disorders are more common in men. Rel terized by pain deep in the ear with irradiation towards
atively more frequent pain syndromes are the syndrome the face, nose, and palate. Vasomotor and secretory
of Charlin and the syndrome of Sluder that sometimes symptoms also occur.
are combined by common erytroprosopalgia. Sympathetic-trigeminal syndrome of Raeder. The
Neuralgia of n. nasociliaris (Neuralgia n. naso- disorder combines neuralgia of the first division of the
ciliaris, syndrome of C. Charlin). The disorder is trigeminal nerve and sympathetic negative symptoms.
described in 1931. The neuralgia results of nasociliar Its main clinical features are strong frontal and orbital
nerve affection which is a division of n. ophthalmicus. pain, reddening of the conjunctiva, increased lacrima
Gangion ciliare that contains sympathetic and parasym tion, and myosis, ptosis and enophthalmos (Claude Ber-
pathetic fibers is also involved. The strong pain encom nard-Horner syndrome). It is evoked by pathologic pro
passes the orbit and the corresponding half of the nose. cesses in the middle cranial fossa where the sympathetic
The pain is permanent, with relapsing worsening, espe fibers of the carotid plexus transfer to n. ophthalmicus.
cially in the night and disturbs the night sleep. It is ac Neuralgia of Jacobson’s nerve (syndrome of plexus
companied by lacrimation and increased nasal secretion tympanicus, syndrome of Reichert). N. tympanicus
on the affected side. The palpation of the internal eye is a division of IX cranial nerve containing parasym
corner is particularly painful. The disorder must be dif pathetic fibers. These fibers together with sympathetic
ferentiated by frontal and ethmoid sinusitis and Sluder’s fibers from the carotid plexus form plexus tympanicus
syndrome. that innervates the ear drum, Eustachian tube and proc.
Neuralgia of ganglion pterygopalatinum (Neural mastoideus. From the plexus starts n. petrosus minor
gia gangl. pterygopalatini, Syndrome of G. Sluder). that travels to gangl. oticum. This connection between
The disorder is described in 1908. Ganglion pterygo ganglion inferior (IX cranial nerve) and gangl. oticum
palatinum has rich anastomoses with: 1) parasympathet (V cranial nerve) is termed Jacobson’s anastomosis. It
ic fibers coming from n. intermedius via n. petrosus ma is damaged by ear inflammation. Jacobson’s neuralgia
jor and from n. glossopharyngeus via n. petrosus minor occurs with strong pain deep in the ear, reddening and
11. Diseases of the peripheral nervous system / 71
edema of the external auditory canal, nasal hyperemia, followed by degenerative changes of the joint. Dull
burning sensation and edema of the face. pains in the joint and corresponding ear are observed.
Syndrome of n. auriculotemporalis (Auricu- They irradiate to the occipital and temporal zones and
lo-temporal hyperhidrosis of Frey) sometimes involve face and teeth. The pain intensifies
N. auriculotemporalis is a branch of n. mandibularis with chewing.
and contains sympathetic fibers coming from gangl. ot- Referred pains in the area of the head, face and oral
icum and parasympathetic fibers from n. glossopharyn- cavity are observed in the course of pathologic process
geus. The clinical features of the syndrome are hyperhi es in the thoracic and abdominal cavities. The pain af-
drosis, reddening and burning behind the ear at mealtime. ferents are transferred by n. vagus, n. phrenicus and the
The hyperhidrosis occurs at the sight of the food or even sympathetic fibers to nucl. tractus spinalis n. trigemini
at the idea of food. It is caused by irritation of the auton and are give projections to the cutaneous areas innervat
omous fibers of n. auriculotemporalis from the parotid ed by n.trigeminus.
gland at mealtime or another possible reason is gustatory Facial angioneuralgia, facial sympathalgia, facial
irritation of the root of the tongue that is transferred via vasalgia. These pains have vascular, pulsating charac
IX cranial nerve to n. auriculotemporalis. ter. They are localized in the course of the blood vessels,
Syndrome of Gradenigo. The syndrome is ob and increase in vessel’s palpation. The pain has mod
served in patients with mastoiditis and involvement of erate intensity, and sometimes relapsing worsening. It
the neighboring apical part of the pyramidal bone by has seasonal frequency and often starts in the night.
the inflammation. N. abducens and n. ophthalmicus are Sometimes the pain involves orbit, nose, cervical area,
affected. Headache, orbital pain and convergent strabis shoulders and arms. Autonomous symptoms are often
mus are observed. observed - facial and conjunctiva hyperemia, saliva
Syndrome of Tolosa-Hunt. The orbit and the fron tion. The pathogenesis is connected with blood vessel
totemporal region are affected by strong pain. Ptosis, spasm, following ischemic changes of the blood vessel
double vision and strabismus due to the damage of n. wall and in the blood supply of the tissues. The most
ophthalmicus and the oculomotor nerves are observed. common causes of the facial angioneuralgia are tempo
The syndrome is caused by idiopathic granulomatous ral arteriitis of Horton, and abnormalities in a. maxil-
inflammation involving the retroorbital space and sinus laries externa and a. facialis. The atherosclerosis of the
cavernosus. The good therapeutic efficacy of steroid cervical and head blood vessels, arterial hypertension,
treatment confirms the diagnosis. aneurisms of the anterior cerebral artery are main rea
Syndrome of Trotter. The syndrome results of hy sons for arising of the disorder. The differential diagno
pertrophy of the mucous membrane of nasopharynx, sis includes as follows: otitis, sinusitis, glaucoma, and
which sometimes is with neoplastic changes. Strong fa migraine. The treatment is with vasodilators, for exam
cial pain, pain in the jaws, tongue and ear are observed. ple calcium channel blockers.
Paresis of the soft palate, decreased hearing and trismus Temporal arteriitis. This is granulomatous inflam
develop. mation of the large and middle extracranial branches of
Syndrome of the first cervical sympathetic gan the carotid artery. The most typical finding is the in
glion. The injury of this ganglion is connected with volvement of the temporal artery which is thickened,
appearance of pain (sympathalgia) in the ipsilateral curving and extremely painful. The visual symptoms
cervical half, in the head, eye, and face, with sweating are often observed and danger of visual loss exists. Typ
and pilomotor reflex loss and with parallel excessive ical combinations are with polymyalgia and increased
lacrimation and nasal secretion due to parasympathet sedimentation rate. The therapeutic response to steroid
ic irritation. Sometimes transient positive sympathetic treatment is very good.
syndrome is observed - lagophthalmos, mydriasis, ex Carotidynia. The disorder is described in 1927
ophthalmos (syndrome of Pourfour du Petit) that can be as a special type of neuralgia which can be evoked by
followed by negative sympathetic syndrome - ptosis, pressure upon the common carotid artery close to its
miosis and enophthalmos (Claude Bernard-Horner syn bifurcation. The pain spreads towards the neck and the
drome). If gangl. stellatum is also involved the sympa homolateral facial half, including ear, gums, and teeth.
thetic pain spreads toward the arm and thoracic half on A symptomatic carotidynia is observed in the course of
the same side. Changes in the blood pressure and heart cranial arteriitis, dissecated aneurysm, tumors, inflam
rate also occur. matory processes and adhesions involving the carotid
Prosopalgia with causalgic character. The pain artery. Increased sensitivity of the carotid artery can
is strong, burning, and worsens by touch, light, strong be observed in migraine and cluster headache. A vari
noises. The skin is pale, cyanotic, and dry. The causal- ant of carotidynia in young people is described in 1967
gia represents sympathalgia resulting of partial damage by Roseman. The headache relapses continue up to 1-2
of nerves rich in sympathetic fibers. weeks. The pain increases in response of head move
Syndrome of Costen (Temporal-mandible ar ments, chewing, and swallow ing.
thralgia). The syndrome is observed in elder^ people Atypical facial pain.
with poorly fitting dentures that leads to painful over The exact reason for the facial pain remains unclear
stretch of the temporal-mandible joints and arthralgia, in some of patients despite of the performed examina
72 / Clinical Neurology
tions. These patients are usually young women with that is not connected with particular tooth or particu
medical history of depressive disorder. They usually de lar tooth damage. The pathogenesis is unclear, probably
scribe their pain as continuous, unbearably strong, lo neurogenic. It appears without clear reason or follows
calized in the angle between nose and cheeks. This type acute and strong psychic traumas, flu, arterial hyperten
of pain is best influenced by antidepressants. sion, stomach ulcer, etc. It occurs most often during the
cold moths of the year.
11.5.2. Pain in the oral cavity. Glossodynia, glossalgia. The pains in the tongue
(glossalgia) can result of inflammation of the tongue
The stomatalgia is a special unpleasant sense in the but they can have neurogenic origin also - in trigeminal
oral cavity like burning, numbness of the mucous mem neuralgia affecting the mandible nerve and its division
brane, dryness, bitter taste that disappears when eating. - n. lingualis the pain is localized in the anterior part
The reasons are different - gastro-intestinal disorders, of the tongue. In neuralgia of n. glossopharyngeus and
diabetes, endocrine diseases, neurotic states, etc. Sto n. laryngeus superior the pain is in the posterior part
matalgia with more intensive focal pain is observed of the tongue. The pain has relapsing character and is
in diseases of the oral cavity - pharyngitis, tonsillitis, provoked by irritation of the triggering zone. The pains
moniliasis, aphthaes, stomatitis, gingivitis, glossitis, in the tongue can have autonomous origin - irradiation
tonsilar herpetic infection, and herpetic infection of the from the superior cervical sympathetic ganglion. Pain
tongue, soft palate, and pharynx. with features of sympathalgia can result of the so called
Pains in the oral cavity are observed in neuralgias visceral crises in tabes dorsalis.
of n. glossopharyngeus and n. laryngeus superior. The The main feature of the glossodynia is glossalgia.
glossopharyngeal neuralgia starts from the tonsil and The disorder is with unknown etiology and pathogene
root of the tongue. It is evoked and worsens with swal sis. Under the term glossodynia are identified different
lowing and chewing and irradiates toward the ear, pal unpleasant sensations in the tongue, accompanied or not
ate, and mandible angle. The neuralgia o f n. laryngeus by undefined and inconstant pain. This positive sensory
superior starts from the larynx. It is also provoked and syndrome is characterized by senses of numbness, tin
worsens with eating and often spreads towards the ear. gling, burning, tearing, etc. These types of sensation are
Very typical is the painful point laterally of the thyroid with undefined localization. More frequently the disor
cartilage. The pain caused by glossopharyngeal neural der is observed in elderly people, in women in the age of
gia disappears after spreading a 10% cocaine solution menopause, and people who are emotionally unstable.
over the root of the tongue and tonsil in contrast to the Toxic impacts are also possible - poisoning with tetrae
neuralgia of n. laryngeus superior. thyl lead, abuse of drugs, etc. Very often the disorder is
The injuries of n. alveolaris inferior are observed with psychogenic induction and the strange sensations
in traumas, conduction anesthesia, manipulations and in the tongue are hypochondriacally interpreted.
operations, for example molar extraction. The main The objective examination does not reveal any abnor
clinical symptoms are pain, numbness or sensory loss malities in the tongue and oral cavity. The worsening of
in the lower teeth row, mouth and chin. the sensations to the degree of pain requires differenti
The injuries of nn. alveolares superiores in trau ation of neuralgias of n. trigeminus, of n. glossopharyn
mas, anesthesia, tooth extraction, maxillar sinusitis and geus, of n. laryngeus superior. The pain in glossody
sinus operation evoke pain, numbness, reduced sensa nia doesn’t have relapsing and shooting character, its
tion or sensory loss. localization doesn’t match to the anatomic innervation
The dentalgia represents a tooth ache, resulting areas, and triggering zones are absent. The diagnosis is
from deep caries, pulpit, and periodontal abscess, i.e. it proved by application of a strong anesthetic drug in the
is caused by direct affection of the tooth, but it also can painful areas which results in pain resolution. The later
result of trigeminal injury. The pain worsens by thermal phenomenon is not observed in cases of neuralgic pain.
irritations and tooth percussion. It has partially pulsat Of diagnostic value is the exclusion of other disorders
ing character and is stronger in the night. The most of of the tongue, nervous system and internal organs. In
ten cause of the dentalgia is pulpit. It is characterized by pernicious anemia or vitamin B12 deficiency state is ob
pain (sinalgia) projecting to the neighboring tooth on the served the so-called syndrome o f Plummer-Vinson - the
same side or to the corresponding tooth on the opposite tongue is with atrophic papillae and has raspberry color
side (dentate-dental sinalgia). In dentate-dermal sinal (Hunter’s glossitis). Tooth decay and gingivitis are de
gia the pain is projecting towards particular cutaneous veloping. The swallowing is painful and difficult; there
facial areas. The pathogenesis of dentalgia is connected is numbness in pharynx and esophagus. The psychogen
with sympathetic fibers irritation. ic origin of glossodynia is accepted only after exclusion
The trigem inal ascending neuritis may follow of disorders of the nervous system, internal organs and
tooth extraction or another manipulation in the oral focal oral cavity pathology.
cavity. Despite tooth ache there is decreased sensation The treatment is directed to replacement of the not
of the tongue and lower lip and weakness of masticator well fitting dentures, treatment of the newly revealed
muscles. teeth disease. Psychotherapy is useful. The patient is
The odontalgia is also special type of tooth ache advised to avoid smoking, to limit the usage of strong
____ 11- Diseases of the peripheral nervous system / 73
spices, to maintain oral hygiene. Sedatives, analgesics, statoacusticus affection that leads to tinnitus, vertgo
and hypnotics are prescribed. and hearing loss. The prognosis for recovery of the fa
cial palsy is poor.
In Herpes zoster infection of the glossophryngeal
11.6. GANGLIONITIS (HERPER ZOS nerve sensory ganglia the pain and herpetic rash are
TER) OF THE SENSORY GANGLIA distributed at the bottom of the oral cavity, pharynx,
soft palate and tonsils.
Herpes zoster infection is a frequent viral infection The cerebral angiitis and encephalitis are infrequent
of the nervous system. The morbidity is 305/1000 per complications of the cranial and cervical herpetic infec
year and has higher rates in elder people. It is charac tion observed mostly in patients with immunosuppres
terized by radicular pain, vesicle rash in the affected sive states. Rare complication of the thoracic herpes is
dermatomes and rarely segmental sensory or motor dis myelitis. Post herpetic neuralgia is observed in 1/10 of
turbances. The infection is caused by DNA virus - Vari the patients above the age of 40. The diagnosis is diffi
cella-zoster virus (VZV), which induces development of cult in patients with pain in whom herpetic rash does not
varicella in children - highly contagious disease, unlike develop (zoster sine herpes).
the herpetic ganglionitis which is a sporadic disorder. A therapy with Acyclovir 5x800 mg daily for 7 days
After the primary infection the vifus remains persisting is applied. In immunosuppressed patients and in pa
in the sensory ganglia in latent state. In reactivation tients with disseminated rash Acyclovir is prescribed i.
causes acute ganglionitis and migrates along the senso v. - 10 mg/kg for 10 days. VZ immunoglobulin (VZIG)
ry nerves to the skin and induces the herpetic cutaneous is also useful in such patients for prevention of herpet
rash. In some patients dorsal horns of the spinal cord ic dissemination. Other antiviral medications as Fam-
are involved with mild CSF pleocytosis and meninge cyclovir and Valacyclovir also take place in the thera
al reaction. The viral reactivation results of immune peutic course. It is disputable if the steroid treatment
deficiency states in elder people, lymph proliferative prevents development of post herpetic neuralgia but it
diseases, neoplasm, immunosuppressive treatment, and is contraindicated in patients with neoplasm and im
irradiation. munodeficiency states. For managing of the neuralgic
Several days before the rash onset, pain, numbness pain Amitriptylin, Carbamazepine, Gabapentine and
and increased sensitivity occur in the affected dermat Thiagabine are used.
ome. Sometimes the pain is so strong that the disorder
might be mistaken with pneumonia, cholecystitis, and
appendicitis. The typical cutaneous rash develops sev 11.7. CERVICAL RADICULOPATHY
eral days after the pain onset - vesicles on an erythe (RADICULOPATHIA CERVICALIS)
matous basis that are replaced by scabs in 5-10 days,
followed by mild cutaneous pigmentation. In rare cases The disease is caused by degenerative changes of the
the rash is severely expressed, confluence of the vesi spinal column (degenerative spondylopathy, spondy-
cles is observed or generalized rash develops (herpes loarthrosis) which develop with age. The genetic predis
multiplex). The strong pains and dysesthesias continue position, micro traumatic lesions of the spinal column,
1-4 weeks, but in some of the patients the pain persists obesity are also of particular importance. The changes
months and years (postherpetic neuralgia). Usually in glycosaminoglycans lead to reduction of water con
there is unilateral involvement of several neighboring tent, elasticity and height of the intervertebral disks.
spinal ganglia, or of ganglion Gasseri. Rarely the senso Subluxation and arthrosis of the intervertebral joints is
ry ganglia of VII, IX and X cranial nerves are affected. developing, and osteophytes are growing. The sudden
Most often the middle thoracic ganglia (Th5-Thl0) are movements and physical exertion create a predispo
involved with clinical signs of girdling thoracic pain. sition to disk protrusion in which annulus fibrosus is
The herpetic infection seldom affects the lumbar spinal pushed dorsally from nucleus pulposus. The rupture of
ganglia with pain irradiating to lower limbs. annulus fibrosus leads to displacement of nucleus pul
Herpes zoster ophthalmicus represents about 10-15% posus outside the disk - disk prolapse (herniation). The
of all cases with herpetic infection. It results of gangli disk herniation is subdivided in: subligamental (if lig.
on Gasseri ganglionitis. The pain and rash are distrib longitudinale post, is intact) and transligamental (if lig.
uted in the territory of n. ophthalmicus. The affection longitudinale post, is ruptured). Most often the disk her
of cornea and conjunctiva carries a risk of blindness. niation is in lateral direction because the weakest part of
Infrequent complications are oculomotor nerve palsy, lig. longitudinale post, is there. This leads to compres
cerebral vasculitis with following stroke and herpetic sion on the lower spinal root. More rarely the hernia
encephalitis. tion is in medial direction and causes spinal stenosis and
Herpes zoster oticum (syndrome o f Ramsey-Hunt) is compression on the dural sac.
caused by ganglionitis of ganglion geniculi. The pain The cervical spinal roots (C1-C8) are most often
and rash are situated behind the ear and in the exter compressed by lateral disk herniations, osteophytes,
nal auditory canal. The herpetic infection is most often and more rarely by luxations of the vertebrae caused
combined with facial neuritis and more seldom with n. by traumas (whiplash), by metastatic extradural turn-
74 / Clinical Neurology
ors, extramedullary tumors (neurinoma, meningeoma), deviation of the arm can exist. C6 root is more often
etc. Usually the spondyloarthrosis is most pronounced involved; the pain covers the lateral surface of the arm
at C5-C6 and C6-C7 levels and respectively C6 and C7 and irradiates to the thumb and index finger; there is
spinal roots are most frequently affected - fig. 11.1 and muscle weakness in flexion at the elbow. The most fre
fig. 11.2. quent is the radiculopathy of C l root - the pain covers
The cervical radiculopathy manifests itself by neck the dorsal aspect of the arm and spread towards II and
pain, spreading to scapula, shoulder and arm, numbness III finger; there is weakness in the forearm extension.
of the arm and fingers, decreased muscle strength of C8 root emerges between C8 and Thl vertebrae. The
the arm. The pain is with causalgic character, amplifies pain involves the medial aspect of the arm and irradi
when sneezing, coughing and with neck movements. ates to IV and V finger; there is weakness of the wrist
The movements of the cervical spinal segment are limit muscles - tabl. 11.1.
ed and painful; sometimes there is forced posture of the The diagnosis is based on the correlation between
head. The examination reveals painful points laterally the clinical signs and symptoms and neuroimaging. The
from proc. spinosi, reduced or absent reflexes in upper cervical X-ray establishes straightening of the cervical
limbs, hypoesthesia in the corresponding dermatomes. lordosis, narrowing of the disk and densification of the
C5 root, which emerges between C4 and C5 verte disk surfaces, osteophytes, arthrosis of the joints. Ex
brae, is infrequently affected. There is pain in the shoul amining method of choice is MRT because it displays
der, anterior part of the arm; some difficulties in lateral the compression on the spinal root, the hypertroph
ic changes of the ligaments, osteophytes, tumors, etc.
When contraindications for MRI exist, CT or CT-assist-
ed myeelography is performed. The EMG identifies the
affected root and specifies the degree of its affection.
Differential diagnosis with periarthritis humeroscapu-
laris and cervicobrachial plexitis is performed.
Treatment with immobilizing cervical collar,
NSAIs, physical therapy, massages and traction meth
ods is performed.
Fig.l 1.1. Relathionship o f the 6lh cervical spinal nerve (Fitzgerald) The cervical plexus (plexus cervicalis) is formed
by the anterior divisions of C1-C4 spinal nerves. It in
nervates the muscles and skin of the neck and occipital
region. The plexus has anastomoses with n. accessorius
(for innervation of m. sternocleidomastoideus and m.
trapezius) and with n. hypoglossus (for the sublingual
muscles). Plexus cervicalis gives rise of the following
sensory nerves: n. occipitalis minor (C2, C3), n. auric-
ularis magnus (C3), n. transverses colli (C3), and nn.
supraclaviculares (СЗ, C4). The mixed nerves that start
from the plexus are: n. occipitalis major (C2) and n.
phrenicus (C3, C4, C5) - for motor and sensory inner
vation of the diaphragm.
The brachial plexus (plexus brachialis) is formed
by the ventral divisions of C5-C8 cervical roots and by
Thl thoracic root. After regrouping between mm. sca-
leni are formed the primary trunks (truncus superior -
Fig.l 1.2. S p o n d y lo sis o f vertebra C l p in ch in g nerve C l from C5 and C6, truncus medius - from Cl, and truncus
(F itzgerald ) inferior - from C8 and Thl) - fig. 11.3. The primary
Table 11.1. C lin ical featu res o f cer v ic a l roots a ffec tio n
Root Motor weakness Hypoesthesia Hypo-/areflexia
C4 Diaphragm Lateral cervical aspect Absent
C5 M. deltoideus Lateral aspect of the shoulder Absent
C6 M. biceps brachii I and II finger Biceps and styloradial reflex
Cl M. triceps brachii II and III finger Triceps reflex
C8 Wrist muscles IV and V finger Absent
11. Diseases of the peripheral nervous system / 75
involves the brachial plexus. The primary inferior trunk m. deltoideus with difficult abduction of the arm and hy-
is mainly involved with clinical sins of pain, numbness poesthesia of the external part of the shoulder develop.
and weakness of the arm and wrist and with syndrome Neuropathy of n. m usculocutaneus. It is observed
of Horner. after humeral bone fracture. The affected functions are
The diagnosis is based on the clinical examination those of flexion and supination of the forearm. The bi
that reveals sensory and motor signs spreading outside ceps reflex is diminished and there is hypoesthesia in
the territory of innervation of a single root or peripher volving the medial aspect of the forearm.
al nerve. EMG specifies the type of the damage - root, Neuropathy of n. radialis. The disorder is caused
peripheral nerve, and plexus. Sometimes neuroimaging most frequently by nerve compression and following is
is necessary -X-ray of the lungs, MRT of the brachial chemic changes when the patient fall asleep on his hand
plexus, etc. The test of Adson and Doppler sonography after excessive alcohol consumption (Saturday night
can establish vascular compression. palsy). There is paresis of the extensors of the wrist and
Differential diagnosis: fingers with drooping of the hand and impossible ex
1. Periarthritis humeroscpularis results of changes tension of the fingers and abduction of the thumb. The
in the periarticular tissues - bursitis, tendinitis, inser- sensory disturbances involve the radial part of the wrist
tionitis. Most often the disorder is associated with ex and the first three fingers. In more proximal lesion the
traordinary strong physical strain of the arm or occurs triceps reflex is diminished and the extension of the
without clear provoking factor. The pain in the shoulder forearm is disturbed.
is extremely strong and irradiates to the cervical area Neuropathy of n. m edianus. The most often cause is
and arm. The abduction and the rotation of the arm in chronic micro traumas of the nerve in the carpal canal
crease the pain. There is focal pain when palpating in in uniform motions, in patients with rheumatoid arthri
the area of tuberculum majus of the humeral bone. In tis, tendovaginitis, pregnancy and diabetes. Especially
chronic cases is observed the “frozen shoulder syn difficult become the following movements: pronation of
drome” with reduced mobility of the shoulder joint and the wrist, abduction and opposition of the thumb, flex
chronic pain. ion of the wrist and of the distal finger phalanges. An
2. The shoulder-arm syndrome can be observed in atrophy of thenar develops (“monkey paw”). There are
cases with immobilization of the arm - for example fol pain, numbness and decreased sensation on the palmar
lowing stroke. The clinical features include pain in the surface of the wrist and of I-III fingers and the radial
shoulder and arm, vascular changes in the arm, arthrop part of the IV finger. The pain and numbness are ex
athy of the arm joints. Lately osteoporosis and atrophy tremely strong at night (Brachialgia paresthetica noc-
of the skin and subcutaneous tissues develop. turna). Very typical finding is the focal pain in response
The treatm ent with NSAIs, vitamins, Nivalin, and of pressing the palmar surface of the wrist.
physiotherapy is applied. In the cases with neuro-vas- Neuropathy of n. ulnaris. The compression of the
cular compression is performed surgical removal of the nerve most frequently occurs in the cubital canal be
additional cervical rib. hind the medial epikondil (continuous flexion at elbow
joint during sleep) and in ulnar canal of the wrist and
more seldom in axilla due to use of crutches. The flexion
11.9. MONONEUROPATHIES OF THE of the wrist, IV and V finger, the adduction and abduc
UPPER LIMBS tion of the fingers become difficuly. The sensory dis
turbances involve the ulnar surface of the wrist, V and
From the brachial plexus emerge the following pe the ulnar part of IV finger. The muscles of the wrist and
ripheral nerves: n. thoracicus longus (C5-C7), n. su- hypothenar become atrophic (“clawhand deformity”).
prascapularis (C5-C6), n: axillaris (C5-C6), n. muscu The treatm ent is performed by NSAIs, Nivalin,
locutaneous (C5-C6), n. radialis (C6-C8), n. medianus physical therapy, local steroid applications and surgical
(C7-C8) and n. ulnaris (C7-C8). The neuritis and neu decompression of the nerve.
ropathies are result of inflammatory, traumatic, ischem
ic and toxic causes. The most often seen disorder is the
entrapment neuropathy connected with nerve compres 11.10. LUM BOSACRAL RADICULOP-
sion on its way through narrow canal. ATHY (RADIC ULITIS LUMBOSA-
Neuropathy of n. thoracicus longus. Palsy of m. CRALIS)
serratus anterior and lateral deviation of scapula (scap
ula alata) is observed. The lumbosacral radiculitis (radiculopathy) is the
Neuropathy of n. suprascpulars. It results of nerve most frequent disease of the Peripheral nervous sys
compression in incisura scapulae caused by the ligament tem. In most of the cases it results of lumbar disk her
travelling above it. Clinically is observed palsy of m. su- niation (fig. 11.4.) that is more common in men in the
praspinatus and m. infraspinatus accompanied by diffi age of 30-50 years due to the stronger physical strain.
culties in abduction and external rotation of the arm. The most frequent place of herniation is L5-S1 level,
Neuropathy of n. axillaris. Most often it fallows the and the second one L4-L5. The chronic tramatization of
luxation of the shoulder joint. Palsy and hypotrophy of the intervertebral disks leads to development of degen-
11. Diseases of the peripheral nervous system / 77
erative changes of the disks, expansion of osteophytes
and following changes of the ligaments and interverte
bral joints (spondyloarthrosis). As a result of a sudden
physical exertion disk prolapse or disk protrusion are
developing and most often the root on the lower level
becomes compressed. Other reasons are spondylolis
thesis, hypertrophy of the ligaments, extramedullary
tumor, metastatic lesions, vertebral fractures, and os
teoporosis.
The clinical presentation depends on the size of
the disk herniation, the width of the spinal canal, the
localization and extent of the compression, the develop
ment of ischemia, edema and subsequent demyelination
or axonal degeneration of the root, and aseptic inflam
mation and fibrosis. The lumbago demonstrates itself
by unilateral or bilateral lumbar pain (low back pain)
while for the lumbar disk herniation is typical lumbar
pain with irradiation in lower limbs (lumbosacral rad
iculitis, radiculopathy, sciatica). Typically the onset is
acute following physical exertion with extremely strong
lumbar pain and immobilization. Each attempt to move,
sneezing and coughing increase the pain and the patient
remains in anthalgic position slightly bent and skewed Fig.11.4. N erves com pressed by posterolateral prolapse o f
aside. In supine position the patient is with flexed leg the two low est intervertebral d iscs (Fitzgerald)
early stages and later with syringomyelitic sensory dis The progression continuous up to 2-4 weeks, followed by
orders and trophic changes. The infectious mononucle stationing and slow recovering. Various clinical variants
osis is caused by Epstein-Bar virus. The clinical picture of the acute inflammatory demyelinating polyneuropathy
combines a variety of CNS and PNS signs and symptoms: • are described: subacute inflammatory demyelinating pol
cranial neuritis, acute pandysautonomic state, multiple yneuropathy, acute motor axonal neuropathy (AMAN),
polyneuropathy with brachial plexus predominant in acute sensory-motor axonal neuropathy (AMSAN), cra
volvement, Guillain-Barre syndrome. In Lyme disease nial polyneuritis, syndrome of Miller-Fisher (external
are observed various CNS and PNS symptoms - crani ophthalmoplegia, ataxia and areflexia), sensory variant,
al neuropathy (predominant facial nerve involvement), cranial-pharyngeal-cervicobrachial variant, and acute
multiple mononeuropathy, subacute and chronic polyneu pandysautonomy.
ropathy resembling acute demyelinating inflammatory The diagnosis is performed by EMG (reduction in
polyneuropathy and chronic demyelinating inflammato conduction velocity) and lumbar puncture (protein-cell
ry polyneuropathy but with CSF pleocytosis. Different dissociation in CSF). Maximal elevation of the CSF pro
complications of the PNS can be observed in AIDS: acute tein is observed to the 3-4 week from the beginning of
demyelinating inflammatory polyneuropathy and chronic the clinical symptoms, but in 10% of the cases the protein
demyelinating inflammatory polyneuropathy, distal ax remains normal.
onal sensory polyneuropathy, multiple mononeuropathy The disease must be distinguished from disorders hav
and autonomous polyneuropathy. ing an acute development of paraparesis in their clinical
Inflammatory demyelinating polyneuropathies. course: poliomyelitis, botulism, porphyrin polyneuropa
This group includes: acute demyelinating inflammatory thy, and toxic polyneuropathy, post diphtheria paralysis,
polyneuropathy, chronic demyelinating inflammatory acute thrombosis of the basilar artery, post-infectious
polyneuropathy, and multifocal motor polyneuropathy transversal myelitis, some muscle disorders (Myasthe
with conduction block. nia gravis, periodic paralysis, etc.). If CSF pleocytosis is
Acute inflammatory demyelinating polyneurop present meningopolyneuritis associated with Lyme dis
athy (Guillain-Barre syndrome; AIDP). The disease ease, HIV and CMV infections must be excluded.
is described in 1856 by Landry as ascending paralysis Treatment with plasma exchange and high doses in-
and later in 1916 by Guillain, Barre and Strohl. After tra venous immunoglobulin (0.4 g/kg/24h in 5 consecu
eradication of the poliomyelitis the acute demyelinating tive days), artificial pulmonary ventilation, prophylaxis
inflammatory polyneuropathy is the most often cause of of the phlebothrombosis and compression neuropathies is
a peripheral palsy in the developed countries. The mor performed. There is good prognosis for full recovery in
bidity is 1-3/100 000. The onset of the acute demyeli 80% of the cases. Residual paresis is observed in 10-15%
nating inflammatory polyneuropathy in 2/3 of the cases of the cases and death in 2-6% of the cases.
is preceded by respiratory or gastrointestinal infection Chronic inflammatory demyelinating polyneurop
(CMV, EBV, Mycoplasma pneumoniae, Campylobacter athy (CIDP). The disease is more common in men at
jejuni), vaccination, surgery, trauma, thrombolytic thera the age of 50-60 years. An autoimmune pathogenesis is
py and use of heroin. The postinfectious autoimmune eti accepted - macrophages mediated demyelination with a
ology of the disease is accepted basing on the similarity participation of antibody and T-cell immune mechanisms
between the experimental allergic neuritis and the acute in the presence of HLA association. Unlike AIDP data
demyelinating inflammatory polyneuropathy. The anti for preceding infection are absent. The pathomorpholog-
bodies towards the ganglosides of the infectious agents ical changes are connected with chronic demyelination
react with similar lipopolysaccharide antigens of the pe and inflammatory infiltration of the more proximal parts
ripheral nerves (GM1, GDlb, GQlb, GTla) - molecular of the peripheral nerves, with signs of axonal degenera
mimicry. The pathomorphologic changes are most pro tion. The clinical course is slow, the disease progression
nounced in the anterior roots, proximal parts of the spinal continues more than 2 months with development of flac
nerves, and the lower cranial nerves with infiltration of cid distal and proximal paresis accompanied by areflexia,
lymphocytes and macrophages, segmental demyelination muscle atrophy and sensory and autonomous disturbanc
and remyelination in recovering. es. The cranial nerves are seldom affected (in 10-15% of
The clinical course is acute or subacute - progres the cases), but demyelinating lesions in the CNS are more
sive symmetric muscle weakness in lower limbs, which often observed (10%).
ascends and involves the thorax, respiratory muscles, Different clinical variants of CIDP are described:
upper limbs and in 50% of the cases the cranial nerves 1) multifocal motor neuropathy; 2) multifocal acquired
(facial bilateral palsy, bulbar palsy). In 10-20% of the pa demyelinating asymmetrical polyneuropathy; 3) distal
tients develops respiratory insufficiency requiring arti acquired demyelinating symmetrical polyneuropathy;
ficial respiratory ventilation. The sensory symptoms are 4) cranial neuritis; 5) brachial and lumbosacral plexus
milder presented then the motor one. Flaccid paraparesis neuropathy; 6) multiple mononeuropathy; 7) combined
which can accelerate to quadriplegia is observed objec central and peripheral demyelination; 8) purely sensory
tively combined with areflexia, distal hypoesthesia and neuropathy.
muscle atrophy. Typical are the autonomous disturbances There is also a group of CIDP associated with other
(sinus tachycardia, and lability of the arterial pressure). disease: 1) HIV infection; 2) lymphoproliferative dis
11. Diseases of the peripheral nervous system / 81
eases (Hogkin lymphoma); 3) monoclonal and biclonal chronic intoxication with organic or nonorganic sub
gammapathy - macroglobulinemia, POEMS, osteoscle stances, drugs, excessive alcohol consumption. Pato-
rotic myeloma; 4) systemic Lupus; 5) Diabetes mellitus; morphologically is observed distal axonal degeneration.
6) chronic active hepatitis B and C; 7) Crohn disease; Only the lead intoxication associated polyneuropathy
8) Thyrotoxicosis; 9) demyelination of the CNS. Nowa shows signs of demyelination. The alcohol polyneurop
days all acquired demyelinating polyneuropathies with athy results mainly of the toxic influence of the alcohol
unknown etiology are reffered to Cl DP. and its metabolites and of the malnutrition and vitamin
The diagnosis is based on the EMG findings (seg B1 deficiency. It starts with numbness in the feet, some
mental demyelination accompanied by signs of axonal times with burning pain (burning feet) and later signs
degeneration), CSF examination (protein-cell dissocia of disturbed deep sensation in the feet are added with
tion) and if necessary peripheral nerve biopsy (demyeli symptoms of alcoholic pseudotabes and distal paresis in
nation, inflammation). The cases of CIDP are subdivided the feet. There exist tendency to compressive neuropathy
to: idiopathic, with unknown etiology; CIDP associated of n. radialis and n. peroneus which develop during sleep
with other disease for determination of which additional and after alcohol consumption. The disorder often com
examinations are performed. bines with alcohol cerebellar atrophy and encephalopathy
The treatment course is long-lasting (for several of Wernicke.
months) and is performed with steroids, immunosuppres The clinical picture of the lead polyneuropathy in
sants (Azathioprine), intermittent schemes with high dos cludes multiple mononeuropathy, especially of nn. radi-
es immunoglobulin and plasma exchange. The prognosis ales, and seldom distal sensory-motor polyneuropathy.
is less favorable - only 40% of the patients remain with The mercury poisoning provokes distal sensory poly
full or partial remission. neuropathy, cerebellar tremor, explosive speech, n. op
Multifocal motor neuropathy (MMN). Predomi ticus lesion and autonomous disturbances. The thallium
nantly men at the age of 20-50 years are suffering from poisoning is associated with distal sensory-motor poly
the disease. The pathomorphologic changes are similar neuropathy, causlgic pain in the feet, balding, and rare
to those of CIDP. The clinical picture is characterized ly lesions of II, III and VI cranial nerves. The arsenic
by predominantly distal asymmetrical pareses involving poisoning is connected with development of distal senso
upper limbs at the beginning, muscle hypotrophy and ry-motor polyneuropathy with pronounced autonomous
fasciculations. Sensory disturbances are not observed. and trophic disturbances. The poisoning with phospho-
EMG finds persisting multifocal conduction block affect rorganic insecticides leads to development of distal
ing motor fibers while the sensory fibers of the peripheral sensory-motor polyneuropathy, and pyramidal signs.
nerves are preserved. The CSF protein remains in normal The medicated polyneuropathies represent compli
ranges. The differential diagnosis includes spinal mus cations of the treatment with Izoniazid, Nitrofurantoin,
cle atrophy, ALS, CIDP, multiple mononeuropathy asso Metronidazole, Chloroquine, Disulfiram, Chlorampheni
ciated with vasculitis and hereditary neuropathies with col, Colchicine, Phenytoin, Vincristine, Vinblastine, Cis-
tendency to compression neuropathies. Increased titres of platina, high doses of Pyridoxine (vitamin B6).
antibodies to GM1 ganglioside and positive therapeutic Deficiency-state polyneuropathies. They develop in
response to intra venous immunoglobulin treatment are deficiency states as a result of malnutrision, deseases of
established. the gastrointestinal tract (chronic gastritis, resection of
Polyneuropathies associated w ith monoclonal gam- stomach, neoplastic lesions, parasitosis), treatment with
mapathies. Gammapathies are observed as an isolated certain drugs, chronic alcoholism. The deficiency state
disease or represent manifestation of other disease - ne polyneuropathies are connected with deficiency of vita
oplasm, chronic infections. Polyneuropathy can develop min Bl, B6, B12, folic and nicotinic acid. They represent
in the course of multiple myeloma, macroglobulinemia themselves clinically with sensory or sensory-motor pol
of Waldenstrom, benign monoclonal gammapathy, and yneuropathies, involving predominantly lower limbs. In
cryoglobulinemia. Polyneuropathy is observed in 3-5% deficiency state of vitamin Bl is observed also encepha
of the patients with multiple myeloma. It manifests with lopathy of Wernicke. The deficit of vitamin B12 is associ
mild sensory-motor and purely sensory polyneuropathy. ated with polyneuropathy, subacute combined degenera
In patients with osteosclerotic myeloma which represents tion of the spinal cord and organic psychic syndrome. An
1-3% of all cases with multiple myeloma, the frequency isolated deficit of Pyridoxine is observed after prolonged
of polyneuropathy is 50%. The benign monoclonal gam treatment with Izoniazid, Hydralazine and Penicillamine
mapathy is discovered in 1.5% of the population above and is demonstarated clinically with predominantly sen
the age of 50 years and in 3% above the age ot 70 years sory polyneuropathy.
and is most often of IgM type. IgM antibodies demon The metabolic polyneuropathies are associated with
strate reactivity with the myelin-associated glycoprotein Diabetes, porphyria and uremia.
(MAG), GM1 and GDlb gangliosides and glycolipids. The diabetic polyneuropathy is the most common
The anti-MAG polyneuropathy is demonstrated by pre one among all polyneuropathies. Different pathogenic
dominantly sensory polyneuropathy combined with atax mechanisms are discussed: 1) endoneural ischemia due
ia of the gait and postural tremor in upper limbs. to increased blood viscosity and diabetic microangiopa
Toxic polyneuropathies. They result of acute or thy; 2) increased glucose concentration in the peripheral
82 / Clinical Neurology
nerves, metabolizing by aldosoreductase and sorbitol de tachycardia, lability of the blood pressure, CNS signs
hydrogenase with worsening of the axonal transport; 3) (delirium, seizures) and PNS signs (acute predominantly
disturbed production and transport of neural trophic fac motor polyneuropathy with ascending character and pos
tors; 4) oxidative stress and endoneural hypoxia; 5) ab sibility of respiratory and bulbar muscles involvement).
normal glycation of the membrane-associated proteins; The diagnosis is confirmed by the detection of increased
6) immune-inflammatory disturbances of the capillary levels of 5-amino levulinic acid and porphobilinogen
endothelium cells in asymmetric diabetic neuropathy. in blood and urine. The urine acquires dark color after
The pathomorphological changes show signs of both standing on light due to the oxygenation of the porpho
segmental demyelination and axonal degeneration. Sev bilinogen to porphyrin.
eral clinical forms are observed. The most common type Polyneuropathies associated with malignant ne
is the distal symmetric sensory-motor polyneuropathy - oplasm. They result of the toxicity of the cytostatics,
numbness, burning sensation, coldness and pain in the irradiation, metabolic and vascular reasons or can have
feet and lower limbs, fatigue in the legs, distal hypoesthe- a paraneoplastic character. The following forms are ob
sia for pain, touch and temperature, disturbed vibration served: 1) subacute sensory neuropathy; 2) subacute mo
sense, diminished or absent knee and Achilles reflexes. tor neuronopathy; 3) autonomous neuropathy. The para
In 10% of the cases peroneal palsy is developing. In sec neoplastic neurological syndromes are associated with
ond place by frequency is the distal predominantly sen antibodies against the tumor antigens exhibiting crossed
sory polyneuropathy. It is characterized by affection of reactivity with neural antigens. The multiple mononeu
the poorly myelinated and nonmyelinated fibers for pain ropathy can be associated with metastatic invasion of the
and temperature (small fiber neuropathy) - numbness peripheral nerves.
and pain in the feet and lower limbs and distal hyper Hereditary polyneuropathies. The Hereditary sen
esthesia. When the thick myelinated fibers are affected sory-motor neuropathy (Charcot-Marie-Tooth disease;
(large fiber neuropathy) the deep sensation is disturbed Neural muscle atrophy) is the most frequent hereditary
and clinical signs of sensory ataxia are present - diabetic disease of the PNS - 1-4/10 000. It is characterized by
pseudotabes. progressive distal muscle weakness, abolished reflexes,
Other forms of diabetic neuropathy are: 1) Painful high arch of the feet, and milder sensory disturbances.
distal neuropathy with weight reduction; 2) Symmetric Eight basic types hereditary sensory-motor neuropa
distal motor neuropathy; 3) Symmetric proximal mo thies (HSMN) are distinguished: HSMN I (demyelinating
tor neuropathy; 4) Cranial neuropathy with oculomotor type, with slow conduction velocities), HSMN II (axon
nerves involvement (preserved pupilary reflexes); and al type, with preserved conduction velocity), HSMN III
with facial nerve involvement; 5) Asymmetric proximal (syndrome of Dejerine-Sottas, hypertrophic neuropathy),
diabetic neuropathy; 6) Diabetic cervicobrachial plex- HSMN IV (with excess of phytic acid, Refsum’s disease),
opathy; 7) Diabetic thoracic-abdominal neuropathy; 8) HSMN V (with spastic paraparesis), HSMN VI (with op
Diabetic compression neuropathies - of n. ulnaris in the tic atrophy), HSMN VII (with pigmentary degeneration
cubital canal; of n. medianus in the carpal canal; of n. of retina), HSMN VIII (w ith other signs and symptoms).
peroneus and of n. radialis. The hereditary neuropathy with tendency to paresis of
The diagnosis is easy when the neuropathy develops compression and the syndrome of Roussy-Levy with pos
in patient previously diagnosed with Diabetes. tural tremor in the hands is referred to type I HSMN.
The treatment is based on a precise metabolic control The hereditary sensory and autonomous neuropathies
of the Diabetes, antiplatelet drugs, vasodilating drugs, (HSAN) are subdivided in 5 types and are character
antioxidants, a-lipoic acid, and vitamins. In patients with ized by congenital disturbances of pain and temperature
neuropathic pain are useful Amitriptyline, Gabapentin, sensation, perforating ulcers of the feet, neuropathic ar
Tiagabine, Carbamazepine, Phenytoin, Tramadol. thropathy and various autonomous changes, especially in
The uremic neuropathy develops in patients with type III (syndrome of Riley-Day). The hereditary motor
long-lasting renal insufficiency and in patients on hemo neuropathies (HMN) are referred also to the distal spinal
dialysis. At the beginning there are excitatory symptoms muscle atrophy.
(numbness, fasciculations, cramps) and subsequent de Polyneuropathies associated with congenital met
velopment of sensory-motor neuropathy. abolic defects. They are associated with lysosomal dis
Porphyry neuropathy. The disease is associated with eases, for example metachromatic leucodystrophy, with
relapses of acute intermittent porphyry that are induced peroxysome diseases (Refsum disease, adrenoleucod-
by drug usage (barbiturates, sulphonamides, analgesics, ystrophy/adrenomyeloneuropathy), with porphyria and
hypnotics). They induce the enzyme 5-amino levulinic amyloidosis. The familial amyloid neuropathies are of
acid synthetase which leads to overproduction of 5-ami several types and are most often connected with extra
no levulinic acid and porphobilinogen due to hereditary cellular deposition of transthyretin, and more rare with
enzyme deficit. The neurological complications are asso apolipoprotein A-l and gelsolin. The polyneuropathy is
ciated with the neural toxicity of 5-amino levulinic acid distal, with predominant affection of the senses for pain
and of deficiency of chem. The relapse starts with strong and touch, and lately it takes the course of sensory-motor
abdominal pain, vomiting, autonomous disturbances with neuropathy.
12. Neuroinfections - meningitis and encephalitis / 83
12. NEUROINFECTIONS -
MENINGITIS AND ENCEPHALITIS
12.1 MENINGITIS III. Spirochetal meningitis: associate with Lues, Lyme
disease, Leptospirosis
The inflammatory process most often affects the
leptomeninges (arachnoidea and pia mater) and more IV. Mycotic meningitis: Cryptococcus, Candida
rare solely dura mater (pachimeningits) or arachnoidea
(arachnoiditis). The inflammation can involve plexus V. Parasitic meningitis: Cysticercus
chorioideus (chorioiditis), the ependyma of the ventri
cles (ependimitis) and the subependymal cerebral tissue VI. Meningitis associated w ith other diseases: Brucello
(ventriculitis). The term meningitis accepted in the clin sis, Listeriosis, Anthrax
ical practice in fact overlaps the term leptomeningitis
- inflammation of the leptomeninges. The meningitis is VII. Serous allergic meningitis: following immuniza
divided to primary and secondary. The primary men tion, vaccination, therapeutic serum application
ingitis results of direct infectious involvement of the
meninges while the secondary represent a complication Aseptic meningitis: following lumbar puncture, intrath
of other systemic or focal inflammation. The infection ecal introduction of contrast substances and medications
of the meninges is carried through blood, in proxim The clinical picture despite of the type of infectious
ity, directly in traumatic injuries or yatrogenic - via agent comprises of two syndromes: 1) general somatic in
introduction of substances. The meningitis is most fre fectious syndrome (elevated temperature, faintness, leuko
quently diffuse, cerebrospinal, and rarely predominant cytosis, elevated sedimentation rate, and tachycardia) and 2)
ly cerebral or predominantly spinal. According to the meningeal syndrome that includes the signs and symptoms
localization they are divided to predominantly convex of the meningoradicular syndrome and inflammatory CSF
(viral meningitis), and predominantly basal (tubercu syndrome.
lous meningitis). The course of meningitis can be acute The meningoradicular syndrome is presented clinical
(viral meningitis, purulent meningitis), subacute and ly with headache, nausea, and vomiting, reduced sensory
chronic (tuberculous, syphilitic, mycotic meningitis). threshold with tactile, auditory and visual hyperesthesia.
According to the type of inflammatory exudate they are The examination reveals neck rigidity and positive symp
subdivided into serous (viral), sero-fibrinous (tubercu toms of Kernig and Brudzinski. Due to the increased CSF
lous), hemorrhagic (influenza) and purulent (bacterial). secretion develops the syndrome of increased intracranial
The inflammatory process can involve the underlying pressure - papilloedema, bradycardia. Very frequent are
cerebral tissue - meningoencephalitis, meningomyeli- observed the so-called general cerebral syndrome (psychic
tis, meningoencephalomyelitis. disorders, qualitative and quantitative changes of conscious
The classification of the types of meningitis is etiolog ness) and the syndrome of focal cerebral injury with affec
ical - according to the type of the infectious agent. tion of cranial nerves and the encephalitic component (mild
paresis, pyramidal signs, speech disturbances)
I. Viral meningitis The CSF syndrome is characterized by an increased CSF
1. Primary viral meningitis - enteroviruses (Coxsackie pressure, changes in the color of the CSF (lucid in viral men
virus, ECHO virus, Polio virus); Benign lymphocytic cho ingitis, opalescent and purulent in bacterial meningitis), an
riomeningitis of Armstrong-Lillie increased number of cells (CSF pleocytosis), increased level
2. Secondary viral meningitis: associated with Herpes of protein (hyperproteinrachia), and reduction of the glucose
zoster, parotid virus, influenza, varicella, measles level (hypoglycorachia). The pleocytosis is lymphocytic in
serous meningitis and neutrophilic in bacterial meningitis.
II. Bacterial meningitis The changes of the CSF differentiate the meningitis from
1. Primary bacterial meningitis: meningococcal, tuber the so-called menindism. The latest is also associated with
symptoms of meningoradicular irritation but the CSf is nor
culous
2. Secondary bacterial meningitis: streptococcal, mal under examination. The meningism can develop after
staphylococcal, pneumococcal, enterococcal, gonococcal, lumbar puncture, intrathecal introduction of contrast sub
etc. According to the source of the primary infection the stances and drugs, in acute feverish conditions in children.
types of meningitis are subdivide to: associated w ith ear,
nasal, sinus, tonsils and teeth infections, with infected
12.1.1. VIRAL MENINGITIS
facial and head injuries and cerebral traumas; associated
The viral meningitis is more frequent than the bac?
with secondary hematogenous dissemination from a dis
terial one. It has acute beginning, the meningeal syn
tant inflammatory focus in bacterial endocarditis, chole
drome is moderately or strongly expressed, there is lym
cystitis, osteomyelitis, etc.
84 / Clinical Neurology
phocytic pleocytosis in the CSF and the prognosis for anthema is observed (Broncholm disease), accompanied
recovery is good (benign serous meningitis). The viral by myalgia, aphthous stomatitis, abdominal ache, hepat-
meningitis is divided to primary and secondary form. osplenomegaly and lymphadenopathy. The diagnosis is
The primary viral meningitis is separate disease, while confirmed by detection of the virus in rhinopharynx,
the secondary is a complication of other viral disease feces, PCR of the CSF, and increased antibody levels
- parotitis, varicella, herpes zoster, adenoviral infec in serum.
tion, etc. The influenza viruses induce both primary Poliomyelitic meningitis. It is induced by I, II, and
and secondary meningitis. More than 90% of the viral III type of the polio virus. The meningitis precedes the
meningitis results of enteroviral infection (polio virus, paralytic stage or can proceed as isolated meningitis
Coxsackie viruses, and ECHO viruses). without paresis.
Enteroviral meningitis. It is observed more fre Lymphocytic choriomeningitis (Armstrong-Lil-
quently in summer and autumn. Sometimes small epi- lie). It is caused by RNA-virus, described in 1935.
demmies are observed in childhood. The infection is oral Reservoirs for the infection are the mice. The mor
or by airborne route. The viruses are reproduced in the bidity is higher in winter. The mechanism of infection
lymph nodes and thereafter via the blood (viremia) reach is airborne. The virus spreads hematogenically to the
the meninges and other organs. Clinically the viremia is meninges and chrioid plexus and induces CSF hyperse
presented by toxic-infectious syndrome - temperature, cretion and increased intracranial pressure. The onset
headache, adynamia, myalgia, angina, conjunctivitis, is acute with high temperature and development of me
gastroenteritis, polyadenitis. The meningitis has acute ningeal syndrome with headache and CSF pleocytosis
course with high temperature, pronounced meningeal (500-1000х10л6/1). The clinical picture is manifested
syndrome, sometimes encephalitic symptoms are ob in three forms: meningitis, meningoencephlitis and flu
served. The CSF is serous, with lymphcytic pleocytosis like. The febrile period is longer and recovery occurs in
100-500х10л6/1, mild hyperproteinorachia and normal 3-4 weeks.
glucose range. The disease has benign course with com The diagnosis of viral meningitis is based on the
plete recovery in 2-3 weeks. In some of the meningitis epidemiologic data, CSF syndrome, and viral detection
types can be observed extra neural symptoms as myal in secretions from the rhinopharynx, in feces, and CSF,
gia, myositis, myocarditis, pericarditis. and viral genome detection in the CSF with PCR.
Coxsackie viral meningitis. The virus is detected in Differential diagnosis with secondary viral menin
1948 in Coxsackie, New York State. Group A consists of gitis parotid, influenza meningitis and meningoenceph
23 antigen strains and group B - of 6. The viruses from alitis and tuberculous meningitis is performed.
group A lead to meningitis and/or herpandina, myositis The treatment is symptomatic - dehydration with
and rarely meningoencephalitis. Types A7 and A9 can Mannitol, steroids 1-3 mg/kg, antibiotic therapy in cas
produce reversible poliomyelitic paresis. Type B also es of accompanying bacterial infection. There is a rule
provokes meningitis which is with milder course but can in the therapeutic strategy of the viral meningitis - an
be associated with epidemic myalgia, myalgia of the di tituberculous treatment is performed until tuberculous
aphragm, myocaritis and pericarditis. In newborns lead meningitis is excluded.
to disseminated infection with severe encephalitis, and
if a pregnant woman is infected during the first months 12.1.2. BACTERIAL MENINGITIS
of the pregnancy there is risk for congenital abnormal
ities. The infection comes by airborne route and infre 12.1.2.1 Tuberculous meningitis.
quently by enteral. The virus reaches the meninges by The infection is induced by the human type of my
hematogenous route. The Coxsackie meningitis starts cobacterium of Koch. The disease is transmitted by air
acutely, with rapid development of a meningoradicular borne droplets. Susceptible to the infection are people
syndrome with mild CSF hyperproteinorachia, mod who suffered from severe somatic disorders, living in
erate pleocytosis (25-250х10л6/1) and lasts up to 2-4 poor hygienic conditions and people with malnutrition.
weeks. Before the invention of Streptomycin the death rate was
ECHO viral meningitis. The name of the virus almost 100%. The introduction in the practice of other
comes from the viral particularities (Enteric Cytopath- antituberculous medicaments makes the prognosis for
ogenic Human Orphan). The ECHO viral group consists recovery better.
of 32 antigen types, of which type 2, 4, 5 and 9 is of The tuberculous meningitis most often results of he
particular importance because can cause reversible po- matogenous dissemination of the lung primary tuber
liomylitic paresis. The route of infection is by the gas cular complex connected with development of scattered
trointestinal tract and the involvement of the meanings tubercles in the cerebral parenchyma and meninges. In
is hematogenous. During the summer small epidemies cases of rupture of such tubercles in the subarachnoid
can be observed. It starts with temperature, gastroin space and ventricles meningitis is developing. The men
testinal symptoms and after 3-4 days occur the menin ingitis can result also of meninges involvement in the
goradicular syndrome, which recovers in 2-3 weeks. In course of miliary tuberculosis. A rare source of menin
the first 24-48 h the CSF pleocytosis is polynuclear and geal infiltration can be a conglomerate cerebral tubercle.
than converts to mononuclear. In V< of the patients ex Pathomorphologically it is characterized by tubercles
12. Neuroinfections - meningitis and encephalitis / 85
scattered in meninges, ependyma and choroidal plexus. for 3 months to a total dose of 120-130 g; 4) Ethambu-
The inflammatory exudate has sero-fibrinous charac tol - 25 mg/kg once daily for two months; 5) Pyrazi-
ter and is most expressed basally involving the cranial namide - 25-35 mg/kg once daily for 18-24 months.
nerves and blood vessels. The basal cisterns, aqueduc- Medications of II order are: Ethionamide, Cycloserine,
tus Sylvi, III and IV ventricle and the spinal arachnoid Paraaminosalycilate (PAS), aminoglycosides, etc. For
space are blocked. Obstructive hydrocephalus develops prevention of polyneuropathy (caused by Rimicid) vit.
and risk of herniation exists. In the chronic stages spinal B6 is presribed; against the neuropathy of the acustic
block and vasculitis are observed. nerve (caused by Streptomycin) - vitamins of group B;
The clinical picture is characterized by subacute against the toxic optic neuropathy (caused by Etham-
course in the patients with poorly cured primary lung butol) - vit. A. The steroids are useful in the treatment
or with other organ localization tuberculosis. The pro of arachnoiditis. For treatment of the intracranial pres
dromal period is connected with the tuberculous in sure Mannitol i. v. or Furosemid and Acetazolamide
toxication and lasts 2-3 weeks - low-grade fever, ady are prescribed. The treatment course continues several
namia, loss of appetite, sleep disturbances. Then within months until normalization of the CSF and two normal
a few days the meningeal syndrome is demonstrated results divided with a month. In 10-20% of the patients
- high temperature, headache, nausea, and vomiting, are observed residual symptoms - arachnoiditis, hydro
disturbed consciousness. The beginning can be more cephalus, spinal block and severe paraparesis, lesions of
acute with symptoms of intracranial hypertension and cranial nerves, etc.
papilloedema. In the disease course cranial nerves are
involved and cerebral vasculitis can develop. The state 12.1.2.2. Bacterial purulent meningitis
of untreated patients soon deteriorates; the intracranial They are subdivided to primary (meningococcal
hypertension increases; the diencephalon involvement meningitis) and secondary (pneumococcal, staphylo
leads to hyperthermia; decerebration and quadriparesis coccal, streptococcal, etc.) which are complication of
are observed. In poorly cured patients the meningitis other inflammatory disorder, caused by pyogenic bac
becomes chronic with development of myeloradiculitis teria. The causative agents differ in the different age
and spinal block presenting themselves with paraparesis groups: newborns - enterobacteria, Haemophylus influ
or quadriparesis. The coonglomerate brain tubercle is enzae; 2-5 years - meningococci, pneumococci, strepto
an infrequent form of cerebral tuberculosis with clinical coccus; 5-50 years - meningococci, pneumococci, Hae
presentation resembling cerebral tumor. mophylus infuenzae, enterobacteria; above 50 years -
The diagnosis is based predominantly on the find pneumococci, rarely meningococci. The penetrations of
ings of the CSF examination - the CSF is slightly opal the infection into the meninges is hematogenic or comes
escent, it forms fine fibrinous network. The pleocytosis from adjacent inflammatory focus, directly in traumas,
in the initial 1-2 weeks accounts 50-500х10л6 lymph- and in artificial (yatrogenic) way. The bacteriemia with
cytes and polymorphonuclear cells. In persistence of the massive disintegration of bacteria leads to intoxication,
polymorphonuclear cells in the late stages of the men disturbances in homeostasis and toxic-infectious shock.
ingitis, other infectious agent must be excluded. The The inflammatory cytokines play an important role in
CSF protein is moderately or highly increased (0.8-4.0- the pathogenesis of meningitis and in the development of
5.0 g/1). The CSF glucose is strongly decreased. The de the cerebral edema, the hyperemia and overproduction
tection of the tuberculous mycobacterium in the CSF of CSF. The pathomorphological examination reveals
is very difficult - the examination of the CSF sediment purulent yellowish exudates diffusely involving cere
is positive in 20-30%, while the cultivation in specif bral convexity and basis, edema and miliary abscesses
ic nutritional environments and inoculation of guinea in the subpial cerebral tissue, smoothing of brain folds
pigs gives a result in a few weeks. The fastest result and filling of the ventricles with purulent exudates.
is obtained by PCR for detection of the mycobacterium
in CSF. Parallel to the CSF examination is performed Meningococcal epidemic purulent meningitis
X-ray of the lungs, hematological examinations, cranial The causative agent is meningococcus (Neisseria
CT and MRI. meningitides), identified by Weickselbaum in 1887. Four
Differential diagnosis with viral, bacterial and my meningococcal subtypes are described - А, В, C and D.
cotic meningitis, syphilitic and neoplastic meningitis The meningococcus is Gram-negative bacterial agent,
and with cerebral sarcoidosis is performed. developing intracellularly in the polynuclear leucocytes.
The treatm ent should begin immediately without In the early stages it can be detected in nasopharynx and
waiting for bacteriological confirmation. It is conduct blood culture. The infection is transmitted by airborne
ed with a triple combination of anti-tuberculosis agents route. From nasopharynx the infectious agent directly
which are divided into drugs of I and II order. Medi reaches the meninges or spreads in hematogenic way.
cations of I order are: 1) Izoniazid (Rimicid) - 5-10-15 Most infected do not get sick and become infection car
mg/kg once daily until complete normalization ot CSF, riers. The meningitis is observed sporadically or causes
moderately for 8-12 months; 2) Rifampicin (Tubocin) - small epidemics. The incubation period lasts from sev
600-900 mg twice daily; 3) Streptomycinum sulluric- eral hours to a few days. Pathomorphologically is ob
urn — 15-20 mg/kg i. m. divided into two applications served diffuse purulent cerebrospinal meningitis. The
86 / Clinical Neurology
clinical onset is acute with high temperature and rapid, tis, mastoiditis, sinusitis, pneumonia. The course of the
in several hours, development of severe meningoradicu- disease is severe; the death rate reaches 20-30%. The
lar syndrome with headache, vomiting and neck rigidity. prognosis is poor when the meningitis develops in asso
Generalized epileptic seizures, decrease in conscious ciation of pneumonia, empiem and lung abscess or when
ness and comatose state are also possible. In 1/3 of the the triad of “pneumonia, meningococcal meningitis and
patients is observed a red-brown petechial rash. The endocarditis” is present. The treatment is performed
disease is sometimes accompanied by conjunctivitis, with cephalosporins, Vancomycin and Meronem.
arthritis and endocarditis. The hematologic examina Staphylococcal meningitis. The causative agents in
tion identifies pleocytosis and increased sedimentation cutaneous infections, abscesses, staphylococcal angina,
rate. The CSF is purulent with highly expressed poly epidural and subdural abscess, and shunting in hydro
morphonuclear pleocytosis - 2000-20 000х10л6/1, the cephalus are S. aureus and S. epidermidis. The meningi
protein is increased and the glucose rates are decreased. tis is observed predominantly in infancy and childhood.
The Gram-negative bacteria are found extracellularly Often develops hemorrhagic-bullous rash, involving
and intracellularly in the CSF sediment. The meningo the limbs. Sometimes abscesses in the cerebral cortex
coccus can be detected in culture in 90% of the cases are observed with clearly developed focal neurological
or the polysaccharide antigen of the bacterial capsule symptoms. The lethality rate is high and 50% of the
can be detected by latex-agglutination. In the extreme recovered have residual neurological symptoms. Treat
ly acute form are developing infectious shock, cerebral ment with Oxacillin, Methicillin, Vancomycin, Mero
coma and death before the manifestation of the menin nem and Imipenem is applied.
geal syndrome. In the course of the meningococcal sep Streptococcal meningitis. It is mostly observed in
sis a necrosis of the suprarenal gland can develop (fVa- the neonatal period and infancy. Source of the infec
terhous-Friedericksen syndrome) with acute suprarenal tion are otitis, mastoiditis, tonsillitis. The lethality rate
insufficiency, circulatory collapse and DIC syndrome. reaches 25%. The treatment is similar to those of the
The timely and adequate therapy reduces the death rate pneumococcal meningitis.
up to 10% but possibility exists for residual lesions of Enterobacterial meningitis. Most of the cases are
the auditory and optic nerve, epilepsy, cognitive impair observed during the neonatal period and in the first 6
ment and hydrocephalus. months after delivery. The most common cause is the
High doses of antibiotics are administered for treat presence of asymptomatic maternal bacteremia. The
ment (Penicillin G 10-40 UI/24h i.m. or i.v.; Cephtriax- clinical picture manifests with low-grade fever, collaps
one, Cephalotine, Ceftazidim, Chloramphenicol), Man es and development of hydrocephalus. The lethality is
nitol, steroids, rehabilitation, anticonvulsive drugs, and 70%. Combinations of Chloramphenicol and Gentamy-
antipyretics. cin or Ampicillin and Gentamycin are applied for treat
Secondary purulent meningitis. They represent ment.
complications of pyogenic inflammations of different Pseudomonas aeruginosa meningitis. It develops
organs: otitis, mastoiditis, sinusitis, abscess, phlegmon, in children with prolonged treatment with immunosup
furuncle, bronchiectasis and endocarditis. The causative pressants or in children with congenital or acquired
agents are pneumococci, staphylococci, streptococci, immune deficit. The prognosis is poor and the lethality
enterococci, pseudomonas, etc. The pyogenic bacteria rate id high. The treatment is performed by Amikacin,
reach cerebrum from adjacent inflammatory focus or in Tobramycin and Gentamycin.
hematogenic way. The direct inoculation in surgery and
trauma and breakthrough from abscess are also possible
mechanisms of infection. The pathomorphological and 12.2. ENCEPH ALO M YELITIS
clinical picture are similar to those of the meningococ
cal meningitis. The cerebral inflammation is referred to as encepha
Haemophylus influenzae meningitis. The disease litis and the inflammation of the spinal cord - to as my
is most frequent in children - 90% of the cases are elitis. According to their pathomorphology the encepha
observed before age of 5 years. With the introduction litis are divided to: polioencephalitis (involving the grey
of Haemophylus influenzae vaccine the morbidity de cerebral matter), leukoencephalitis (involving the white
clined sharply. But the morbidity in elderly people with matter) and panencephalitis. The course of disease can
reduced immunity, Diabetes mellitus, and alcoholism be acute, subacute and chronic. The primary enceph
has increased. A source of the primary infection is si alomyelitis are caused by direct viral invasion, while
nusitis, otitis, or skull fracture. The course and diagno the secondary are post-infectious and post-vaccinal and
sis are similar to those of the meningococcal meningitis. have autoimmune pathogenesis.
Ill generation cephalosporins are applied for treatment.
In childhood the prognosis for recovery is poor in 90% Classification of the types of encephalomyelitis
of the cases. 1. Viral polioencephalomyelitis: 1) poliomyelitis of
Pneumococcal meningitis. This is the most com Heine-Medin; 2) Pseudopoliomyelitic enteroviral dis
mon type of meningitis in adulthood. It is caused by S. eases; 3) Current acute Parkinsonian encephalitis; 4)
pneumoniae. The infection of meninges occurs in oti Rabies
12. Neuroinfections - meningitis and encephalitis / 87
2. ARBO viral encephalitis - tick-borne encephalitis sis can develop in the first 24 h —the so-called morning
(the Far Eastern and Central European tick-borne en palsies. The involvement of the motor nuclei in medulla
cephalitis) oblongata leads to bulbar palsy (dysphonia, dysphagia)
3. Demyelinating viral and possibly viral encepha and ot the motor nucleus of the facial nerve - to pe
lomyelitis:!) in acute rash infections and post-vaccinal; ripheral facial palsy. In such cases children who have
2) acute cerebellitis; 3) Inluenza meningoencephalomy- not been vaccinated should always think about polio
elitis; 4) acute demyelinating encephalomyelitis; 5) uv- myelitis. In severe cases the paralyses start from lower
eoencephalitis limbs, and involve the thoracic muscles and the muscles
4. Chronically-persisting viral encephalitis and ot upper limbs (ascending paralysis o f Landry), includ
recurrent herpes viral infections: 1) herpes simplex ing the intercostals muscles and the diaphragm with
meningoencephalitis; 2) herpes zoster meningoenceph risk of respiratory insufficiency. In brainstem forms the
alitis reticular formation is involved with disturbed vascular
5. Slow viral infections of common persisting virus and respiratory control demonstrated by hiccough, re
es: 1) subacute sclerosing panencephalitis; 2) Rubella duced respiratory rate, cyanosis, restlessness and anxi
chronic progressive panencephalitis; 3) acute necrotiz ety, arterial hypertension and circulatory collapse. The
ing hemorrhagic leukoencephalitis; 4) progressive mul recovery stage starts in 2-3 weeks after the development
tifocal leucoencephalopathy; 5) adenoviral multifocal of paralyses and continues up to 2-3 years. The patient
encephalitis remains with permanent residual paralyses, with short
6. Prionic diseases: 1) Kuru; 2) Creutzfeld-Jacob’s ening and thinning of the limb and joint contractures. In
disease some patients 20-40 years after the initial infection can
develop the so-called post-polio syndrome - slowly pro
12.2.1. Poliomyelitis ( Poliomyelitis anterior gressive muscle weakness, atrophy and fasciculations.
acuta; Heine-Medin disease) The diagnosis is based on the epidemiologic data,
The disease is described by Heine (1840) and inves the lymphocytic pleocytosis in the CSF, the detection of
tigated by Medin (1890). It is caused by enteroviruses the infectious agent in rhino-pharynx, feces and C SF,
(RNA viruses) that are subdivided into three antigenic the increase antibody levels and PCR.
types (I, II,and III). The infection by one of them does In the spinal form the differential diagnosis in
not result in immunity to the other. The poliomyelitis cludes polyneuritis, transverse and disseminated mye
was the most frequent viral epidemic disease of CNS litis, acute spinal compression; the ascending paralysis
before the vaccine introduction in 1956. Currently in must be differentiated from acute inflammatory demy
some countries there are still isolated cases in omis elinating polyneuropathy and disseminated encephalo
sions in vaccination. The morbidity is higher in summer myelitis; the bulbo-pontine form - from cranial poly
and autumn months. At epidemic mostly children and neuritis, acute Multiple sclerosis and brainstem enceph
immunocompromised adults are affected. The infection alitis.
occurs by the faecal-oral route via contaminated food The treatment is symptomatic, including steroids
and water. The incubation period lasts 5 to 35 days. The for the brain edema and inflammation. In cases with
viruses multiply in the lymphoid tissue of tonsils and respiratory disturbances and bulbar palsy is necessary
in the intestinal tract and invade the cervical and mes artificial respiratory ventilation. In the recovery state
enteric lymph glands. They reach CNS in hematogenic are recommended physiotherapy and rehabilitation.
and neural way. The viruses cause selective destructive Prophylaxis with oral vaccine of Sabin is performed
lesions of the anterior and lateral horn spinal motor neu but cases with post-vaccine poliomyelitis have been de
rons and of the grey nuclei in the brainstem.The disease scribed and currently the intramuscular inactivated vac
has asymptomatic course in 90% of the affected and cine is preferred.
only 5-10% of the infected patients develop the typical Pseudopoliomyelitic enteroviral diseases. They
paralytic form. are caused by Coxsackie-viruses (herpangina, hemor
Clinical picture. The first stage (stage o f viremia) rhagic conjunctivitis and myocarditis), ECHO-viruses
manifests itself by flu-like symptoms (temperature, (rashes), entero virus 71 (in newborns and infant can
headache, pains in the throat and muscles, nausea, in cause bulbar palsy and respiratory insufficiency and in
testinal symptoms) and continues 1-4 days. Than be adolescence - limb’ palsies)
gins the stage of ostensible recovery, followed by the
preparalytic stage —with meningeal syndrome, febrility, 12.2.2. Rabies (Rabies, Lyssa)
headache, vomiting, and neck rigidity, positive symp
toms of Kernig and Brudzinski, and hyperesthesia. In The infection is transmitted via byte by sick wild or
the CSF serous meningitis is found with lymphocytic domestic animal (foxes, wolves, dogs, cats, etc.). Cases
pleocytosis 50-300х10л6 (at the beginning with mild of infected cavers have been described via inhalation
neutrophylic pleocytosis) and mild proteinorachia. The of aerosols, containing excrements of sick bats. The
paralytic stage is demonstrated by acute development inoculated via saliva virus travels along the peripheral
in 24-48 h of asymmetric muscle paresis, involving the nerves and reaches the spinal ganglia, where multiplies
proximal parts of the limbs. In small children the pare and invades the CNS. The virus reaches the salivary
88 / Clinical Neurology ________________________
glands via the efferent nerves. The incubation period is sheep and goats, infected by the tick). The course of the
between 1 and 3 months, and exceptionally is 10 days or disease is with the clinical picture of panencephalitis
more than a year. The duration of the incubation period with typical development of flaccid paralyses of the cer
depends on the site of the bite - shorter in bites close to vical muscles with drooping of the head, epilepsy, etc.)
the face and head. The pathomorphological changes are Forms with chronic progression with clinical features
most pronounced in the bulbar grey nuclei, hippocam of chronic anterior poliomyelitis and syndrome of ALS
pus, and hypothalamus. Of pathognomic importance are described.
are the eosinophilic cytoplasmic inclusions (Negri bod Moskito transmitted panencephalitis. They carry
ies) in the pyramidal neurons of hippocampus and in different names according to the area of their primary
Purkinjie cells. They contain the viral necleocapsid. Fo distribution: in USA (eastern and western horse enceph
cal microglia aggregations are also observed - nodules alitis, encephalitis St. Louis, Californian encephalitis),
o f Babesh. Venezuelan horse encephalitis, Japanesse B enceph
Clinical picture. On the site of the bite persist pain, alitis, Australian encephalitis, etc. They have severe
numbness and sensory loss. The prodromal stage con course, high lethality rate and residual symptoms.
tinues several days and demonstrates by high tempera
ture, headache, adynamia, sleeplessness. The next stage 12.2.4. Acute disseminated encephalomyelitis
is the excitatory one - increased headache, psychomo (ADEM)
tor agitation, anxiety. Hyperexcitability of the bulbar
nerves occurs with spasms of the laryngeal, pharynge ADEM is a rare complication of some acute, most
al and respiratory muscles attempting to drink water. often exanthemic infections in the childhood (post-in
There is fear even at the sight of water and the mention fectious encephalomyelitis) or of vaccination (post-vac
of the word causes spasms (hydrophobia). The air flow cinal encephalomyelitis).
may cause spasms of the facial and respiratory muscles The etiology includes enormous number of diseas
(aerophobia). The light also can evoke similar spasms es as measles, varicella, smallpox, rubella, parotitis,
(photophobia). The focal spasms can generalize to gen influenza, infectious mononucleosis, viral infections
eralized tonic-clonic seizure. The condition progresses of the upper respiratory tract, mycoplasmal infections.
to extreme agitation, delirium and hallucinations. The Vaccinations against smallpox, rabies, measles, rubella,
next stage .is the paralytic one - the bulbar excitement parotitis, diphtheria, Infuenza, etc. also can induce de
transfers to bulbar palsy - palsy of the pharyngeal, la velopment of ADEM.
ryngeal and respiratory muscles, coma and death in 1 The pathogenesis is autoimmune - the viruses or
to 3 weeks. the vaccinal agents can evoke activation of the auto
Treatment. The site of bite is washed by water and reactive T cells through the mechanism of molecular
soap and then is treated with benzyl ammonium chlo mimicry or through nonspecific mechanism. The au
ride (Zephiran) that inactivates the virus. If the animal toimmune reaction is against the myelin basic protein
looks healthy it is observed for 10 days for symptoms of or other antigens. The development of ADEM follow
Rabies. Prophylaxis with human anti-Rabies immuno ing vaccination against Rabies with vaccine containing
globulin is performed (HRIG) at a dose 20 Ul/kg - the suspension of rabbit’s spinal cord led to the description
half of it is infiltrated in the tissue surrounding the site of the experimental allergic encephalomyelitis (EAE)
of bite and the remaining is injected i.m. On the day of that has allergic autoimmune pathogenesis. The patho
the incident and on days 3, 7, 14 and 28 is performed morphology of the EAE and ADEM is similar - they
active immunization with vaccine containing human are types of leukoencephalitis with scattered lesions
diploic cellular line (HDCV) at a dose 1 ml i.m. of perivenous demyelination, edema and inflammatory
infiltration with mononuclear cells. The acute hemor
12.2.3. ARBO-viral panencephalitis rhagic necrotizing encephalitis represents a fulminant
form of ADEM which develops after Influenza and res
This is a group of acute primary panencephalitis, piratory infections with Mycoplasma pneumoniae. It is
transmitted through mosquito and ticks bites - Ar- characterized by scattered hemorrhagic and necrotizing
thropode borne. The ARBO-viruses are small RNA vi lesions accompanied by polymorphonuclear inflamma
ruses that are transmitted by mosquito and ticks. Hosts tory infiltration.
are the people and the horses and reservoir of the infec The clinical picture develops acutely during the
tion are the birds and the rodents. These types of en period of recovery of the exanthema or 1 to 6 weeks
cephalitis have certain seasonality and geographic dis following the vaccination with high temperature, head
tribution. They do not occur in our country. ache, epileptic seizures, pareses and paralyses, ataxia,
Tick-borne encephalitis: spring-summer far East disturbances in consciousness. According to the type of
ern tick-borne encephalitis (transmitted by Ixodes per- the preceding infectious disease or vaccination there ex
sulcatus), summer-autumn Central European tick-borne ist particular selectivity in the CNS involvement - with
encephalitis (transmitted by Ixodes ricinus), and du meningitis (in parotitis), with encephalitis (in measles),
al-wave spring-summer meningoencephalitis (the infec with cerebellitis (in varicella), with myelitis (following
tion is transmitted via consumption of raw milk from vaccination against Rabies and smallpox). In some of
12. Neuroinfections - meningitis and encephalitis / 89
the forms the PNS is predominantly involved - ascend opment of the exanthema or within 1 to 3 weeks - the
ing polyradiculoneuritis type Guillain-Barre (vaccina vaccination.
tion against Rabies, following Influenza and respiratory The differential diagnosis includes other types of en
viral infections). An acute toxic encephalopathy accom cephalomyelitis, cerebral phlebothrombosis, and acute
panied by development of severe cerebral edema (syn form of MS.
drome of Reye) is a rare complication of varicella, in I he treatment is performed with high doses ster
fluenza and rubella. It is accepted currently that most of oids, immunoglobulin and plasma exchange.
the cases of ADEM develop following respiratory viral
infection and despite of the clinical picture of enceph 12.2.5. Herpes simplex viral encephalitis
alomyelitis they can demonstrate themselves by optic
neuritis, opticomyelitis and transeverse myelitis. This is the most common form of sporadic encepha
The post-measles encephalitis has frequency 1:1000 litis (1/250 000) and has high lethality rate. The diseased
cases. It is observed currently very rare because of the are predominantly above the age of 20. The encephalitis
introduction of anti-measles vaccine. The disease usu has no seasonality. It is caused by DNA-virus - HSV-
ally takes the course of diffuse encephalomyelitis or en type 1, which evokes the development of the oral and
cephalitis with high lethality rate (10-30%) and residual nasal herpetic lesions. HSV-type 2 can provoke acute
neurological symptoms in 20% to 40% of the cases. encephalitis in newborns in the presence of maternal
Post-varicella encephalitis occurs with the clinical genital herpes infection. In adults HSV-type 2 leads to
picture of cerebellitis represented with gait ataxia and development of serouse meningitis, polyradiculoneuri
myelitis. The acute onset ataxia in the childhood may tis or myelitis.
result also of enteroviral infection, EBV, CMV and HSV It the course of the primary infection the virus
infection and of respiratory viruses. reaches gangl. Gasseri via the branches of the trigem
Post-rubella ADEM manifests as meningomyelitis inal nerve and remains there in latent state. Years af
and rarely as ascending myelitis of Landry. ter the primary infection as a result of reactivation the
Post-parotitis ADEM is demonstrated predominant latent-persisting virus can reach the cerebral tissue of
ly with the clinical presentation of meningitis combined anterior and middle cerebral fossa via the meningeal
with mild signs of encephalitis and has benign course. branches of the trigeminal nerve. Other possible way
Post-influenza meningoencephalitis develops in of transmission towards the brain is via the olfactory
0.3% of the diseased people. It takes the clinical course tract. The pathomorphological changes have necrotiz-
of severe encephalitis, meningoencephalitis, meningi ing-hemorrhagic character and are most pronounced in
tis, myelitis and polyradiculoneuritis and has high le the basal parts of the temporal and frontal lobe (limbic
thality rate. Pathomorphologically are observed signs encephalitis). The cerebral edema is strongly expressed.
of capillary-toxic inflammatory encephalopathy -scat The eosinophylic inclusions in the nuclei of the neurons
tered small hemorrhagies, cerebral edema and mildly and glial cells are typical finding.
expressed inflammatory infiltrations. It develops in the The clinical picture develops acutely with high
course of the acute infection or after 2-3 afebrile days temperature, headache, neck rigidity, epileptic seizures,
with elevation of the temperature, headache, epileptic decrease of consciousness and coma. Initially olfacto
seizures and decreased consciousness. Differential di ry and gustatory hallucinations, anosmia, psychotic
agnosis with subarachnoid hemorrhage is recommend behavior, temporal epileptic seizures, and memory dis
ed. turbances can be observed. These signs and symptoms
The anti-rabies post-vaccinal encephalomyelitis had correspond to the frontal-temporal localization of the
high frequency (1/600) and lethality following vaccina encephalitis. The condition soon deteriorates. The cere
tion w ith the vaccine prepared by suspension of rabbit’s bral edema and temporal herniation lead to deep coma
cerebral tissue. It has had the clinical picture of myelitis, tose state. Respiratory arrest develops in 48-72 h.
sometimes ascending and combined with bulbo-pontine Important diagnostic finding detected by CT and
signs. Currently human diploid cell vaccine is used that MRI is the typical distribution of the changes in the
is not connected with neurological complications. temporal basal and orbital-frontal cerebral areas. The
Post-smallpox-vaccinal encephalitis has had a se diagnosis is confirmed by PCR. The CSF pressure is
vere course with high lethality rate bur currently it is highly increased. The CSF examination reveals lym
not observed after the dropping of the vaccine from phocytic pleocytosis mixed with neutrophils and eryth
immunization calendar due to eradication of smallpox rocytes, increase in protein, reduction in glucose levels.
The latest finding makes the differential diagnosis with
worldwide.
The CSF examination demonstrates mild to moder tuberculous meningitis and mycotic meningitis difficult.
ate lymphocyte pleocytosis (25-250х10л6/1) and hyper- The differential diagnosis includes other types of
proteinorachia (0.5-1.5 g/1). The CT discovers scattered viral and post-infectious encephalitis, acute hemor
hypodense lesions and MRI — similar hyperintense rhagic encephalitis of Hurst, cerebral abscess, subdural
empyema, stroke, rupture of aneurism, cerebral venous
leasions in T2 sequences.
thrombosis, septic embolism, glioblastoma.
The diagnosis is based on the clinical signs ot en
The treatment is successful if started immediate
cephalomyelitis following within 4 to 21 days the devel
90 / Clinical Neurology
ly after the suspicion for herpetic encephalitis arising. external auditory canal and the skin behind the ear. It is
It begins with intravenous application of Acyclovir (30 often accompanied by facial palsy and n. statoacusticus
mg/kg) for 10 to 14 days. Other antiviral drugs as Val- affection (Herpes zoster oticum).
acyclovir, Famcyclovir, Foscavir, and Gancyclovir can In complicated course of the infection in immuno-
also be useful. Treatment against the cerebral edema suppressed patients (zoster multiplex) exists risk for de
and seizures is started. The prognosis for recovery is velopment of transeverse or ascending zoster myelitis.
poor in cases of late start of the treatment with lethality It is observed 1 to 3 weeks after the rash presentation
rate 30-80%. Different residual symptoms are observed and is characterized by asymmetric lower paraparesis,
in the surviving patients - amnesia, epileptic seizures, sensory and pelvic reservoir disturbances. Herpes zos
dementia, aphasia, and paralyses. ter encephalitis is the most severe complication of the
cranial herpetic infection. It is manifested by headache,
12.2.6. Herpes zoster viral encephalitis confusion, ataxia, epileptic seizures, focal and general
cerebral signs and has poor prognosis.
The herpes zoster viral infection is often observed. The diagnosis is based on the presence of a typi
The morbidity rate is 3-5/1000 cases per year. The Vari cal skin rash. If the rash is absent (zoster sine herpete)
cella-zoster virus (VZV) is DNA virus which is a common in presence of radicular pain, differential diagnosis is
causative agent of the cutaneous herpes and varicella. made with thoracic and abdominal disorders. VZV can
After the recovery of chickenpox the virus persists be identified by PCR of the infectious exudates and of
in latent state in the sensory ganglia neurons. When a CSF. Lymphocytic pleocytosis is present in the CSF
decrease in immunity occurs (elderly people, lympho even if no complications occur. CT and MRI in patients
ma, HIV, splenectomy, irradiation, neoplastic diseases, with zoster angiitis reveal focal infarction lesions. The
immunosuppressive treatment) the virus reactivates and angiography findings include stenosis or thrombosis of
via neural way reaches the corresponding dermatomes. the internal carotid artery or diffuse vascular changes.
In a complicated course of the spinal herpes the adja Antibodies against the membrane viral antigen (VAMA)
cent dorsal roots, meninges and spinal cord is involved. are detected in serum and CSF samples of the patients
The cerebral herpetic infection can be spread towards with zoster encephalitis. The isolation of VZV from
the brain tissue and cerebral blood vessels with devel CSF samples is also possible.
opment of encephalitis and vasculitis. The inflammato For prevention of complications treatment with
ry-necrotizing changes in ganglionitis involve several Acyclovir and other antiviral medicaments is applied.
adjacent spinal ganglia, usually unilaterally. The pos Acyclovir is applied intravenously in patients with dis
terior horns are most severely damaged in myelitis, but seminated herpes, myelitis, encephalitis and angiitis.
involvement of the anterior horns is also possible. The For prophylaxis of the disseminated zoster and vari
leptomeningeal reaction is restricted and mildly ex cella in immunosuppressive patients is prescribed var
pressed. icella zoster immunoglobulin (VZIG). The treatment
Clinical features. During the childhood neurologi of post-herpetic neuralgia includes antidepressants,
cal complications can develop in the course of varicella anticonvulsants, and opioid analgesics. Treatment with
(frequency 1/1000-4000) - encephalomyelitis, serouse steroids is not recommended due to the risk of viral dis
meningitis, polyneuritis, cranial neuritis. The varicel semination.
la encephalitis has autoimmune pathogenesis or can be
caused by direct viral invasion. Cerebellum is predomi 12.2.7. Chronic, slow-viral and prionic
nantly affected (cerebelitis) with features of acute ataxia infections of the Nervous system
and benign prognosis.
The clinical picture in adults is most frequently pre They result of: 1) habitual persisting pathogenic
sented by ganglionitis, ganglioneuritis and gangliorad- viruses - subacute progressive panencephalitis; pro
iculitis - pain, numbness, and usually unilateral thoracic gressive rubella encephalitis; 2) habitual persisting
vesicular cutaneous rash. The isolated muscle paralyses nonpathogenic viruses - progressive multifocal leuko-
followed by atrophy are seldom observed. Sometimes encephalopathy; 3) retro-viruses - HTLVl-associated
mild meningeal sign are present - hyperpyrexia, ady myelopathy (tropic spastic paraparesis), HlV-encepha-
namia, headache, neck rigidity, lymphocytic CSF pleo lomyelitis; 4) infectious proteins (prions) - spongiform
cytosis. After the rash recovery remain strong neuralgic encephalopathies.
pains (postherpetic neuralgia).
The herpetic rash in Herpes zoster ophthalmicus 12.2.7.1. Subacute sclerosing panencephalitis (SSPE)
involves the territory of innervation of the ophthalmic It was described in 1933-1945 under different names.
nerve. Risk of keratitis, panophthalmitis and oculomo In Bulgaria the disease was investigated after 1953 un
tor palsies exists. Cerebral arteriitis of the carotid artery der the name o f hyperkinetic progressive panencepha
followed by infarction can develop on the side of the litis. The frequency of the disease was 5-10xl0A6 but it
infection. is very rare observed after the introduction of anti-mea
The herpetic rash in herpes zoster infection of gan sles vaccine in 1960. In the developing countries with
glion geniculi (Ramsey-Hunt syndrome) involves the frequent measles epidemic the frequency of the disease
12. Neuroinfections - meningitis and encephalitis / 91
reaches 20-100/10A6. It is observed in infancy and ad before the onset of encephalitis. Due to the deficiency
olescence in patients with history of measles infection ot cell-mediated immunity develops severe encephalitis
in early child’s age (under the age of 2). In the cases of with massive necrotizing areas and without inflammato
insufficient maternal antibodies, the measles virus per ry reaction. The course is acute, with epileptic seizures,
sists in the so-called defective form which cannot be refractory to the therapy, sometimes with the charac
attacked by the immune mechanisms. ter of epilepsia patialis continua, paralyses, ataxia, dis
The pathomorphological changes are diffuse and af turbed consciousness and death. CT and MRI visualize
fect the cerebral cortex of the frontal and occipital lobes different in size lesions. The typical changes in the CSF
predominantly, the white matter of the hemispheres, ba are absent. The diagnosis is confirmed by detection of
sal ganglia (thalamus, hypothalamus, substantia nigra), the viral RNA with PCR or by cerebral biopsy. Treat
the brainstem nuclei and cerebellum. Destruction of the ment with Ribavirin and symptomatic treatment of the
neurons with microglial aggregations, demyelination cerebral edema and seizures is applied.
and degeneration of the axons, perivascular inflamma
tory infiltrations and gliosis is observed. In the nuclei 12.2.7.3. Rubella progressive panencephalitis
and cytoplasm of the nerve cells are observed typical This is an extremely rare panencephalitis develop
eosinophilic inclusions which contain measles virus. ing in children with congenital rubella infection or even
The clinical symptoms start 6-8 years after the more rare in patients with history for rubella infection
initial measles infection. The panencephalitis has sub in the childhood. The congenital Rubella infection is re
acute progressive course and terminates lethally for 1 sult of placental and fetal infection during the maternal
to 3 months. Some cases with duration more than 10 viremia. The congenital Rubella syndrome is extreme
years are also described. The disease demonstrates it ly severe if infection occurs during the first trimester
self by typical symptoms: 1) progressive dementia; 2) of the pregnancy - delayed development, microcepha
extrapyramidal hyperkinesias; 3) pyramidal syndrome ly, deafness, cataracts, glaucoma, cardiovascular ab
with progressive muscle spasticity, sometimes decere normalities, etc. Infrequent complications of Rubella
bration; 4) partial and generalized epileptic seizures; are the post infectious encephalitis and post-infectious
5) hypothalamic syndrome - hyperhydrosis, salivation, polyradiculoneuritis. The subacute progressive Rubel
bulimia, anorexia, cachexy, and terminal hyperpyrexia. la panencephalitis develops in children aged between
The hyperkinesias are involuntary movements with var 8 and 19 years with medical history of congenital Ru
ious characters - myoclonic, ballistic, choreoathetotic, bella in stationary state. The course is similar to those
and dystonic. The most typical are synchronous, like of the subacute sclerosing panencephalitis - behavioral
electric shock hyperkinesias resembling “sudden bows”, changes, ataxia, tremor, spasticity, epileptic seizures,
sudden openings of the mouth resembling “fish out of pseudobulbar symptoms - dysarthria, and dysphagia. In
water” and sudden cries like “cry of a seagull”. In the the CSF is present mild pleocytosis, strong increase and
terminal stage occur decortication, autonomous distur fractioning of the gamma-globulins and anti-Rubella
bances, hyperpyrexia and cachexy. antibodies.
The diagnosis is based on the typical findings of the
EEG, MRI and CSF. EEG registers generalized periodic 12.2.7.4. Progressive multifocal leukoencephalopa-
discharges of sharp waves, followed by series of slow' thy (PML)
waves (Radermecker complexes). MRI reveals symmet PML is demyelinating disease, caused by reactiva
ric or asymmetric large cortical and subcortical hyper- tion of habitual non pathogenic viruses - Papovaviridae
intense lesions on T2 sequences. The CSF examination family (JC, BK, SV40) in immunosuppressive patients.
shows mild lymphocyte pleocytosis, strong oligoclonal The viral infection has occurred in the childhood and
type increase of immunoglobulins, and intrathecal syn the virus persists in the reticulo-endothelial system.
thesis of IgG. The oligoclonal bands contain antibodies JCV reactivates in suppression of the cellular immunity
against the measles virus. Anti-measles antibodies are and lowering of CD4 below 200/mmA3. It infects and
identified in serum and CSF of the patients. damages the oligodendrocytes. Scattered focal demyeli
Differential diagnosis is performed with other sub nating lesions are observed. Such immunosuppressive
acute encephalitis (measles, rubella), cerebral tumors, states are connected with lymphoproliferative disor
Chorea minor, epilepsy, dementia, leukodystrophies, ders, transplantations, chemotherapy, irradiation, auto
diffuse sclerosis of Schilder, etc. immune disorders, tuberculosis, sarcoidosis, and carci
The treatment is non effective. Isoprinosin 50-100 noma. After the development of HIV-epidemic in 1981
mg/kg, Interferon alpha and Ribavirin can increase the the cases of PML have increased with more than 20%.
The multifocal demyelinating lesions are distributed
life expectancy.
predominantly in the subcortical white matter of the
12.2.7.2. Subacute measles encephalitis in immuno hemispheres, corpus callosum, brainstem, cerebellum
and spinal cord. Hyperplasia of astrocytes is observed.
deficiency states
The disease represents a rare fatal opportunistic Vascular and inflammatory changes are absent.
measles infection in immunosuppressed children and The disease is demonstrated with behavioral chang
es, progressive dementia, central paralyses, visual dis
adults with a history of measles infection 1-9 months
92 / Clinical Neurology
turbances, aphasia, dysarthria, and qualitative changes of the neurons, reactive astrocytosis and microgliosis
in consciousness. CT and MRI reveal scattered and and intraneural storage of PrPsc and amyloid plaques
confluent demyelinating lesions in the subcortical white are observed. Signs of inflammation are absent. In CJD
matter. The CSF examination is normal. The viral DNA the changes are predominantly in the cerebral cortex, in
is detected by PCR of the CSF. In negative PCR results Kuru disease - in cerebellum, in FFI - in thalamus, and
is recommended cerebral biopsy. The differential diag in GSSD - in pons and medulla oblongata.
nosis includes other opportunistic cerebral infections.
The treatment with anti-viral medicaments (Cyto C reutzfeldt-Jacob’s disease (CJD)
sine arabinoside, Vidarabine, Cidovir, Acyclovir, Inter The most common form which includes more 85%
feron alpha, Interferon beta) and anti-retroviral therapy of the cases is the sporadic (sCJD). Its frequency is
against HIV (HAART) increases the life expectancy. 1:1х10л6. It involves usually people above the age of
60 years. Approximately 10% of the cases are familial
12.2.7.5. Transmissible spongiform encephalopathies and have autosomal-dominant transmission route. It is
(Prionic diseases) more frequently observed in Jews born in Libya and in
The transmissible spongiform encephalopathies Slovakia. The family type of CJD, GSSD and FFI are
(TSE) represent a new, unique group of disorders that more common in people aged between 45 and 49 years.
are transmitted in genetic and infectious way. In peo In 1994 in England is described the first case of variant
ple are observed 4 prionic diseases: 1) Creutzfeldt-Ja- o f CJD (vCJD) connected with consumption of infected
cob’s disease (CJD) or Subacute spongiform encepha bovine meat. In the yatrogenic form (jCJD) the infec
lopathy; 2) Hereditary syndrome of Gerstmann-Straus- tion is transmitted via blood transfusions, medical and
sler-Scheinker (GSSD); 3) Fatal family-type insomnia surgical manipulations with contaminated instruments
(FFI) and 4) Kuru disease or cerebellar spongiform en and materials, tissue transplants of cornea and dura
cephalopathy. In animals are described 6 types of prion matter, etc.
ic diseases: Bovine spongiform encephalopathy (“mad The clinical picture is characterized with behavio
cow” disease), Scrapie in sheep and goats; Chronic fatal ral and emotional changes, intellectual decrease, speech
disease in mules, deer and elk; Transmissible encepha disturbances, hallucinations and progressive dementia.
lopathy in minks; Spongiform encephalopathy in cats. Myoclonias occurred - asynchronous multiple con
The first case with CJD is descirbed in 1921 by H. tractions, that are spontaneous or provoked by senso
Creutzfeldt. A. Jacobs described the neuropathologic ry stimuli like loud noise. Ataxia and instable gait are
picture of the disease. Initially it is named spastic pseu observed. Pyramidal and extrapyramidal signs, partial
dosclerosis. In 1969 D. Gaidusek studied Kuru disease and generalized epileptic seizures, visual disturbanc
in primitive tribes, practitioners of ritual cannibalism es, amyotrophy, and fasciculations are developing. The
in New Guinea. He succeeded to transmit the disease in symptoms have descending distribution. In the terminal
primates via injections of brain extracts. Similar trans- stages are observed akinetic mutism and autonomous
missibility is observed in Scrapie in sheep. In 1982 S. disorders and death occurs in 4 to 8 months. The family
Prusiner identifies “infectious protein”, containing only form starts earlier. In the yatrogenic form the cerebellar
proteins in absence of nucleic acid and named it PRION. signs precede the appearance of memory disorders. In
The prionic protein (PRP) is glycoprotein which exists the variant of CJD the affected people are younger (a
in two forms: normal - with dominating globular alpha mean age - 28 years) and the course of the disease is
spiral and pathogenic - with fibrilar beta structure. The prolonged (8-38 months).
prionic protein gene mutation (PRNP), which is locat The hereditary syndrom e of G erstm ann-Straus-
ed in 20 chromosome, the non-pathogenic form (PrPc) sler-Scheinker (GSSD) is an autosomal-dominant dis
is transformed into pathogenic (PrPsc). As a result the ease with mean age of onset 40-50 years and duration
normal metabolism of PrP is disturbed and it is stored 2-10 years. It results of mutations of the PrP gene. Patho-
in the cells. In the hereditary forms of the prionic dis morphologiacally are observed spongiform changes in
eases the mutations of PRNP lead to synthesis of mutant the grey cerebral matter, amyloid plaques and amyloid
protein PrPm which makes easy the transfer of PrPc into angiopathy. It starts with cerebellar ataxia, nistagmus
PrPsc. PrP is expressed in multiple tissues, but predom and dysarthria and later pyramidal signs and dementia
inantly in the neurons. The pathogenic prions initially are added.
replicated in the lymphoid tissue (tonsils, lymph nodes, The fatal fam ily-type insomnia (FFI) is an auto
and lien) and are transported in hematogenic way to the somal-dominant disease, characterized by treatment
CNS and invade the neurons. Prions can reach cerebrum refractive persistent insomnia and autonomous sympa
via n. vagus also. The incubation period of the prionic thetic disorders - hyperhydrosis, tachycardia, tachyp
diseases is 30-40 years. nea, hyperpyrexia, arterial hypertension. Pyramidal
The pathomorphologic changes involve mainly the signs, cerebellar ataxia, myoclonias, hallucinations and
grey matter of the cerebral and cerebellar hemispheres, memory loss are developing. The onset of the disease
basal ganglia, and brainstem. Vacuolation of the cyto is in younger people (18-61 years), and its duration is
plasm is developing and it acquires spongiform appear from 7 to 36 months. The pathological changes are most
ance (status spongiosus). Degeneration and destruction pronounced in thalamus.
12. Neuroinfections - meningitis and encephalitis / 93
Kuril disease (cerebellar spongiform encephalop The HIV infection affects all age groups and is dis
athy). The disease is common in New Guinea where in tributed in all countries. The infection occurs in het
the course ritual cannibalism are consumed the paren erosexual or homosexual contact, exposition to con
chymal organs of the deceased relatives or the infected taminated blood and from mother to fetus. Especially
tissues are rubbed into the skin. The incubation period risk group are the drug addicts using injectable drugs.
is 5-10 years long. The pathomorphological changes are Alter the introduction in 1996 of the highly active an
expressed mostly in cerebellum. The clinical picture is tiretroviral therapy (HAART) the cases of AIDS in the
manifested by severe cereballar ataxia, body and limbs developed countries demonstrated decrease by 38%, of
tremor, dementia, abnormal eye movements with con the deaths - by 67% and life expectancy was increased
vergent strabismus, paralyses, incontinence, and death by 15-20 years. The disease has become chronic with
in 4-24 months. possibility of development of various neurological com
The diagnosis of CJD is difficult in the early stages plications.
and easier in presence of the typical constellation of pro The infecting of the T4 lymphocytes is mediated by
gressive dementia, myoclonias, and EEG changes (dif their membrane CD4 receptors and by supplementary
fuse slow-wave activity and paroxysms of generalized chemokine co-receptor. The genetical variability of the
three phase sharp waves). CT and MRI visualize the latest determines perceptiveness to HIV. Despite of the
changes in the cerebral cortex, cerebellum, thalamus, fact that neurons and glia cells also have CD4 receptors,
and basal ganglia. PET reveals cortical hypometabolism viral replication has been proved only in lymphocytes,
in CJD, and in thalamus - in FFI. The Western blot re macrophages and monocytes.
action detects 14-3-3 protein in the CSF as a non specif The course of HIV infection has three phases: acute
ic marker of neuronal damage and also neuron-specific infection phase, chronic infection phase and phase of
enolase, protein S-100 and tau-protein. Genetic testing developed AIDS.
is performed in family cases. Definite diagnosis is per I. The acute phase begins 2-4 weeks after the viral
formed with cerebral biopsy. explosion and lasts an average of 10 days. A dramatic
The differential diagnosis includes: Alzheimer’s increase of the viral load in the blood, high rate of viral
disease, frontotemporal dementia, cortico-basal de replication and high serum rates of the viral p24 antigen
generation, multisystem atrophy, leukodystrophy with are observed. The virus can readily be isolated from the
adult onset, myoclonus-epilepsy, HIV-encephalopathy peripheral blood lymphocytes. The primary HIV infec
with dementia, progressive multifocal encephalopathy, tion may remain asymptomatic, but in 50-70% of the
isolated cerebral angiitis, subacute progressive enceph cases are observed symptoms similar to mononucleosis
alitis, Herpes simplex encephalitis, litium intoxication, - hyperpyrexia, headache, fatigue, myalgia, sore throat,
etc. lymphadenopathy, maculopapular rash, and painful ul
Treatment with Quinacrine and monoclonal antibod cerations of the buccal mucosa. At the end of the acute
ies against the prionic protein are tested, but as a rule phase occurs seroconversion - the synthesis of neutral
the therapeutic attempts are non-effective. izing antibodies of class IgM and IgA leads to clearance
of the viraemia and reduces the serum level of p24. In
12.2.7.6. Neurological complications in HIV and this process are involved the cytotoxic lymphocytes and
AIDS the cytokines of CD8 lymphocytes.
The first clinical cases of AIDS are described in II. In the next, chronic phase is observed a relative
1981. The infectious agent was identified in 1983 and in balance between the immune responses and viral rep
1986 was named HIV (human immunodeficiency virus). lication. The duration of this latent stage even without
It was believed initially that the neurological compli treatment is 8-10 years and it can be asymptomatic.
cations are result of opportunistic infections and neo III. The HIV-infection and the viral replication in
plastic lesions due to the immunodeficiency state. But the lymphoid tissue continue to persist during this phase
in 1985 HIV-1 was detected in cerebral and spinal cord and lead to decrease in the number of CD4 cells and the
tissue, in CSF and peripheral nerves and was accepted cell-mediated immunity. When the number of the CD4
that the neurological complications result ot primary cells decreases below the level of 200/mmA3 starts the
HIV infection of CNS and PNS. phase o f AIDS. In timely initiation of the therapy the
The etiological agent is HIV —retrovirus, belonging duration of the phase is between 5 and 8 years.
to the genus of Lentiviruses. The retroviruses are sub The virus invades CNS at the time of the primary
divided into two groups: HTLV-1 and HTLV-2 (Human infection but does not evoke any clinical symptoms
T-lymphotrophic virus) and HIV-1 and HIV-2. HI\-1 is (asymptomatic early HIV) or manifests by various, usu
the major causative agent of AIDS, while HIV-2 rarely ally transient neurological syndromes (clinically mani
causes AIDS and is detected predominantly in Western fested early HIV). In the next stages of the chronic HIV
Africa. HIV is RNA virus, containing the enzyme re infection is observed a variety of clinical symptoms, re
verse transcriptase, which encodes the transcription ot sulting from the direct damage from the virus or in the
the viral RNA. The proviral DNA integrates with the phase of AIDS - due to the opportunistic infections and
DNA of the host-cell where it starts to replicate or per neoplastic lesions, and drug induced toxicity. Neurolog
ical symptoms are observed in 70% of the patients and
sists in latent state.
94 / Clinical Neurology
can be the first disease manifestation in 10-20%. The strated by rapidly progressive dementia;
early CNS invasion is confirmed by cerebral or spinal c) remitting-relapsing leucoencephalopathy simu
cord, CSF and PNS isolation of the virus and by pres lating Multiple sclerosis. Pathomorphologicaly is ob
ence of intrathecal antibody synthesis. HIV crosses the served leukoencephalitis with pronounced perivascular
blood-brain barrier independently or via the infected infiltrates of HIV-gp41 positive immune reactive mac
macrophages. HIV replicates in plexus chorioideus and rophages and lymphocytes.
in endothelium of the blood vessels, but predominantly Epileptic seizures - focal and generalized are ob
in the perivascular microglial cells and in macrophages served in all disease stages.
which are converted into a reservoir of the infection. Cerebral strokes - ischemic and hemorrhagic may
The selective sensitivity of the brain tissue, the persis result of HIV-vasculitis, cardiogenic emboli, thrombo-
tence and replication of HIV in the cerebral macrophag genic conditions, low PLT count, etc.
es and microglia, despite of the good control of the The chronic progressive myelopathy is manifested
peripheral viral replication, is the reason for accepting by rapidly progressive paraparesis, spastic-ataxic gait
HIV as a neurotropic virus. The cerebral cells are not and pelvic reservoir symptoms. The upper limbs are
infected by the virus. The mechanism of their damage usually not involved because the changes are most pro
is via apoptosis. nounced in the thoracic spine. The condition is due to a
Neurological symptoms are observed in all phases of vacuole myelopathy with vacuole changes in the myelin
the infection and result of involvement of CNS and PNS. and demyelination of the lateral and posterior funiculi.
The neurological manifestations of the primary in The clinical features are similar to those of the subacute
fection are similar to those in other viral infections and combined degeneration of the spinal cord due to vitamin
are usually transient: aseptic meningitis, encephalitis, B12 deficiency state.
partial and generalized epileptic seizures, transversal Syndrome, similar to ALS is also observed and is
myelitis, cranial and peripheral neuropathies (neuritis presented by muscle atrophy, fasciculations, pyramidal
of the facial nerve, acute inflammatory demyelinating signs, bulbar and pseudo-bulbar symptoms.
polyneuropathy, brachial neuritis, ganglioneuritis). The PNS involvement is observed in all stages. It results
aseptic meningitis is characterized by hyperpyrexia, of the effects of the direct infection, opportunistic in
headache, meningism, and CSF pleocytosis up to 200 fections, and the drug-induced toxicity. The following
cells/mmA3, with or without cranial nerves involve types of polyneuropathies are observed:
ment. The anti-HIV-antibodies can be negative in serum a) acute inflammatory demyelinating polyneuropa
as the neurological symptoms may precede the serocon thy (AIDP) can develop during the acute phase of the
version, but the viral antigen p24 in serum is positive. seroconversion, but is observed in the subsequent stages
In the stages following the seroconversion are ob also. Its clinical features are similar to AIDP; the differ
served different neurological syndromes due to the ence is in the CSF lymphocyte pleocytosis;
chronic HIV infection. b) chronic inflammatory demyelinating polyneurop
Persisting or recurrent meningeal pleocytosis which athy (CIDP) is observed in the middle and late phase of
can be asymptomatic or can be manifested by mild me AIDS. It has autoimmune pathogenesis or may result of
ningeal symptoms. The presence of CSF pleocytosis re opportunistic infection with CMV;
quires exclusion of secondary viral infection. c) the multiple mononeuritis is more rare compli
AIDS-dementia complex is the most frequent neu cation. In the early stages it results of immune mecha
rological presentation of the HIV infection. Most typ nisms and vasculitis. In the late stages is complication
ical are the behavioral and cognitive impairments, fol of opportunistic CMV or VZV infection;
lowed by progressive dementia with subcortical frontal d) The distal axonal sensory polyneuropathy is the
character due to a subacute encephalitis. Parallel to the most common HIV-associated complication of PNS.
psychoorganic syndrome are developing motor distur The non-myelinated nerve fibers are affected predomi
bances - pyramidal, extrapyramidal, cerebellar. The nantly and it is presented by strong burning neuropathic
condition has steadily progression to akinetic mutism. pain in feet;
The CSF is normal or demonstrates mild pleocytosis, e) the syndrome of the distal infiltrative lymphocy
oligoclonal bands and positive p24 antigen. MRI reveals tosis is manifested as multiple mononeuritis accompa
cortical atrophy, dilated ventricles and periventricular nied in some cases of parotid gland enlargement. The
confluent hyperintense lesions. PET demonstrates corti peripheral nerves are infiltrated by CD8 lymphocytes;
cal and subcortical hypometabolism. The pathomorpho- 0 the autonomous neuropathy is developing in 30-
logical findings include neuronal loss predominantly of 60% of the cases and is demonstrated by sympathetic or
the frontal lobe, perivascular demyelination, gliosis and parasympathetic disturbances;
microglial nodules. 9) the HIV-associated myopathy is due to the direct
Leukoencephalopathy except in the course of the HIV infection or may has toxic, metabolic, vasculitic or
acute infection is observed also in chronic HIV infec neoplastic etiology.
tion but has different presentation: In the stage o f AIDS are observed secondary oppor
a) extremely acute and fatal leukoencephalitis; tunistic infections and neoplastic lesions. The rate of
b) multifocal vacuole leukoencephalopathy, demon these complications was significantly reduced after the
12. Neuroinfections - meningitis and encephalitis / 95
HAART introduction in 1996. inhibitors (Zalcitabine, Stavudine, and Didanosine),
The different types of meningitis can result of viral which induce distal sensory polyneuropathy and with
(HSV, VZV, CMV, EBV, hepatitis B and C), bacterial Zidovudine that induces toxic myopathy. A syndrome
(Listeria, treponema pallidum, staphylococcus, atypical of acute neuromuscular weakness, combined with lactic
and typical mycobacteria), fungal infections. The most acidosis, leading to death in 15% of the cases.
common causative agent is Cryptococcus. The diagnosis is based on the serologic examination
The different types of encephalitis are due to herpet by ELISA. The positive result requires confirmation by
ic viruses (CMV, HSV, and VZV), hepatitis C, myco Western blot test. In positive patients the CD4 blood
bacterium avium, and toxoplasmosis. count is examined. CT and MRI reveal cerebral atrophy
The focal cerebral syndromes are provoked by tox and focal or multifocal hyperintense lesions. The CSF
oplasmosis, lymphoma, multifocal progressive leucoen- examination demonstrates mild hyperproteinorachia
cephalopathy, abscessus resulting of listeria, C an d id a, and lymphocytosis.
Cryptococcus, aspergilus, pyogenic bacteria, etc. The introduction of HAART transformed the disease
The spinal cord can be affected by herpetic viruses from fatal to chronic with various neurological compli
(CMV, HSV, and VZV), mycobacteria, pyogenic bacte cations. More than 19 different medicaments, grouped
ria, fungi, toxoplasmosis, and lymphoma. in 4 main classes are used:
CMV is a herpetic virus by which is infected 60-80% 1) nucleoside reverse transcriptase inhibitors (nRTI);
of the population. In AIDS it reactivates and causes: 2) non-nucleoside reverse transcriptase inhibitors
1) progressive lumbar and sacral polyradiculopathy (NNRTI);
with rapidly progressing syndrome of cauda equina; 3) nucleotide reverse transcriptase inhibitors (ntRTI);
2) multifocal polyneuritis; 4) protease inhibitors (PI).
3) CMV encephalitis;
4) multifocal necrotizing encephalitis.
HSV type l infects the individuals at an early age, 12.3. NEUROSYPHILIS (NEUROLUES)
while the infection by HSV type 2 results of sexual con
tact as 70-90% of the adults are infected. HSV-1 can Treponema pallidum (Spirochaeta pallida) was iden
cause necrotizing temporal meningoencephalitis; HSV- tified in brain tissue in 1913. It belongs to Spirochaeta-
2 - diffuse meningoencephalitis, lumbosacral radiculi ceae family. Epidemics of syphilis are described further
tis and transversal myelitis. in the 15th century. With the introduction of the antibi
- Toxoplasma gondii infection occurs by eating poorly otic treatment with Penicillin in 1945 and the effective
heat-treated meat and contact with objects contaminated treatment of the primary and secondary syphilis the
with cat feces. The parasite can persist long time in dif rates of neurosyphilis were significantly decreased. In
ferent organs, including brain. In cases with decreased the last decades there is tendency for new increase in
immunity it activates and in AIDS induces toxoplasmal morbidity and especially in cases with AIDS.
encephalitis, and solitary or multiple cerebral abscess Classification. Syphilis can be congenital and ac
es. Typical are the lesions involving basal ganglia. quired. The latest go through the following stages:
Mycobacterium tuberculosis can cause meningitis, 1) primary;
cerebral abscessus and tuberculomas. 2) secondary (hematogenic dissemination);
The neural syphilitic infection in AIDS has atypical 3) early and late latent syphilis;
course. Dominate the cases of early syphilis - meningi 4) tertiary syphilis. Neurosyphilis is developing in
tis, meningovasculitis. 10% of the patients who were not cured. It is subdivided
The multifocal progressive leukoencephalopathy to early (asymptomatic meningitis and acute syphilitic
can be first manifestation of AIDS. It results of oligo meningitis) and late (meningovascular and parenchyma
dendrocyte infection with JC-virus as 70% of the popu tous - tabes dorsalis and paralysis progressiva). With the
lation is infected. Demyelinating lesions are developing. antibiotic treatment there is pronounced reduction in the
In AIDS are observed more often space-occupying frequency of the parenchymatous forms of syphilis.
cerebral processes - lymphomas, gliomas, etc. In primary Pathogenesis. The transmission of the infection
CNS lymphoma isolates EBV, which is oncogenic. In sys is predominantly sexually and rare hematogenic. The
temic lymphoma CNS is affected in 40%. The leiomyosar primary syphilis is represented with development of
coma originates from the mesenchime of the blood vessels. painless ulcer at the inoculation site. If left untreated
Other typical cerebral tumors are the metastatic lesions in the lesion recovers for 3-6 weeks, but most ol the pa
sarcoma of Kaposi and the primary glial tumors. tients are progressing towards the secondary stage of
Different types of myositis and myopathies are de systemic spirochaetemia. It is manifested by flu-like
veloping in opportunistic infections with Staphylococ symptoms, non-itching skin rash, involving the hands
cus aureus, Pneumocystis carini, Toxoplasma gondi. and feet and generalized lymphadenitis. If left untreated
The sarcoma of Kaposi and non-Hodgkin lymphoma the patients with secondary syphilis are recovering in
weeks or months. The next stage is those of the latent
may infiltrate the muscles.
The toxic complications o f the therapy are most of asymptomatic period, after which 1/3 of patients alter
ten connected with nucleoside reverse transcriptase months and years are entering the tertiary stage with
96 / Clinical Neurology
involvement of the cardiovascular and nervous system. Meningovascular syphilis o f the spinal cord. It is
The invasion of CNS happens during the hematogenic manifested as transversal myelitis or spinal stroke with
dissemination, when CSF changes are observed in 40% lower paraparesis, conductive sensory loss and pelvic
of the affected. reservoir symptoms. More rarely are observed syn
Patomorphology. In the course of the primary neu drome of ALS and spastic paraparesis of Erb.
rosyphilis the meninges are infiltrated by lymphocytes Gummouse neurosyphilis affects the leptomeninges,
and other mononuclear cells. In the stage of the menin basal cisterns and cerebral parenchime and may cause
govascular syphilis the chronic basal meningitis in intracranial hypertension and focal neurological symp
volves the cranial nerves and blood vessels (the atreriitis toms, resembling cerebral tumor.
of Nilss-Alzheimer affects the small-sized blood vessels The parenchymatous neurosyphilis is elicited more
and the arteriitis of Heubner involves the medium-size than 10 years after the primary infection. It results of
blood vessels) with subsequent thrombosis and more chronic progressive meningomyelitis and meningoen
rarely aneurisms. In meningeal-gummous lues forma cephalitis. It is demonstrated by two types of disorders
tion of the so-called gummas is observed - tumor-like - tabes dorsalis and paralysis progressiva.
avascular lesions resulting of cell-mediated immune Tabes dorsalis was the most frequent form of neu
response to tremonema. In tabes doralis is observed rosyphilis before the era of antibiotics. The disease is
inflammatory infiltration and degeneration of the pos also termed progressive locomotor ataxia. It is develop
terior roots and demyelination of the posterior funiculi. ing from 10 to 20 years after the primary infection. Three
The pathologic process involves the optic nerve and the stages are observed in the course of the disease: preatax-
grey matter in midbrain also. The progressive paralysis ic, ataxic and paralytic. During the preataxic stage dom
is due to a chronic meningoencephalitis with perivas inate the excitatory dorsal roots signs - strong piercing
cular and cortical inflammatory infiltrates, ependimitis encircling pains or pains irradiating to lower limbs (tabes
and cerebral atrophy and gliosis. dolorosa). Due to the involvement of the autonomous fib
ers appeared pharyngeal and laryngeal spasms, spasm of
Clinical syndromes. esophagus, bronchial spasms, and stomach aches with
Asymptomatic neurosyphilis. Clinical symptoms are vomiting of hematinic materials (crises noires of Char
absent but the syphilitip blood and CSF samples are pos cot). Due to the pain sensation loss from the internal or
itive. The CSF pleocytosis is above 5х10л6/1. The me gans is possible painless asymptomatic and fatal man
ninges are visualized hyperintense on MRI. ifestation of visceral disorders. Primary atrophy of the
Meningeal neurosyphilis (Acute syphilitic meningi optic nerves is observed. The pupils become narrower,
tis). It is observed during the first 2 years after the infec non-reactive to light, while the reaction of accommoda
tion. The clinical picture resembles those of other types tion is preserved (syndrome of Argyll-Robertson). Typ
of serous meningitis - headache, nausea, vomiting, ical finding in the ataxic state is the dissociated senso
neck rigidity, and hyperpyrexia. The CSF pressure is ry loss - severely reduced proprioceptive sensation and
increased, the CSF pleocytosis reaches 400-500х10л6/1 relative preserved pain and temperature senses. Due to
and the glucose is in normal ranges. The clinical symp the disturbed joint-position sense sensory ataxia is devel
toms are recovering spontaneously. The diagnosis can oping - positive Romberg test. The gait is broad-based
be omitted unless targeted serological tests in serum (tabetic gait).The denervation of the dorsal roots leads
and cerebrospinal fluid are performed. The affection of to muscle hypotonia, knee and Achilles areflexia. In the
VII, VIII, VI and II cranial nerves, hydrocephalus, the terminal stage standing and walking become impossible;
acute meningitis and encephalitis are infrequent com trophic ulcers occur (mal perforans); osteopathias and ar
plications. thropathias are observed (joints of Charcot).
Meningovascular neurosyphilis. It is observed usu Progressive paralysis (Paralysis progressiva).
ally more than 5 years after the primary infection. It The disease is developing more than 10 years after
results of chronic meningeal granulomatous infiltra the primary infection. It is due to a chronic polioen
tion and obliterative endarteritis of the small and me cephalitis with predominant cortical involvement and
dium-sized blood vessels. The clinical presentation is development of progressive cerebral atrophy. The dis
with non-typical symptoms - headache, vertigo, behav ease is manifested by psychiatric symptoms - emotional
ioral, emotional and memory disturbances, and subse changes (mania, depressive disorder), impairment in the
quent progressive vascular syndrome. Relapsing cere mentation (delusions of greatness, paranoia), auditory
bral strokes are observed. The syphilitic stroke comes hallucinations and progressive dementia.
in younger age of the patient, absence of risk factors Congenital neurosyphilis. Treponema pallidum af
for vascular disease, positive serologic samples and in fects the fetus between the 4 and 7 months of pregnan
flammatory signs in CSF. When the small-sized blood cy. Typical findings are hydrocephalus and the triad of
vessels of the cerebral cortex are affected is observed Hutchinson (keratitis, teeth deformities and deafness).
development of progressive dementia. Due to the basal Of diagnostic value is the examination of the CSF
localization the cranial nerves are involved, the CSF and the serologic tests. The presence of positive VDRL
flow is disturbed with development of hydrocephalus or FTA-ABS tests in serum, positive VDRL in CSF and
and intracranial hypertensive syndrome. pleocytosis confirm the diagnosis. There is lymphocytic
12. Neurointections - meningitis and encephalitis / 97
and plasmocytic pleocytosis in the CSF. When the ther and PNS and of immune mechanisms of T-cells activa
apy with Penicillin is effective the pleocytosis decreases tion and secretion of inflammatory cytokines. Borrelia
in 12 weeks. The protein content is increased and the do not evoke lasting immune response
glucose is in normal ranges. Gamma-globulins are in Pathomorphology. In the early stages of neurobor-
crease in oligoclonal or polyclonal manner. reliosis are observed inflammatory infiltrates in menin
The isolation of Treponema pallidum is difficult and ges, around the blood vessels and the roots of the cranial
is not used for diagnostic purposes. The serologic tests and spinal nerves and multifocal or diffuse panencepha-
are divided to specific and non-specific. litic and myelitic lesions. In the later stages predominate
1) With non-specific tests are examined non-trepone- the degenerative changes, with neuronal loss, astrocyto-
mic antibodies towards reagin with the complement-fix sis, microglial activation, perivascular areas of demy-
ating test of Wasserman and the more sensitive VDRL elination in the white matter, axonal degeneration and
(Veneral Disease Research Laboratory) and RPR (rapid demyelination of the peripheral nerves.
plasma reagin). These tests are used for screening and Clinical features. Lyme disease goes through three
monitoring of the therapy. If VDRL test is positive in the different stages.
CSF, the result is of diagnostic value for neurosyphilis. In the first stage at the site of the bite 3 to 30 days
2) More specific are the tests with treponemal anti after the primary infection occurs typical erythematous
gens - FTA (Fluorescent Treponemal antibody), FTA- rash (erythema migrans) that increases to the periph
ABS and MHA-TP (Microhemaglutination Assay for ery and fades centrally. It can be accompanied by mild
antibodies to T. pallidum). These tests are highly specif general infectious syndrome - hyperpyrexia, fatigue,
ic and demonstrate current or past infection. The nega myalgia, arthralgia, regional lymphadenitis. This stage
tive FTA in the CSF excludes the diagnosis. VDRL and can result in spontaneous recovery in 3-4 weeks. The
some of the other tests can be false positive in minimal clinical neurological symptoms are observed during the
admixture of blood to the CSF. The most sensitive test is second and third stage and are divided to early and late.
TPI. Currently PCR for identification of the treponemal There is a wide variety of symptoms due to the involve
genome is applied. ment of CNS and PNS with acute, subacute and chronic
The differential diagnosis includes a wide range of character.
diseases in the different stages and forms of the neu During the second stage are observed lymphocytic
rosyphilis: serous meningitis, vascular disorders, radic meningitis, meningopolyradiculoneuritis, radiculoneu-
ulopathies, spinocerebellar ataxia, diabetic and alcohol- ritis, multiple mononeuritis, encephalitis, myelitis.
4c polyneuropathy. The Lyme meningitis sometimes has asymptomatic
Treatment. The standard treatment in the early syph course with inflammatory CSF changes only, or mani
ilis is with Benzathin-Penicillin 2.4 million UI i.m. or fests as chronic basal meningitis lasting several weeks.
Doxycycline 100 mg twice daily for 14 days, or Azitro- In the clinically manifested cases is observed headache,
mycin 2 g. The treatment is successful in 4-fold reduction photophobia, and mild neck rigidity. The CSF exami
in VDRL titer on the 3rd month and 8-fold reduction in nation demonstrates lymphocytic pleocytosis, and in
the titer on the 6,h month. In the stage of neurosyphilis is creased levels of protein and gamma-globulins with
applied Penicillin G 18-24 million UI daily i. v. for 10-14 oligoclonal bands. The meningitis is accompanied in
days. In cases of allergy to Penicillin is used Ceftriax 30-50% of the cases by peripheral nerves affection.
one i. v. The results of the treatment are monitored with The lymphocytic meningopolyradiculoneuritis is the
serum tests on every 3 months and with CSF tests - on most frequent presentation of the European variant of
every 6 months to normalization of the results. the Lyme disease. In acute development the clinical pic
ture resembles the syndrome of Guillain-Barre but with
CSF pleocytosis.
12.4. NEUROBOR HELIOSIS (LYME Cranial neuritis may be the only clinical presenta
DISEASE) tion. It is observed in 50% of the cases with neurobor-
reliosis. The most typical manifestation is unilateral or
Lyme disease is antropozoonzis, in which reservoirs bilateral facial paralysis, in which are detected inflam
of infection are rodents, and some domestic and wild matory changes in the cerebrospinal fluid. Rarely other
animals. The causative agent is Borrelia burgdorferi and cranial nerves are affected with the clinical picture of
is identified in 1981. The disease is transmitted by ticks migrating cranial neuritis.
of the genus Ixodes. The Infection occurs trom a tick The combination of lymphocytic meningitis, pain
bite. Other possible mechanisms are via conjunctival in ful radiculoneuritis and cranial neuritis is known as the
t r i a d o f Garin-Bujadoux and is typical for the disease.
fection and transplacental. The disease is seasonal - late
The multiple neuropathies have subacute develop
spring and early summer.
Pathogenesis. The infectious agent proliferates at ment and recovery in 3-6 months. They proceed with
the site of the bite and cause local skin reaction. The asymmetric sensory and motor changes. EMG shows
following stage is of spirochaetemia and dissemination data for both axonal degeneration and demyelination.
The Lyme encephalitis is manifested with mild gen
in lymph nodes, meninges, heart, eyes, joints. The in
volvement of NS is result both of direct invasion ot CNS eral cerebral and various focal signs. It is observed more
98 / Clinical Neurology
of the cyst can develop multiple hydatid disease of the subarachnoid space and in the ventricles and can lead to
brain. There is a risk of allergic reactions including an internal hydrocephalus.
aphylaxis. The clinical picture depends on the number and
Clinical picture. The cerebral Echinococcus may location of cysticercs and the reactivity of the organ
persist long time asymptomatic with atypical headache ism. Four clinical forms are distinguished: hemispheric,
and general symptoms - loss of appetite, fatigue, itch cistern-basal, ventricular and spinal. In the hemispher
ing, hives. Upon reaching larger sizes or in compres ic form the cortical cysts cause partial or generalized
sion of cerebrospinal fluid pathways and veins develop seizures, less often negative focal events - hemiparesis,
symptoms of increased intracranial pressure - head hemihypoesthesia, aphasia or cerebellar ataxia. In acute
ache, vomiting, congestive papilla. The focal syndromes course the miliary cysticercs appear with the clinical
depend on the location of the cyst - epileptic seizures, picture of encephalitis. In cistern-basal form develops
hemiparesis, spinal compression with lower paraparesis, meningeal syndrome combined with cranial nerve in
etc. Upon reaching larger dimensions dislocation occurs volvement, development of hydrocephalus and intrac
in the brain structures with a danger of herniation. ranial hypertension. In the ventricular form the mobile
The diagnosis is easy if a history of contact with cysts cause “valve” effect with hydrocephalus crises,
dogs and stay in endemic areas is available and if cysts while in cysts of the IV ventricle are observed the so-
are detected in other organs. CT and MRI visualize called Bruns crises - the sharp reversal of the head caus
rounded low-density formation without visible capsule. es headache, dizziness, vomiting, slow heart rate and
In craniography can be established “skull pressure”, respiratory disorders. Sometimes even in asymptomat
calcified parasite or local thinning of the cranial bone ic patients may develop sudden death. The spinal form
in superficial cysts. In cerebrospinal fluid is revealed a manifests as cervical extramedullary or intramedullary
slight pleocytosis with presence of eosinophils, hyper- process.
proteinorachia and succinic acid. Biopsies of the cysts The diagnosis is easier when history for reside in
are not performed because of the risk of dissemination. endemic areas is available, if palpating a seal in the
The diagnosis is confirmed by the reaction of passive skin, subcutaneous tissue and muscle and in typical
hemagglutination (RPHA), enzyme immunoassay reac clinical picture - a combination of epilepsy, increased
tion (ELISA) and the reaction of immunofluorescence intracranial pressure, meningeal syndrome and inflam
(RIF). matory changes in the cerebrospinal fluid - pleocytosis
Differential diagnosis is made with a brain tumor, with presence of eosinophils. In craniography can be
brain abscess, subdural hygroma, and other parasitic found rounded calcifications, in CT and MRI are dis
and non-parasitic cysts. played multiple rounded hyperintens lesions. For immu-
The treatment of the uni-chambered echinococcus nodiagnostics are applied RPHA, ELISA, and RIF.
is surgical. Medical treatment is conducted with Alben Differential diagnosis is made with brain tumors,
dazole, Mebendazole, Zentele, Praziquantel for months. encephalitis, meningitis, mycotic or tuberculous menin
gitis, toxoplasmosis, sarcoidosis, and multiple sclerosis.
12.6.2. Cerebral cysticercosis The treatment is surgical removal of the available
cysts, placing the shunt in hydrocephalus and adminis
The neurocysticercosis is parasitic infection of the tration of drugs - Albendazol.
CNS and the most common cause of epilepsy in the en
demic areas. 12.6.3. Cerebral toxoplasmosis
Pathogenesis. The causative agent of neurocystic
ercosis is Cysticercum cellulosae - embryonic stage Toxoplasmosis is a protozoal disease that is caused
of pig’s tapeworm (Taenia solium). The infection with by Toxoplasma gondii - a small obligate intracellular
eggs of the porcine tapeworm occurs by oral route with parasite. In the CNS cells are formed pseudocysts con
faecal contaminated food and water or in an endoge taining 10-15 parasites. About 1/3 of the population is
nous anti-peristaltic way (vomiting during anesthesia). positive for antibodies against toxoplasmosis, but the
In the acidic stomach environment the shell of the egg cases of clinically apparent infection are less. A source
is dissolved, and the released embryo pass through the of the infection is domestic and wild animals, most
intestinal wall in the systemic circulation and reach the ly cats. The infection is alimentary, through damaged
tissues - brain, eyes, muscles, skin, liver, and lungs. skin, in blood transfusions and organ transplants and
The nervous system is affected in 50-70% of cases. placental. After the infection in some cases develops
For about two months into the brain develop multiple regional lymphadenitis and antibodies occur, but ac
cysticercs, which have a bubble form with sizes 5-20 tive disease does not develop. In immunosuppressive
mm, containing clear liquid and scolexes (head). Some condition occurs dissemination ai*i toxoplasma most
times larger cysts are formed or they are multivesicular frequently invade the central nervous system and eyes.
(Cysticercus racemosum). In the surrounding tissues an Scattered inflammatory granulomas are observed. Sub
inflammatory reaction occurs and with the passage of acute meningoencephalitis is developing.
time are formed granulomas, which subsequently calci Clinical picture. The toxoplasmosis is divided to
fy. The cysticercs are located in the brain parenchyma, congenital and acquired.
12. Neuroinfections - meningitis and encephalitis / 101
The congenital form is due to transplacental infec muscle biopsy. The blood samples demonstrate elevated
tion, leading to encephalitis and chorioretinitis of the creatine kinase and eosinophilia.
fetus. The result is a miscarriage; stillbirth or newborn The differential diagnosis is very extensive - gastro-
babies are born with various defects - microcephaly, enterocolitis, acute viral infections, edema of Quincke,
microphthalmia, hydrocephalus, deafness, blindness, dermatomyositis, and in CNS involvement - primary
cerebral palsy, brain calcifications, and developmental and secondary meningoencephalitis.
delay. At birth the newborns may have signs of active The treatment is carried out with Mebendazol, Zen-
infection - fever, rash, liver and lien enlargement, and tele, and Tiabendazole.
meningoencephalitis.
The acquired form usually develops in immunosup- 12.7. CEREBRAL ABSCESS
pressed people, and is especially common in AIDS, due to
reactivation of the pathogen. The course is acute, subacute The brain abscess is a pus collection lesion in the
or chronic with the formation of granulomas, abscesses; brain parenchyma (encephalitis circumscripta purulen-
development of encephalitis, meningoencephalitis and ta), surrounded by inflammatory capsule. The frequen
chorioretinitis. The acute form manifests with fever, rash, cy is small - 0.5 / 100 000, but the prognosis is very
lymphadenitis, myocarditis and development of menin serious. It is more commonly observed in patients with
goencephalitis with focal and general cerebral signs. The AIDS and other immunodeficiencies, congenital heart
chronic form occurs with low-grade fever, headache, sei disease, diabetes mellitus. With the more frequent use
zures, and intellectual degradation. In adults the toxoplas of broad spectrum antibiotics and corticosteroids the
mosis has mostly asymptomatic course, which requires increasing incidence of fungal abscesses is observed.
tests conducting in pregnant women. As a result of the modern antibiotic therapy and the op
The diagnosis is verified by the detection of Toxo portunities created by the imaging methods mortality is
plasma gondii in CSF sediment or by lymph node biop reduced to 10-15%.
sy. The following immunological tests are applied: ELI Depending on their location the abscesses are sub
SA, RIF, RPHA, PCR, reaction of Sabin-Feldman, patch divided to intracranial and spinal. The intracranial ab
testing. In craniography are visualized calcifications in scess is divided into intracerebral, subdural and epidur
the brain, in CT - periventricular calcifications and hy al abscess (empyema).
drocephalus, in MRI - scattered areas of inflammatory Etiology and pathogenesis. The cerebral abscess
necrosis, granulomas and micro abscesses. causative agents are bacteria - Gr + (Staphylococcus au
. The differential diagnosis includes infectious dis reus, Streptococcus and others.), Gr- (E.coli, Proteus,
eases (infectious mononucleosis, rubella), hematologic Pseudomonas aeruginosa, Enterobacter), anaerobes
diseases, tuberculosis, cryptococcosis, encephalitis and (Bacteroides), in immunocompromised - Candida, As-
meningoencephalitis, neoplasms and others. pergilus, Toxoplasma gondi, Nocardia. The infection
The treatment is carried out with Spiramycine, Py- can result of adjacent focal inflammatory processes -
rimethamin, and Sulfadiazin. otitis media, mastoiditis and sinusitis with subsequent
focal osteomyelitis, which leads to extradural or sub
12.6.4. Trichinelosis dural abscess. The second mechanism is through veins
- the thrombophlebitis of mucous veins of the paranasal
The trichinosis is a helminth disease that is caused sinuses spreads in retrograde way towards the emissary
by the intestinal nematode Trichinella spirallis. The in veins of the skull, dural venous sinuses, subdural and
fection occurs by eating raw or improperly heat-treated cerebral veins and thus develops the subdural empyema
pork or wild game meat containing encapsulated larvae and brain abscess. The direct inoculation of the infec
of the parasite. In stomach the larvae are released and tion occurs in open cranial cerebral trauma and neuro
in hematogenous way reach tissues. Only the larvae surgical operations. In 25% of cases is observed hae-
reached muscles and brain survived. They form cysts matogenous dissemination most frequently in the basin
and granulomas and, optionally, calcify. The trichinosis of the middle cerebral artery, thereby forming multiple
causes sensitization of the body and has metabolic-toxic abscesses. Source of infection is endocarditis, lung ab
action of curare-like effect. scess, empyema, bronchiectasis, congenital heart mal
The incubation period is 2-45 days. Early symptoms formations with right-to-left shunt and others. In 20% of
are those of gastroenteritis, fever, muscle tenderness, the cases the source of infection remains unclear (cryp
fatigue, swelling and redness of the eyelids and con togenic infection). Depending on the source of infection
junctiva. The involvement of tongue and oculomotor abscesses are divided to: 1) Rhinogenic (sinugenic) - in
nerves is typical. In severe cases the cardiac muscle is frontal sinusitis the abscess is localized in the frontal
affected with tachycardia and arrhythmia. Sometimes lobe, while in sphenoidal - in the medial basal parts of
meningoencephalitis is observed - meningeal syndrome the temporal lobe, 2) Otogenic - in otitis media and mas
with headache and inflammatory CSF changes, general toiditis the abscess is localized in the temporal lobe or
cerebral syndrome and focal signs. in cerebellum.
P a t h o m o r p h o lo g y . The formation of the abscess
The diagnosis is made with compressive trichinos-
copy, immunodiagnostic tests (ELISA, ТРИА, RIF) and goes through four stages: early cerebritis - 1-3 day; late
102 / Clinical Neurology
cerebritis - 4-9 day; early abscess with formation of the pears with the increase of the intracranial pressure. Very
capsule - 10-13 day; late capsulated abscess - after the important is the medical history of prior inflammatory
14lh day. In the cerebritis stage develops focal inflam bacterial disease, most commonly in the area of the head
matory infiltration with neutrophils and macrophages, - otitis, sinusitis, and dental infections. The blood tests
necrosis of the brain parenchyma, septic thrombosis of reveal increased sedimentation rate, mild leukocytosis
the vessels and perifocal edema. Then, the central area and positive C reactive protein. The CSF examination
is filled with pus and circumferentially fibroblasts pro demonstrates inflammatory changes, but the examination
liferate to form a capsule which is built at the beginning is not recomended because risk of cerebral herniation ex
of vascularized granulation tissue, and later - of colla ists. The location, size, stages of abscess formation and
gen connective tissue. perifocal edema are visualized by CT and MRI as low
Clinical picture. The course can be slow and im density area bounded by a dense annular shaft and pro
perceptible with gradual progression of the symptoms nounced perifocal edema. The microbiological diagnosis
or rapid and fulminant. At the initial stage of focal is performed by examination of material from the abscess
encephalitis the clinical picture is dominated by high, taken by needle aspiration under CT control.
sometimes septic temperature to 39-40°, severe head The differential diagnosis includes meningitis, ex
ache, decreased consciousness, generalized epileptic tradural or subdural empyema, cerebral infarction, cer
seizures, meningeal symptoms, hemiparesis and other ebral hemorrhage, primary or metastatic tumor, throm
focal symptoms. After the formation of the capsule the bosis or trombflebitis of dural sinuses, echinococcal
clinical presentation is of space occupying process - cyst, tubercle, a large plaque of demyelination, etc.
headache, vomiting, papilloedema, compression of III The treatment is neurosurgical - aspiration, exci
and VI cranial nerves combined with focal neurologi sion and drainage, antibiotics and anti-edema agents.
cal symptoms. The intracranial hypertension depends The antibiotic treatment is empirical - a combination of
on the volume of the abscess and the perifocal cerebral cephalosporins III generation, Methronidazole, Vanco
edema. The most serious complications include: 1) rup mycin, Amikacin, and Chloramphenicol. In the selec
ture of the abscess into the ventricles or less often to the tion of antibiotics are taken in mind the results of the
subarachnoid space with the development of meningitis microbiological examination and the antibiogram of the
and 2) dislocation with cerebral herniation due to the primary focus. The sinugenic abscesses are caused most
severely elevated intracranial pressure. often by streptococci, otogenic - of mixed anaerobic
The diagnosis is based on the presence of the triad flora, those after injury - by staphylococci. The brain
of fever, headache and focal neurological symptoms. The edema is treated with Mannitol and corticosteroids, but
most frequent and early symptom is headache, which is these therapeutic agents may reduce the penetration of
initially local and then becomes diffuse; vomiting ap antibiotics through the blood-brain barrier.
13. Demyelinative diseases 103
The demyelinative diseases are characterized by es) the virus persists in a latent form, and in reactivation
destruction of the myelin shelves while the axons are after years causes clinical relapse.
relatively preserved. Due to the etiology they are di The autoimmune pathogenesis is supported by the
vided into: 1) Inflammatory (Acute postinfectious and similarity with the experimental allergic encephalomy
postvaccinal disseminated encephalomyelitis; Multiple elitis, which is evoked by injection of myelin proteins of
sclerosis; Tropical spastic paraparesis; Opticomyelitis), genetically susceptible animals and can be transmitted
2) Metabolic (Central pontine myelinolysis; Hypoxic to animals by activated T cells. It is assumed that MS is
encephalopathy), 3) Resulting of deficiency states (Sub due to the activation of the autoreactive T cells that have
acute combined degeneration of the spinal cord due to escaped the thymic control, through the mechanism of
vitamin B12 deficiency). “molecular mimicry” (molecular similarity) of the path
ogen with myelin basic protein or other proteins during
the viral or bacterial infection. Another mechanism for
13.1. MULTIPLE SCLEROSIS (MS) non-specific activation is by superantigens or by a high
level of cytokines during the inflammatory response.
Multiple sclerosis (MS) is an inflammatory demyeli- The activated T-cells are transfered in the CNS and in
nating illness, characterized by relapses and remissions, the meeting with similar antigen provoke clonal expan
affecting predominantly young and middle-aged people sion of T cells, activation of macrophages, B-cells, se
and often resulting in disability. cretion of inflammatory cytokines and antibodies with
Epidemiology. MS most commonly starts between subsequent demyelination.
15 and 50 years of age. The women are suffering twice Pathomorphology. Typical lesions are the plaques
as often as men. MS has a unique geographical and ra o f inflammatory demyelination, scattered in the white
cial distribution. The incidence increases with moving matter with a predilection in the cervical portion of the
away from the equator and decreases again in the Polar spinal cord, brainstem, cerebellum, corpus callosum,
Regions. The highest morbidity is observed (over 30 to optic nerves, and around the ventricles. The lesions are
100-200 / 100,000) in Northern Europe, North America, arranged beside the small veins, around which there are
South Canada, South Australia and New Zealand. Mod inflammatory infiltrates of T lymphocytes, macrophag
erate frequency (5-30/100 000) is established in the oth es, and less B lymphocytes and plasmocytes. The axons
er parts of Europe (Bulgaria over 40/100 000). Very low are demyelinated, the macrophages are activated and
(less than 5/100 000) is the disease frequency in Africa, contained myelin fragments. In the inactive plaques
Asia and South America. This distribution is explained grows astroglia and occurs sclerosis (hence the name
by the higher predisposition to disease development of multiple sclerosis), the axons are damaged, the number
Caucasians and with environmental factors - climate, of the oligodendrocytes is decreased and the opportuni
soil and water composition, diet, hygiene habits, and ur ties for remyelination are reduced.
banization. Pathophysiology. The demyelinated axons conduct
The etiology still remains unclear. It is assumed that nerve impulses at a low speed and can generate ectopic
one or more exogenous factors (for example, viral infec action potentials in response to mechanical stimuli (for
tion), following a long latency period provoke the dis example, electric shock sensation with irradiation along
ease onset in genetically susceptible individuals. the spinal cord and limbs resulting from neck bend
The genetic predisposition can explain the greater ing- a phenomenon o f Thermite). The conduction ot the
incidence among Caucasians and monozygotic com impulses disturbs upon increase in body temperature
pared to dizygotic twins. About 15% of patients have (worsening eyesight - phenomenon o f Uhthoff). In large
relatives with MS. It is now accepted that the disease plaques occurs conducting block, which is the cause of
predisposition is polygenic and is associated with HLA- persistent neurological deficit.
DR15, DQ6, Dw2, with the genes for the T-cell receptor, The clinical picture is rich and varied. The most
common initial symptoms are sensory and motor dis
for immunoglobulins, and for some cytokines.
The viral etiology is supported by the presence of turbances, followed by visual disturbances. Most pa
viral demyelinating diseases in animals and in man tients develop subacute complaints within a few days to
weeks. An acute stroke-like beginning or very slow and
(HTLV-l-associated tropical spastic paraparesis, pro
gressive multifocal encephalopathy which is due to chronic progressive course is possible.
Motor disturbances. They are due to the involvement
infection of oligodendrocytes with JC-virus, subacute
of the pyramidal tracts with development of spastic mus
sclerosing panencephalitis, etc.). It assumed that after a
cle weakness. At first occur weakness and tightness in
viral infection at an early age (HSV-6, EBV, retrovirus
104 / Clinical Neurology
one or both lower extremities, more rarely the weakness nence due to detrusor’s hyperreflexia. In later stages as
involves one side of the body and subsequently trans a result of sphincter spasticity urinary retention can oc
forms to quadraparesis with severe lower spastic para cur. In more than 50% of the patients develops erectile
paresis. The examination reveals deep tendon reflexes dysfunction and other sexual disorders.
hyperreflexia, abdominal reflexes areflexia, and abnor From the psychic disorders in the early stages pre
mal reflexes from the group of Babinski and Rossolimo. vails depression, and in the later stages - the euphoria
The muscle tone is spastically increased, especially in and cognitive decline.
standing position. The fatigue syndrome is common in MS. It is de
The sensory symptoms at the beginning are of the scribed as lack of energy, motivation and a sense of
type of paresthesia (numbness, tingling) and dysesthe physical exhaustion, not associated with muscle weak
sia in one or both legs, later with formation of sensory ness and depression. It worsens after physical exertion,
conduction level or are distributed in the 4 limbs. They stressful situation and fever.
result of plaques involving the posterior funiculi. In the The paroxysmal symptoms are typical of MS - they
cases of plaques involving the spinothalamic pathways last seconds to 2-3 minutes and repeat many times -
are observed thermal paresthesia - sense of limb perfu painful tonic spasms, paresthesias, dysarthria, ataxia,
sion with hot or cold water. The plaques in the posteri facial myokymia, and trigeminal neuralgia.
or funiculi lead to instability when the eyes are closed Clinical forms and course of the disease. Currently
and cause the so-called symptom of “useless” hand due is accepted the presence of the so-called clinically iso
embarrassed joint-position and vibration sense. The lated syndrome as a primary manifestation of MS. Four
plaques located in the brain stem can evoke numbness clinical forms are distinguishedt: a) relapsing-remitting,
of the face, trigeminal neuralgia. b) secondary progressive c) primary progressive and d)
The visual disturbances are initial manifestation in progressive relapsing. Most common is the relapsing re
25% of patients. For hours to days occurs reduced vision mitting form (70-80%), where 70% of cases after 10-15
most often in one of the eyes, accompanied by orbital years are transformed into secondary progressive form.
pain. Usually the vision restores partially or completely, The rarest is the primary progressive form (10%) - it af
but later temporal pallor of the papilla is observed due fects age after 40 and demonstrates as progressive my
to the spent retrobulbar neuritis. Half of patients with elopathy. In the benign form of MS (about 'A) patients
isolated optic neuritis then developed MS, the risk of retain functional independence until the 15th year since
which can be evaluated with MRI. the beginning of the disease. The acute MS, concentric
The oculomotor disorders (diplopia) are rarer than sclerosis of Balo, diffuse sclerosis Schilder, and neuro
the visual and are due to plaques in the brain stem, af myelitis optica of Devic are referred to the malignant
fecting fasciculus longitudinalis medialis that leads to form of MS. The nosological affiliation of these diseases
development of internuclear ophthalmoparesis - abnor is not fully understood.
mal adduction of the eye on the side of the lesion and Diagnosis. The examination of the cerebrospinal
nistagmus in abduction of other eye. The disorder can fluid establishes normal or slightly elevated protein con
be combined with horizontal gaze paresis - one and a tent, mild lymphocytic pleocytosis, which is indicative
half syndrome. of the activity of the process and increased and divided
The coordination disorders are due to plaques in into fractions gamma-globulins (oligoclonal bands) in
cerebellum or in its peduncles. Static and locomotor agarose electrophoresis, an increase of immunoglobu
ataxia, dismetria, intention tremor and adiadohokinesia lins, mainly IgG with data for intrathecal synthesis.
are observed. The plaques in the upper cerebellar pe The examination of evoked potentials (EP) aims to
duncle lead to large amplitude and disabling tremor - a detect clinically asymptomatic lesions. The most fre
tremor form of MS. The speech becomes explosive and quent changes are observed in the visual EP, followed
dysarthric. The combination of nystagmus, dysarthria by the somatosensory EP and brainstem auditory EP.
and intention tremor is known as triad o f Charcot. The MRI is the most sensitive method to visualize plaques
gait is ataxic - broad-based, staggering or spastic-atax and revolutionize the diagnosis of MS. The plaques are
ic. In plaques involving the posterior funiculi the stand hypointense T1 (dark) and hyperintense of T2 (light).
ing and walking are disturbed when the eyes are closed They are ovoid, with sizes greater than 5 mm, and are
- positive Romberg’s test. arranged periventricular and perpendicular to corpus
The plaques in the brainstem except for sensory, callosum, in the brainstem, cerebellum and cervical spi
pyramidal, oculomotor and coordination symptoms are nal cord. The infratentorial and the cervical lesions are
responsible for vestibular disorders also - vertigo crisis, typical for MS and are not observed in vasculitis, lacu
central vestibular syndrome with presence of nystag nar infarctions, neuroinfections, and leukodystrophies.
mus. In 5% of the patients develops a peripheral lesion For establishment of the active plaques with impaired
ol the facial nerve, and in the 1-5% - trigeminal neural blood-brain barrier is performed contrasting with gad-
gia. The facial paresthesias are more common than the olinum-DTPA of Tl. In FLAIR sequences - the plaques
neuralgic pains. are visualized better.
The pelvic reservoir disorders can occur early in The diagnosis of MS is made in the presence of at
the course of the disease - urinary urgency and inconti least two relapses and the establishment of at least two
13. Demyelinative diseases 105
lesions and confirmed by the typical changes in MRI, malformations, central pontine myelinolysis, subacute
i CSF examination and EP. combined degeneration of the spinal cord in vitamin
The differential diagnosis is difficult, as there are B12 deficiency state.
no pathognomonic for MS examinations. Many diseas Treatment of MS. The follow ing types of treatment
es resemble MS in their clinical picture, MRI and CSF are conducted: 1) treatment of the relapses - w ith ster
findings: acute disseminated encephalomyelitis, neurob- oids, 2) between relapses - with immunomodulators
orelliosis, HTLV-l-associated tropical spastic parapa and immunosuppressants, and 3) symptomatic treat
resis, progressive multifocal encephalopathy, HlV-en- ment. The attacks are treated with mega doses Meth-
cephalopathy and myelopathy, neurosyphilis, systemic ylprednisolon 500-1000 mg intravenously for 3-7 days
lupus erythematosus, polyarteritis nodosa, Sjogren’s sometimes followed by a rapid decrease for 10-15 days.
syndrome, primary angiitis of CNS, sarcoidosis, para The treatment between attacks is carried out with In
neoplastic encephalomyelitis, primary CNS lymphoma, terferon-beta lb, Interferon-beta la, Glatiramer acetat
brain metastases and parasitosis. and currently - with a humanized monoclonal antibod
Another group of diseases resemble MS in their clin ies against adhesion molecules (Natalizumab) and oral
ical picture and MRI findings, but differ in CSF chang DMT (disease modified therapy) - Fingolimod, Alemtu
es: Binswanger’s disease, multiple embolisms, strokes zumab, Dimethylfumarat, Teritlunomide. The sympto
and transient ischemic attacks, Sneddon’s syndrome, matic treatment aims to infiuence the spasticity (muscle
MELAS (mitochondrial encephalopathy), CADASIL relaxants), the intention tremor and urinary urgency
(cerebral autosomal dominant arteriopathy with subcor (anticholinergic medicaments), the pains, fatigue and
tical infarcts and leukoencephalopathy), arteriovenous psychic disturbances.
106 / Clinical Neurology
Cerebrovascular disease (CVD) is the most common nicans posterior - posterior trifurcation. Both carotid
cause of neurological deficit and death among the neu systems perform the so-called anterior circulation of
rological diseases. the brain and the vertebral-basilar system - posterior
Anatomy of cerebral blood flow (fig. 14.1 and cerebral circulation. Through the anterior and the two
fig.14.2). The brain blood supply is performed by four posterior communicates arteries at the base of the brain
main arteries: two internal carotid arteries and two ver is formed the circle of Willis (circulus arteriosus cere
tebral arteries. The common carotid artery (a.carotis bri), which ensures a collateral circulation in cerebral
communis) is separated from the aortic arch on the left artery occlusion.
and from truncus brachiocephalicus on the right. At the Physiology and pathophysiology of the cerebral
level of C4 vertebra it is divided into a.carotis interna circulation. The brain in adult weighs 1500 g or 2% by
and a.carotis externa. The internal carotid artery enters weight of human, and cerebral blood flow constitutes
the skull through canalis caroticus and passes through 15-20% of the cardiac output. Every minute 350 ml of
sinus cavernosus, making a curve (carotid siphon). It blood is supplied through the carotid system and 100-
gives the following branches: 1) a. ophtalmica (anas 200 ml through the vertebral-basilar system and so the
tomosed with extracranial vessels); 2) a. communicans normal total cerebral blood flow is 50-55 ml / min / lOOg
posterior (anastomosed with a. cerebri posterior); 3) brain tissue. The reduction of the cerebral perfusion be
a. chorioidea anterior (anastomosed with a. chorioidea low 23 ml / lOOg / min causes loss of consciousness,
posterior, branch of a. cerebri post); 4) a. cerebri ante and below 10 ml / lOOg / min - necrosis (cerebral infarc
rior and 5) a. cerebri media. The anterior cerebral ar tion). The brain has a high aerobic metabolism, which
tery connectes with the eponymous artery on the other requires a high consumption of oxygen - 3,0 - 3,5 ml /
side through a. communicans anterior, travels along the lOOg / min or 72 I / 24h. This is 20% of the total oxygen
medial hemispheric surface and supplies the medial, ba
sal and a small port of the lateral surface of the frontal
lobe, a part of the medial surface of the parietal lobe (in
cluding lobulus paracentralis), and the anterior part of
corpus callosum and basal ganglia. The arising of both
anterior brain arteries from one of the internal carotid
artery is possible - anterior trifurcation. The middle cer
ebral artery is a direct continuation of the internal ca
rotid artery and travels into the Silvian furrow between
the frontal and temporal lobe. It gives deep branches for
the basal ganglia and internal capsule (lateral and medi
al aa. lenticulostriatae) and then gives frontal, parietal
and temporal branches for blood supply to the lateral
parts of the hemisphere.
The vertebral artery (a. vertebralis) originates from
a. subclavia, at the level of C6 vertebra enters the verte
bral bone channel formed by the vertebral proc. trans
verse, passes into the cranium through foramen mag
num and gives descending branches of the spinal cord
(aa. spinales post, and a.spinalis ant.) and a.cerebelli
inferior posterior. At the level of pons the two vertebral
arteries unite to form the basilar artery (a. basilaris),
from which divides a. cerebelli inferior anterior, a. cere-
belli superior, and median and paramedian branches for
the brainstem. At the level of mesencephalon the basi
lar artery divides into left and right a. cerebri posteri
or. The posterior cerebral artery supplies the occipital
lobe, the basal surface of the temporal lobe, a part of
thalamus and mesencephalon. Sometimes the posterior
cerebral artery receives blood supply from a. commu
14. Vascular diseases of the nervous system / 107
3. Thrombosis of cerebral veins and dural sinuses 24 hours. They result from acute disorders of region
4. Disorders of the spinal blood circulation al brain blood supply and have highlighted tendency to
relapse. They are known under the name transient is
chemic disturbances o f the cerebral blood flow.
14.1. ASYMPTOMIC FAILURE OF Etiology and pathogenesis. The most common
THE BRAIN CIRCULATION cause is atherosclerosis of extra- and intracranial vessels
with a formation of plaques that are a source of emboli
The asymptomatic failure of the brain circulation or disturb the cerebral vascular flow by a hemodynamic
(AFBC) is the earliest stage of the cerebral vascular dis mechanism. Heart diseases such vices, artificial valves,
ease. It occurs with nonspecific neurological and psychi rhythm and conduction abnormalities, coronary artery
atric disorders, but without focal neurological symptoms. disease and heart attacks lead to TIA by embolizations
By instrumental methods vascular pathology is detected. and in hemodynamic way. Other causes are hyperten
Etiology and pathogenesis. The main reason for sion, diabetes, vascular malformations (abnormal cur
AFBC are atherosclerosis and hypertension. They are vature, stitches, hypoplasia), cervical spondylarthrosis
the foundation of asymptomatic stenosis of extra- and compression of vertebral arteries, cerebral vasculitis,
intracranial blood vessels including in perforating arter blood disorders leading to increased viscosity (throm
ies with development of asymptomatic lacunar infarcts. bocytosis or erythrocytosis), acute anemia combined
Some cardiac diseases such as atrial fibrillation are also with vascular stenoses, antiphospholipid syndrome, etc.
important. Risk factors include diabetes, hyperlipidem The age and gender were significant risk factors - inci
ia, high blood viscosity, overweight, bad habits (smok dence over 65 years averaged 1.5 / 1000 for men and 1.0
ing, alcohol abuse, drug use, etc.). The cerebral athero / 1000 for women.
sclerosis increases the cerebral vascular resistance and A major pathogenetic mechanism for TIA is the mi
causes a decrease in the regional cerebral blood flow. In croembolisation from ulcerated atherosclerotic plaques
these areas the extraction of oxygen (critical perfusion of the aorta and principle vessels of the brain (most of
- “misery perfusion”) increases and in some situations ten of the carotid bifurcation) and of the left atrium in
requiring increased cerebral blood flow decompensation atrial fibrillation. More rarely it is due to paradoxical
of reduced cerebral blood flow occurs. In combination embolism of peripheral circulation in persistent foramen
of vascular stenosis, increased blood viscosity and em ovale. Another mechanism is the reduction of regional
bolization of small arteries lacunar infarcts develop. brain blood supply in conjunction with hypotonic crisis
The clinical picture is atypical - frontal or occipital (overdose of antihypertensive medications, dehydration,
headache, disturbances in attention, memory impair heart rhythm disorders). Increased total or local blood
ment, decreased mental functions, emotional lability, viscosity in combination with arterial hypotension and
tinnitus, dizziness, insomnia, depression or anxiety ten vascular stenosis can develop TIA. The compression of
sion. These symptoms are initially erratic - after over the principle arteries for example, by vertebral cervical
exertion or stress. osteophytes and disc protrusion resulting of rotation of
The diagnosis is based on the neuropsychological the head is also a cause of TIA. In steal syndromes, for
tests, detection of asymptomatic carotid stenosis with example subclavian steal syndrome due to stenosis of a.
Doppler sonograthy, visualization of asymptomatic cer subclavia in physical exertion of the arm, there occur
ebral infarcts (“silent infarcts”) by CT / MRI, and leuco- direction of the blood through the ipsilateral vertebral
araiosis (hypodense changes around the ventricles and artery and TIA can occur. In brain blood supply reduc
in the white matter of the brain). tion of less than 20 ml / lOOg / min changes occur in the
The prevention is complex - treatment of the risk brain metabolism and functions that are reversible and
factors and change of lifestyle. with complete recovery. In more prolonged regional hy
The treatment is carried out with: 1) medicaments poperfusion lacunar cerebral infarction develops.
which have neuroprotective, nootropic, antiplatelet, The clinical picture is with acute onset and stereo
rheologicaly active, and antioxidant and vasodilating typical course. TIA has a tendency to relapse. The clin
action: Pentoxifylline, Vinpocetine, Nicergoline, Pi ical symptoms are determined by vascular basin with
racetam, Ginko biloba; 2) calcium channel blockers: impaired blood supply.
Nimodipine; 3) statins; 4) antiplatelet agents: Aspirin, TIAs in the carotid system are manifested with cor
Clopidogrel. In the high grade carotid stenosis (over tical symptoms primarily of the middle cerebral artery
70%) a stent is placed, and for unstable atheromatous basin: paresthesias and / or transient weakness in the
plaques endarterectomy is undertaken. contralateral limbs, often in upper limb, and speech dis
orders (dysarthria or aphasia). In 1/3 of patients ipsi
lateral visual disturbances (amaurosis fugax, transient
14.2. TRANSIENT ISCHEMIC ATTACKS monocular blindness) are observed due to ischemia in
the basin of a. ophtalmica. More rarely sensory or motor
TIA (transient ischemic attack) is characterized by transitional Jacksonian seizures are observed.
development of acute focal neurological deficit that lasts TIAs in the vertebral-basilar system are more com
from few minutes to several hours, but never more than mon and occur with transient symptoms of brainstem,
14. Vascular diseases of the nervous system / 109
visual cortex and cerebellum: occipital headache, diz (hemorrhagic infarction) and mixed. The IS represents
ziness, staggering, tinnitus, nystagmus, dysarthria, 60-80% of all strokes. The incidence increases with age
visual disturbances (flashes, scotoma, homonymous and is an average of 2-3 / 1000.
hemianopsia, quadrantanopsia, and cortical blindness), Etiology. The main reason for IS is the atheroscle
sensory and motor disorders (paresthesia and transient rosis of the carotid, vertebral and cerebral arteries with
weakness). Less frequently the so-called “drop attacks” subsequent thrombus formation. A common place is the
are encountered - sudden fall without loss of conscious carotid bifurcation and rarely the carotid siphon. The
ness or sudden feeling of weakness in the legs. Some risk factors for atherosclerosis are age, hypertension, di
times transient global amnesia or dreamlike states of abetes, dyslipidemia, obesity, increased blood viscosity,
consciousness can be observed. smoking, etc. In arterial hypertension fibrinoid necrosis
TIAs with combined carotid and vertebral-basilar and lipohialinosis of the penetrating arteries develop
system symptoms are manifested by a variety of symp with subsequent lacunar infarcts. On second place are
toms of both vascular basins. the heart diseases: atrial fibrillation, artificial valves,
The diagnosis of TIAs is based on: 1) a history of mitral stenosis, myocardial infarction, congenital and
recurrent transient stereotypical clinical symptoms; 2) acquired heart defects, persisting foramen ovale. Oth
acute development of focal neurological deficit that re er causes are: vascular anomalies (hypoplasia, stenosis,
covers within 24 hours; 3) the presence of risk factors abnormal curvature, dolichoectatic vessels), fibromus-
for cerebral vascular disease; 4) detection of athero cular dysplasia, vasculitis and vasculopathies (syphilitic
sclerotic changes in extracranial and intracranial blood arteritis, thromboangiitis obliterans, giant cell arteritis,
vessels by Doppler sonography; 5) detection in 20-40% a disease of Takayasu, vasculitis in drug addicts, etc.).
of patients with CT and MRI of focal ischemia, brain Changes in hemostasis that lead to increased viscosity
atrophy, limited area of brain edema. and hypercoagulability - polycythemia, thrombocyto
The differential diagnosis includes a wide range of sis, protein C and S deficiency, hormonal contraception,
diseases: syncope, vertigo, migraine with aura without antiphospholipid syndrome, an increase of homocyst
subsequent headache, partial sensory or motor seizures, eine, etc. In traumas or spontaneously dissections of
acute hypertensive encephalopathy, a brain tumor, mul cerebral arteries with subsequent obturation are possi
tiple sclerosis, psychogenic disorders, familial paroxys ble. Rare reason are CADASIL, moya-moya, vascular
mal ataxia, etc. compression by tumor, etc.
Treatment and prevention. Primary prevention Pathogenesis. The obstructive IS are due to throm
aims influence upon risk factors: low calorie diet, lim bosis or embolism. Thrombosis develops on ulcerated
iting animal fat and carbohydrate foods, control of hy atherosclerotic plaque. Due to the injury of the endothe
pertension, blood sugar and lipids, heart rate, etc. The lium and prostacyclin deficiency adhesion and aggrega
treatment and secondary prophylaxis is carried out tion of platelets and erythrocytes occur. The thrombus
with antiplatelet agents (Aspirin, Dipyridamole, Clopi- formation is facilitated by increased blood viscosity,
dogrel), vasoactive agents, statins, anticoagulants in increased erythrocyte aggregation, decreased erythro
patients with non-rheumatic atrial fibrillation and pros cyte flexibility, hypovolemia, hyperfibrinogenemia, de
thetic heart valves. In high stage carotid stenosis stents creased activity of antithrombin III, protein C and S,
are implanted, while unstable atheromatous plaques are impaired fibrinolysis, presence of antiphospholipid an
treated by endarterectomy. tibodies, etc. The cerebral embolisms originate from ul
The prognosis worsens by the increased risk of cer cerated plaques of aorta, neck and brain vessels or have
ebral infarction - 10% in the first six months and 6% in cardiac origin in atrial fibrillation, after heart attack,
the subsequent years, especially in patients with high- artificial valves, in dilated cardiomyopathy, persisting
grade carotid stenosis. foramen ovale, etc. The disorders o f the general hemo
dynamics (rhythm disorders, acute hypotension, AV-
block, myocardial infarction, etc.) in combination with
14.3. ISCHEMIC STROKE (CERE atherosclerotic stenosis and insufficiency of the collat
BRAL INFARCTION) eral circulation lead to local hypoperfusion and infarc
tion in the so-called “borderline” areas between the two
The ischemic stroke (IS) is an acute ischemic disor vascular basins. The arterial vasospasm in irritation of
der of the cerebral circulation with irreversible damage the endothelium of embolus and the release of throm
to the brain parenchyma and permanent neurological boxane A2 and serotonin from platelets lead to complete
symptoms. Depending on the pathogenic mechanisms obturation even in small-sized embolus. Vasospasm de
the IS is divided into obstructive and non obstructive, velops in subarachnoid hemorrhage and hypertensive
and according to the course - acute, subacute, chron encephalopathy also, and sometimes in migraine status.
ic, recurrent, completed infarction with complete and Another mechanism is the steal syndrome with reroute
incomplete recovery or infarction in progression. Cur of the blood flow to other vascular basin. The reduction
rently atherosclerotic, cardioembolic, lacunar and cryp or interruption of the brain blood supply leads to devel
togenic infarctions are distinguished. Depending on the opment of cerebral infarction. In the neighboring of the
infarction area (ischemic penumbra) the lesions in the
pathomorphological picture IS is divided into white, red
110 / Clinical Neurology
first hours are still reversible. with an increase in blood pressure. In embolic stroke the
Pathomorphology. The ischemic necrosis is pre patients are pale, complaining of headaches on the side
sented macroscopic as white infarction. The hemorrhag of the stroke. Sometimes they manifest with focal epi
ic infarction is red; it is specific of cerebral embolism leptic seizures. In embolism of large vessel disturbanc
and affects mainly the gray matter. In a massive stroke es of consciousness occur. In the vasodystonic strokes
with cerebral edema there is a risk of cerebral hernia (high blood pressure) the patients are with flushed face
tion - most often below the tentorium with compression and with a severe headache and vomiting. The throm
of the midbrain and less often of the cerebellar tonsils. botic and hemodynamic strokes occur during sleep or
In the recovery period gradually a glial scar is formed, rest and in low blood pressure. They develop gradually
while in large infarction - encephalomalatic cyst. On (infarction in development) and later in a large infarc
the sites of the small lacunar infarcts (below 12 mm2) tion disturbances of consciousness occur. In IS are ob
pseudocysts (lacunas) are formed. They are a common served 1) focal neurological symptoms, which depends
finding in recurrent TIA, long lasting hypertension and on the vascular basin and 2) general cerebral symptoms,
vascular dementia. which are determined by the massiveness of infarction
Clinical picture. The embolic and vasodystonic and cerebral edema.
(from vasospasm) IS have an acute onset. Usually they Syndrome o f a. carotis interna (fig. 14.3, fig. 14.4).
develop in the daytime in physical or mental tension Most often, the obturation is in the area of the bifurca
tion and if proximally by the divi
sion of the ophthalmic artery may
remain asymptomatic or manifests
by recurrent TIA. In involvement
of a. ophtalmica occur ipsilateral
decrease of vision (amaurosis fu-
gax) and contralateral hemiparesis
and hemihypoesthesia (optical-py
ramidal syndrome) and Horner’s
syndrome by affecting the sympa
thetic plexus surrounding the ar
tery. Depending on the collateral
circulation infarction in the basin
of its largest branch (middle cere
bral artery) develops or in the ba
sins of the middle and anterior cer
ebral artery.
Syndrome o f a. cerebri media.
The obstruction of the main stem
Fig. 14.3. Arterial supply of the primary motor and sensory cortex - coronal, view
. /(Aminoff)
a cc\ leads to a massive infarction: con-
Motor cortex
Sensory cortex
Frontal eye
Broca area
Wernicke area
Superior division o f
middle cerebral artery
radiatio optica
division o f middle
cerebral artery
a.cerebri media
the first day, as well as the diagnosis of IS in the brain sion vessel wall (“hematoma type”) and b) diapedesis of
stem. It reveals hyperintense area in T2 and hypointense blood through the walls of small blood vessels (“hemor
in T1 sequences - fig. 14.5. With Doppler sonography rhagic impregnation type”). The hypertonic PBH most
(extra- and intracranial) stenoses are diagnosed, ob often develops in the subcortical nuclei, brainstem and
structions and collateral circulation is estimated. MRI cerebellum, and those with non-hypertonic etiology - in
angiography demonstrates most accurately the vascular the cerebral lobes. The subcortical PBH are divided into
changes. capsule-lateral (50%) and the capsule-medial (10%). The
Differential diagnosis is necessary between embol internal capsule with tightly arranged myelinated fibers
ic and vasodystonic ischemic strokes, and with brain is a powerful barrier against the spread of hematoma.
hemorrhage, which is very important for the treatment. The hemorrhages in the brainstem are primary and sec
In infarctions with subacute clinical course, the clini ondary. The primary is localized mainly in pons (10%).
cal picture resembles subdural hematoma that may lack The cerebellar hemorrhages develop most often in nu
a history of trauma. In IS with chronic, pseudotumor cleus dentatus (10%). The lobar PBH are about 10% and
course (for example, carotid artery thrombosis) should affect the individual lobes - frontal, parietal, temporal
be excluded brain tumor. Some tumors such as glio and occipital. The predilection development of PBH in
blastoma can manifest themselves with acute pseudo the subcortical nuclei can be explained by the emerging
stroke onset. In large strokes differential diagnosis with at right angles of the lenticulostrial and thalamoperfo-
meningoencephalitis, herpes encephalitis, and coma rating arteries from the circle of Willis, with the thinner
with another etiology is necessary. walls and the wider lumen of these vessels, and the lack
The therapy aims to maintain the vital functions, to of anastomoses with penetrating deep cortical arteries.
control blood pressure and intracranial pressure and to This creates conditions for a relatively high blood pres
restore the regional blood flow in the ischemic penum sure in the arteries of the subcortical ganglia. A similar
bra by thrombolysis in the first 3,5 hours (therapeutic explanation exists for the paramedian arteries coming
window) with recombinant tissue plasminogen activator out of the basilar artery and supplying pons.
(rt-PA). Also antiplatelet agents are prescribed (ASA, Pathomorphology. Depending on the size haemato-
Dipyridamol, Clopidogrel), rheological active medica mas is divided into small (1-2 cm in diameter, less than
tions (Pentoxifyllin), neuroprotective (Nimodipine, Cer- 20 ml of blood), medium (20-40 ml) and large (more
ebrolysine, Piracetam), and after the acute stage - vas than 60 ml of blood, several cm). The brain hemat
oactive agents with complex action (Pentoxifyllin, Nic- oma presses and destroys the brain tissue and causes
ergoline, Vinpocetine, Piracetam, Flunarizine, Ginko development of perifocal edema. The erythrocytes are
biloba, Citicoline). An anticoagulant therapy with hepa phagocytosed by macrophages. At 5-6 day from hemo
rin or sintrom is performed in thromboembolic stroke of globin are formed hemosiderin and hematoidin and the
cardiac origin. The low molecular weight heparins have clot turns brown. The hemorrhage is gradually resorbed
a protective effect against deep venous thrombosis and and depending on the size form glial scar or post hemat
pulmonary embolism. Brain edema is treated with 10% oma cyst.
Mannitol and Furosemide intravenously. The clinical picture consists of focal symptoms,
The primary prevention aims to influence the risk which depend on the location of the hematoma and
factors. The secondary prophylaxis aims to prevent re general cerebral symptoms, which are a consequence
currences (antiplatelet agents, anticoagulants, carotid of the intracranial hypertension, cerebral edema and
endarterectomy, placing a stent). subsequent dislocations with compression of the brain
stem. The general cerebral syndrome includes acute de
velopment of headache and vomiting and rapid onset of
14.4. PARENCHYMAL BRAIN H EM quantitative disturbances of consciousness to coma with
ORRHAGE (PBH) damage of the vital functions.
In a massive breakthrough o f blood to the ventri
The brain hemorrhages represent approximately 10- cles or subarachnoid space the general cerebral symp
20% of all strokes, but are of great importance because toms deteriorate - the blood obstructs the CSF system
of the high mortality rate, severe disability in survivors and leads to the development of acute hydrocephalus,
and affection of the younger age group. The morbidity meningoradicular irritation and diffuse spasm of the
rate is 10-15 / 100 000. superficial arteries. The penetration of blood in the ven
The etiology of non-traumatic PBH includes: hy tricles causes tonic extensional convulsions and acute
pertension, brain aneurysms, brain angiomas, arterio deteriorating of the vital functions.
venous malformations, primary and metastatic tumors, The focal symptoms in capsule-lateral hematomas in
bleeding diathesis, vasculitis, brain infarction, brain striatum grow acutely with headache and vomiting and
phlebothrombosis, amyloid angiopathy, anticoagulant rapid development of contralateral hemiparesis / hemi
therapy. plegia with conjugated deviation of the head and eyes to
The pathogenetic mechanisms are basically of two the side of the lesion. Sensory, aphasic and agnostic dis
types: a) aneurysm rupture or rupture of blood vessel orders and rarely epileptic seizures are observed. The
with pathologically changed from the arterial hyperten condition acutely deteriorates if the bleeding involves
14. Vascular diseases of the nervous system / 113
the lateral ventricle or subarachnoid space. In midbrain
compression develops quadriplegia and decerebration.
In some cases, muscle tone remains diffusely decreased
(atonic coma), which has a poor prognosis.
The capsule-medial hemorrhages affect thalamus
and parts of the midbrain and drill in the lateral or in
the third ventricle. Acutely develop severe general cer
ebral and focal symptoms - flaccid hemiplegia, hemian
esthesia, divergent strabismus, anisocoria, vertical gaze
paresis, diverse deviation of the eyes, etc. Sensory tha
lamic aphasia can be observed, and because of hypotha
lamic affection - hyperpyrexia, labile blood pressure,
tachycardia, etc.
The lobar hemorrhages also start acutely with head
ache, which is not diffuse, but more localized. The oc
cipital hematoma start with a headache and contralater
al hemianopsia; the temporal - with temporal headache
and sensory aphasia, the frontal hematoma - with fore
head pain, ataxia and weakness in the upper limb. Some Fig. 14.6. CT scan in hypertensive intracerebral hemorrhage.
times epileptic seizures and general cerebral symptoms B lood is seen as h igh -d en sity signal in the thalam us and ex
tends into ventricles
are observed in massive hematomas with dislocation.
The brainstem hemorrhages are common in pons
and usually complicated with breakthrough to the IV 1) antihypertensive agents; 2) anti-edema agents (Mannitol,
ventricle and internal hydrocephalus. They are clini Furozemide); 3) medications that reduce vascular perme
cally characterized by acute onset, quadriplegia, head ability (Vitamin C, Rutascorbin); 4) coagulating agents in
ache with persistent vomiting and rapid development of impaired coagulation (Vitamin K, plasma); 5) low molecu
coma, tonic seizures, spasticity and decerebration and lar weight heparins for the prevention of pulmonary embo
severe disturbances of vital functions. The examination lism and phlebothrombosis; 6) antiepileptic drugs; 7) neu-
observes narrow pupils, quadriplegia, gaze paresis with roprotective treatment (Piracetam, Citicoline) in the later
deviation of the eyes to the paralyzed limbs, and alter stage. In cerebellar haematomas and lobar hematoma with
nating syndromes. mass effect surgical evacuation is recomended.
In cerebellar hemorrhage also develops severe head The prognosis depends on the amount of bleeding,
ache, vomiting, ataxia, and dysarthria. The condition presence of a breakthrough to the ventricles and the degree
can quickly deteriorate due to the brainstem compres of consciousness impairment. In result below 8 points on
sion and the intracranial hypertension with disturbance the Glasgow scale outcome is lethal in 90% of cases.
of the vital functions. Of diagnostic value is the dissoci
ation between severe condition and the preserved limb
movements, and the presence of muscle hypotonia. 14.5. SUBARACHNOID HEMORRHAGE
The diagnosis is made with CT, in which the hemat
oma is visualized as hyperintense area immediately af The subarachnoid hemorrhage (SAH) is ranked fourth
ter its occurrence. The complications are also observed in frequency after the atherothrombotic, embolic and hem
- breakthrough to the ventricles, brain swelling, disloca orrhagic stroke. Its frequency among all strokes is 10-12%.
tions, and the development of hydrocephalus (fig. 14.6). The annual incidence of SAH is 10/100 000. It affects the
In the first days after the hemorrhage MRI is not able age group of 40-60 years.
to visualize hematoma, and in the later stages the PBH Etiology. In most cases (over 60%) SAH is due to rup
is seen “dark” on T2 and “light” on T1 sequences. The tured saccular aneurysm (aneurysmal SAH). In other cas
lumbar puncture detects bloody or xantochrome CSF, es (non-aneurysmal SAH) is due to trauma, arteriovenous
but risk of cerebral herniation exists. malformation, intracranial arterial dissection, cerebral
The differential diagnosis is difficult in patients in vasculitis, coagulopathy, brain tumors, usage of drugs
coma and impossibility of CT/MRT performance - is with sympathomimetic effect (cocaine, amphetamine), etc.
chemic stroke, subarachnoid hemorrhage, brain tumor, The autopsy and angiography studies demonstrate
traumatic hematoma, brain abscess, encephalitis, status that 2% of adults have brain aneurysms. The saccular
epilepticus, hypo- or hyperglycemic coma, etc. For the aneurysms occur in the area of the bifurcation of the
PBH is typical the sudden development of focal symp large vessels at the base of the brain. They are formed
at the place where the elastic lamina and tunic media
toms and coma.
The treatment is performed in an intensive care unit are defective and aneurysms gradually increase with
where the vital functions are monitored and controlled. If age. A typical aneurysm is composed of intima and
necessary, nasotracheal or orotracheal intubation w ith con adventitia. Some aneurysms are filled with thrombotic
trolled hyperventilation is performed. The therapy includes: masses which become a source of emboli. Over 90%
114 / Clinical Neurology
of aneurysms are situated in the anterior circulation - hours and in the first two weeks. In the next 6 months,
at the point of separation of a.communicans posterior recurrences receive 40-60% of patients, and then the
from the internal carotid artery (40%), of a.communu- recurrence rate reduces to 3% per year. Between the
cans anterior (30%), of the bifurcation of the middle 4th and 14th day on the place of the effused blood de
cerebral artery (20%). Only 10% of aneurysms are in velops vasospasm under the influence of vasoactive
the posterior circulation - at the apex of the basilar substances released by the blood cells and the vessel
artery at the site of divison of the a. cerebelli infe wall (spasmogens). Later ischemic brain dysfunction
rior posterior - Fig. 14.7. Approximately 20% of pa develops, as this contributes to the reduced perfusion
tients have more than two aneurysms. Over 90% of pressure.
aneurysms are small in size below 10 mm and usually Pathomorphology. The blood effused into suba
remain asymptomatic lifelong and the risk of SAH is rachnoid space mix with the CSF and distributs diffuse
0.7% per year. Most often bleed aneurysms with a size ly, as on the site of the aneurysm accumulates a larger
greater than 10 mm. The annual risk of bleeding for amount of blood - for example in the Silvian fold in
aneurysms larger than 25 mm is 8%. The risk of SAH aneurysm of the middle cerebral artery, in the anterior
increases with age, uncontrolled hypertension in the interhemispheric fissure in aneurysms of the anterior
presence of family history for SAH, polycystic kidney communicating artery, in the anterior mesencephalic
disease, coarctation of the aorta, smoking, alcoholism. fissure in aneurysm of the posterior communicating
In atherosclerosis can develop fusiform aneurysms, artery or basilar artery. In parenchymal-subarachnoid
which often contain atherothrombotic masses. hemorrhage blood can effuse into the ventricles.
Pathogenesis. In physical or mental tension and Clinic. The initiation of SAH is usually associated
in acute rise of the blood pressure occurs aneurysm with physical exertion, sexual and other activities, lead
rupture and effusion of blood in the subarachnoid ing to an acute rise in blood pressure. Suddenly, without
space, and sometimes in adjacent brain parenchy prodromes occurs extremely severe headache (such as
ma. The amount of the effused blood depends on the “hammer blow to the head”), followed by nausea and
intracranial counter pressure and the thrombus for vomiting. Approximately 10-25% of the patients can
mation. The effusion of blood in the basal cisterns experience epileptic seizures. In a massive effusion
with disturbance of the CSF flow and blocking of the of blood the patient is unconscious, and in decerebra
arachnoid villi and CSF resorption causes acute hy tion state. If less amount of blood is effused develops
drocephalus. As a result, the intracranial pressure in somnolent state, confusion and amnesia for the acute
creases, the cerebral perfusion pressure and cerebral period. The examination reveals meningoradicular syn
microcirculation decreases, with the development of drome (neck stiffness, positive symptoms of Kernig and
general cerebral ischemia. The most serious compli Brudzinski) and lack of focal symptoms. In cases with
cation is re-bleeding, the risk is highest in the first 24 highly elevated intracranial pressure occurs compres
sion of the VI cranial nerve. Sometimes minimal focal
symptoms are observed, referring to the location of the
aneurysm.
In involvement of the III cranial nerve is suggest
ed aneurysm of the posterior communicating artery; in
impaired movements in the legs and apathetic-abulic
syndrome - aneurysm of the anterior communicating
artery, in oculomotor disorders - siphon aneurysms of
the internal carotid artery. The severity of the SAH is
estimated on the scale of Hunt and Hess.
fu n icu lu s lateralis
I tractus
arm I c o rticosp in alis
a.sp in alis ant. \ hand
paraparesis, hypoesthesia for pain and temperature complication of anticoagulation therapy. They begin
with a sensory level, loss of sphincter control and re with acute manifestations of compressive myelopathy -
flex ileus with bloating. The spinal infarctions can lower flaccid paraplegia or quadriplegia, sensory level
be preceded by transient ischemic attacks. In athero and loss of sphincter control. In lumbar puncture is de
sclerosis of the aorta and segmental arteries develops tected blood and xantochromia in the CSF.
spinal claudication - walking evokes weakness in the The vascular malformations are of two types - dural
legs, which disappears at rest. The infarctions in the fistulas and arteriovenous malformations (AVM). In du
basin of a. spinalis posterior are extremely rare. Also ral fistulas abnormal communications between arteries
rare is spinal phlebothrombosis. and veins lie in the dura near the root split. The vaso
A common disease is the so-called chronic cervical dilation induces compressive myelopathy with exacer
myelopathy, which is due to chronic ischemia, and spi bations (sometimes after physical exertion), which re
nal cord compression. It occurs with lower progressive semble relapses of multiple sclerosis. MRI can visualize
paraparesis - similarly to spinal cord comression by tu the dilated vessels and the selective spinal angiography
mor or spinal form of Multiple sclerosis. - feeding arteries and dilated draining veins.
The diagnosis is performed by MRI, which visu The arteriovenous malformations are localized in
alizes the spinal infarction. The spinal angiography is the posterior surface of the spinal cord, and some of
technically difficult to achieve. Differential diagnosis is them penetrating into it. The dilated vessels press the
made with other vascular (hematomyelia, vascular mal spinal cord and cause chronic myelopathy or occur with
formations) and non-vascular diseases of thespinal cord recurrent subarachnoid hemorrhage and acute trans
- tumors, myelitis, demyelinating plaques. verse myelopathy. For the establishment of the diagno
The hemorrhages o f the spinal cord (hematomyelia), sis is necessary MRI and selective spinal angiography.
the subdural and epidural hemorrhage are due to trau Sometimes the auscultation on the spine detects noise.
ma, vascular malformations, hemorrhagic diathesis or The treatment of the vascular malformations is surgical.
15. Tumors of the nervous system 119
15.1. BRAIN TUMORS ties - astrocytoma (chromosome lOp 17p, 13q, 9), oligo
dendroglioma (lp, 19q), meningioma (22q).
The tumors of the CNS are relatively frequent. They The brain tumors can have expansive growth, lead
account approximately 10% of all oncological diseases. ing to compression, dislocation and deformation of the
The incidence in adults is 15-18 / 100 000 per year, and surrounding cerebral tissues, or infiltrative (invasive)
nearly the half are metastatic. In children, the incidence growth. The newly formed blood vessels have imper
is 3-4 / 100 000. The intracranial tumors represent fect walls with increased permeability, causing the de
6-10% of all tumors in adults and in children - 50% of velopment of peritumoral vasogenic edema. The tumors
neoplasms. Some tumors have sexual predilection. The growing in proximity of the III and IV ventricle ham
meningiomas, neurinomas and pituitary adenomas are per the CSF flow and cause hydrocephalus earlier. The
more common in women, the gliomas - in men. In adults growing tumor mass, brain edema and hydrocephalus
2/3 of the tumors were supratentorial, while in the child lead to increased intracranial pressure in the closed cra
hood predominate the subtentorial tumors. Over 50% of nial cavity. This contributes to the compression of the
the brain tumors are found in the age group 40 to 60 veins and impaired CSF absorption. The rate of growth
years with a peak 55 years. The primary tumors orig of the tumor mass determines the possibilities for com
inate from the intracranial tissues. The secondary are pensation. In slow growth the clinical symptoms appear
metastatic or are tumors that originate from the adjacent later, unlike in rapidly growing tumors.
tissues and invade the CNS. Most frequent from the pri The clinical picture consists of two syndromes: gen
mary tumors are gliomas, meningiomas, pituitary ade eral cerebral syndrome, due to the intracranial hyper
nomas and neurinoma of the auditory nerve. tension and focal neurological syndrome which depends
The classification of brain tumors is based on their on the localization of the tumor. Some symptoms such
cellular origin, histology and biological behavior. In a as headache, vomiting and epileptic seizures could be
shortened version this classification is as follows: due to both the intracranial hypertension, and the tu
I. Neuroepithelial tumors: astrocytoma, oligoden mor itself. The slowly growing tumors, especially those
droglioma, glioblastoma, ependymoma, papilloma, pi- in the so-called “quiet” areas (for example, the frontal
neocytoma, gangliocytoma, ganglioglioma, embryonic lobe) show treacherous course and are detected when
tumors (medulloblastoma, primitive neuroectodermal they reach a large sizes. The small tumors with a stra
tumor), etc. tegic location affecting the vital centers and pathways
II. Tumors of the cranial and spinal nerves: schwan occur much earlier.
noma (neurinoma), neurofibroma, malignant schwanno The main manifestations of the syndrome of intracra
ma, etc. nial hypertension (ICH) are headache, vomiting and con
III. Tumors of the meninges: meningiomas, malig gestive papilla. The headache covers the entire head, it has
nant meningiomas, etc. stretching character, and increases on exertion, coughing,
IV. Lymphoma, plasmocytoma, etc. and bending forward. It is stronger in the morning after
V. Germ cell tumors: germinoma, embryonal carci getting up. In the beginning it can be local. In the supraten
torial tumors the headache is stronger in the frontal area,
noma, teratoma, etc.
VI. Cysts, epidermoid, dermoids, colloid cyst of the while in the subtentorial - in the occipital region. The vom
iting occurs suddenly without nausea, often accompanies
III ventricle, etc.
the headache that temporarily decreases after vomiting.
VII. Tumors of the sellar area: pituitary adenoma,
The congestive papilla is with relatively late onset - the pa
pituitary carcinoma, craniopharyngeoma.
pilla is enlarged, with unclear boundaries and prominence,
VIII. Local extension of regional tumors: chordoma,
which is measured in diopters. The tumors in the posteri
chondroma, chondrosarcoma, osteosarcoma, carcino
or cranial fossa lead early to congestive papilla due to the
ma, etc.
rapid development of obstructive hydrocephalus. The com
IX. Metastatic tumors
pression o f n. abducens is also a manifestation ot the ICH,
X. Unclassified tumors
since this nerve has a long way in the skull base. In ICH
The etiology of the brain tumors is still unclear. In
can be observed epileptic seizures, mental, memory and
the process of oncogenesis participate ionizing radiation
consciousness disturbances. The syndrome of ICH usually
(in meningiomas, gliomas), oncogenic viruses, chemi
develops slowly and gradually. Sudden and acute increase
cal carcinogens, genetic factors, etc. An increased risk
of the intracranial pressure is also possible in haemorrhage
of brain tumors is associated with a number ol genetic
in the tumor mass, and infarction of the adjacent or distant
syndromes - neurofibromatosis types I and II, tuberous
areas due to poor circulation or drainage, and acute occlu
sclerosis, a disease of von Hippel-Lindau, retinoblasto
sion of the ventricles.
ma, etc. In many tumors are proven genetic abnormali
120 / Clinical Neurology
The local increase in the intracranial pressure leads transcortical motor aphasia, melokinetic apraxia and
to dislocation and herniation (wedging) of the brain mental disorders - apathetic-abulic (emotional indif
parenchyma under the falx cerebri, through incisu- ference and lack of initiative) and Moria syndrome (sil
ra tentorii or through foramen magnum - Fig. 15.1. ly humor). Frontal ataxia is also observed.
The following types of herniation are subdivided: 1) Parietal lobe: contralateral sensory Jacksonian
Subfalx herniation of gyrus cinguli with subsequent seizures, monohypoesthesia, hemihypoesthesia, ste
compression of a. cerebri anterior; 2) Transtentorial reoagnosia, syndrome of Gerstmann in left-sided lo
descending herniation of uncus gyri hyppocampi with calization (finger agnosia, impaired orientation for
compression of the III cranial nerve (ipsilateral mydri left-right, acalculia and agraphiya), ignoring the sen
asis), midbrain (contralateral hemiparesis, impaired sory stimuli in the left half of the body in right-sided
consciousness), and compression of a. cerebri posterior tumors, ideomotor apraxia.
with occipital infarction; 3) Transtentorial ascending Occipital lobe: simple or more complex visual hal
herniation - in tumors growing in the posterior cranial lucinations, contralateral quadrantanopsia, hemian
fossa the upper parts of vermis are herniated in the opsia, cortical blindness, visual agnosia (for objects,
tentorial foramen with compression of the midbrain colors, faces), alexia without agraphia in involvement
and development of vertical gaze paresis, occlusive of the left area 17 and the posterior part of corpus cal
hydrocephalus and disturbance of consciousness; 4) losum.
cerebellar tonsillar herniation into foramen magnum Temporal lobe: simple partial seizures with pre
with appearance of neck rigidity, involuntary posture served consciousness (auditory, vestibular, olfactory
of the head, and compression of medulla oblongata and gustatory hallucinations, visceral seizures, psycho-
with quadriplegia and disturbed vital functions. sensory derealization and depersonalization seizures),
The focal neurological manifestations can be ei complex partial seizures with impaired consciousness
ther excitatory of irritation of the neurons (for exam (psychomotor, delirium epileptic equivalents), audito
ple, epileptic seizures) or negative from damage of the ry agnosia (subject, speech), sensory aphasia (cortical,
functions. The neurological disorders are determined transcortical, amnesic), contralateral quadrantanopsia.
by the location of the tumor - motor and sensory Jack Pituitary tumors: hormonal hyper- or hyposecre-
sonian seizures, hemiparesis, hemihypoesthesia, apha tion, bitemporal hemianopsia, headache.
sia, ataxia, lesions of cranial nerves, etc. Tumors o f the brainstem: alternating syndromes
Frontal lobe. In tumors affecting the motor cortex (ipsilateral lesion of cranial nerve with contralateral
(area 4) are observed partial motor Jacksonian sei hemiparesis and/or hemihyposthesia).
zures, more frequently involving the upper limb and Craniospinal tumors: lesion of the bulbar group
the corresponding facial half, with a subsequent de cranial nerves, quadriparesis to quadriplegia, sensory
velopment of central paresis of face and arm and in disturbances in the 4 limbs.
parasagittal tumors - central lower paraparesis. For tu More common are the following intracranial tum
mors involving the premotor cortex (areas 6 and 8) are ors:
typical adversive epileptic seizures, in which the head, Gliomas have infiltrative-invasive growth; grow in
eyes, and sometimes the whole body deviate in the con depth and through corpus callosum. The most common
tralateral direction. Also typical are the motor aphasia, are astrocytomas which, depending on the degree of
malignancy are divided into: I grade - the most benign,
II grade - moderately differentiated, III grade - low
differentiated, IV grade - glioblastoma multiforme.
Glioblastoma multiforme is the most common malig
nant tumor in adults, and in children - medulloblas
toma. Oligodendroglioma and ependymoma are rare,
have slow growth, and sometimes are anaplastic.
Meningeomas have extracerebral slow growth, of
ten contain calcifications, and are localized mainly
on the base (orfactorius meningeoma, sphenoidal, su
prasellar, pontocerebellar), on convexity and parasag
ittal (falx-meningeomas). The meningeomas are mul
tiple sometimes. The angiomatous malignant variants
with invasive growth are very rare.
The pituitary tumors originate mainly from the ad
enohypophysis. They have intra-, para- or suprasellar
growth, in 15% invade dura and grow in the sphenoidal
sinus. They cause hyper- or hypofunction of the gland
and can compress the optic chiasm, oculomotor nerves,
F ig .15.1. Brain herniation (Fitzgerald) 1. Subfalx herniation, hypothalamus, etcs. Microadenomas have sizes up to
2.1 ranstentorial herniation, 3. Cerebellar tonsillar herniation 10 mm, mesoadenomas - 10-25 mm and macroadeno
15. Tumors of the nervous system 121
mas - over 25 mm. Most frequent are the prolactinomas
15.2. TUMORS OF THF. SPINAL CORI)
- secrete prolactin and cause amenorrhea and galactor A M ) SPINAL COLUMN
rhea, impotence and infertility. Corticotropinomas se
crete ACTH with development of subsequent Cushing
The primary tumors of the spinal cord are rare - 15%
syndrome (arterial hypertension, hyperglycemia, obe
of tumors of the nervous system. In contrast extradur
sity, etc.) or syndrome of Nelson (adrenalectomy on the
al metastatic tumors are relatively common - 5-35% of
occasion of Cushing’s syndrome is followed by hyper
patients with neoplasms have spinal metastascs. The
secretion of ACTH and melanin stimulating hormone). spinal tumors are divided into extradural and intradur
Somatotropinoma (secrete growth hormone) induces al. The extradural tumors are most often metastases in
gigantism in children and acromegaly in adults. Crani- vertebrae with invasion of the epidural space. The in
opharyngeomas are parasellar tumors in childhood, tradural tumor according their localization and origin
contain calcifications and have slow growth. are subdivided into extramedullary and intramedullary.
The vestibular schwannoma (acusticus-neurino-
ma). The tumor derives from Schwann cells of the ves I. I he extramedullary tumors derive from the
tibular-auditory nerve. It grows in the ponto-cerebellar shelves of the roots (schwannoma and neurofibroma), of
angle and presses the V, VII, VIII, IX and X cranial leptomeninges (meningiomas), from ependiyma of filum
nerves, brainstem and cerebellum. The acusticus-neu- terminale (ependymoma). More rare extramedullary tu
rinoma cause tinnitus and hearing loss, dizziness, mors are dermoids, epidermoid, teratomas, lipomas, etc.
staggering gait, compression of cranial nerves. It has Neurinomas affect equally both sexes in the age 40-
slow growth and can reach large sizes. 60 years. They originate from Schwann cells of the roots
Metastatic tumors. They originate from a carcino (also called schwannomas). Malignant neurofibroma
ma of the lung, breast, kidney, gastrointestinal tract, variants are observed in Recklinhausen’s disease. The
melanoma and others. The metastasis are rarely single tumors are located in the posterior-lateral or anterolat
(solitary) and often are multiple. Around them devel eral sections of the spinal canal. In 10% they invade the
ops large edema. They cause epileptic seizures, motor paraspinal tissues, growing through foramen intcrver-
deficits and rapid development of intracranial hyper tebrale and expanding it (neurinoma type “hour-glass”).
tension. Meningiomas are observed more often in women.
The diagnosis of brain tumors is established with They are localized mainly in the thoracic area, followed
neuroimaging methods including CT and MRI that re- by cervical and craniospinal area and predominantly in
yeal the location, size, and density of the tumor, the lateral part of the spinal canal. The tumors rarely have
surrounding edema and dislocation (mass effect). In extradural grow and infrequently invade the spinal cord.
vascular tumors is recommended cerebral angiogra They do not cause changes in bone structures.
phy. In suspicion of tumor (headache combined with The clinical symptoms develop slowly and grad
focal syndrome) the examination of ocular fundus for ually, without the presence of remissions. The earliest
congestive papillae is obligatory. EEG detects focal symptoms are radicular pain and paresthesia, which
slow wave activity. Lumbar puncture is contraindicat have encircling character or irradiate towards the limbs
ed because of the risk of herniation. The examination depending on the location of the tumor. The pain in
is performed only for search of tumor cells in suspi tensifies in coughing, sneezing and pressure in the
cion of carcinomatosis of the meninges. The modern paravertebral area. Then, the symptoms of spinal cord
diagnostics allow detection of the tumor at an early compression occur. In anterolateral location due to the
stage when surgical capabilities are larger. With CT compression of the spinothalamic pathway occur par
and MRI-based stereotaxic biopsy is clarified the his esthesia and disturbed senses of pain and temperature
tology of the tumor. starting in the distal leg and ascending upward. In more
The differential diagnosis includes other space severe compression w ith hemisection of the spinal cord
occupying brain diseases (abscess, focal encephalitis, develops progressive ipsilateral central mono- or hemi-
parasitic cysts, and large demyelinating plaque), vas paresis and disturbed joint-position sense with con
cular malformation, ischemic and hemorrhagic stroke, tralateral hypoesthesia for pain and temperature (syn
epilepsy, headache, hydrocephalus, etc. drome Braun-Sequard). The delayed diagnosis leads to
The treatment is surgical and objectives total or a complete transverse lesion of the spinal cord - lower
subtotal removal of the tumor, decompression, stereo spastic paraplegia or quadriplegia, conduction anesthe
taxic biopsy, implantation of radioactive isotopes (in sia below the level of the tumor and loss of pelvic res
terstitial brachytherapy), stereotaxic radiosurgery with ervoir control.
a gamma knife. Radiotherapy is used to prevent relapse. The diagnosis is established by MRI - the neurino
Chemotherapy is conducted with nitrosourea - Carmus- mas are isointense or hypointense in T1 and hyperin-
tine (BCNU), Lomustine (CCNU), platinum cytotoxic tense in T2 sequence; meningiomas are hyperintense
drugs - Cisplatin, vinca alkaloids (Vincristine, \ inblas- in Tl. The CT-assisted myelography with water-soluble
tine), Methotrexate, Temozolamide, Fluorouracil, etc. contrast reveals partial or complete defect in filling of
For the treatment of cerebral edema is applied Dexa- the subarachnoid space. By X-ray examination can be
methazone 24-48 mg / 24h, Mannitol 1-2 g / kg / 24h. detected an increase in the distance between the roots
122 / Clinical Neurology
of the vertebral arches (symptom of Elsberg) and en ductive symptoms of the spinal cord occur with devel
largement of the intervertebral foramen in neurinoma. opment of paresis / plegia, hypo- to anesthesia below the
The CSF examination demonstrates a strong increase in level of compression and sphincter disorders. In patho
protein and normal number of cells - protein-cell dis logical fracture with collapse of the vertebral body, the
sociation. compression of the spinal cord develops acutely. Due to
compression of the blood vessels further develops spinal
II. The intramedullary tumors according their his cord infarction. In the area of the affected vertebra is es
tological structure are most often ependymoma, astro tablished pain, paravertebral rigidity, and straightening
cytomas and rarely hemangioblastomas, dermoids, and of the lumbar lordosis.
epidermoids. The astrocytomas are more common in Diagnosis. The radiography establishes osteolytic
younger age and are localized mainly in the cervical and foci in the basal part of the vertebral arcs and verte
cervical-thoracic area. The ependymomas unlike astro bral body, bone destruction, pathological fractures, and
cytomas are well bounded from the spinal parenchyma subluxations. The absence of changes in the interverte
and are easier to extirpation. The onset of the clinical bral discs is typical. The degree of invasion of the bony
symptoms is barely noticeable - dull pain and paresthe structures and compression is specified with CT and
sias that gradually radiate downward of the level of the MRI.
tumor (descending type sensory disturbances unlike the Differential diagnosis. The nonspecific and specif
ascending type in extramedullary tumors). In engage ic spondylitis and spondilodiscitis with or without an
ment of a blood vessel and tumor vascular accident the epidural or paraspinal abscess occur with febrile-intox
symptoms onset can be acute. Gradually, due to com ication syndrome, leukocytosis, increased sedimenta
pression of the pyramidal tract develops lower central tion rate, local and polyradicular pain and symptoms of
monoparesis or paraparesis, and in tumors in the level spinal compression. Specific findings are the narrowing
of the cervical intumescense develops flaccid paralysis disc and the reactive inflammatory changes in the ver
in the arms and spastic paralysis in the legs. In parallel, tebrae, which were established on radiographs. With CT
sphincter disorders occur. and MRI the changes in the vertebrae and surrounding
The diagnosis is established by MRI - a fusiform tissues are visualized better. The degenerative diseases
enlargement of the spinal cord with isointense or het- of the spinal column (spondyloarthrosis, spondylosis,
erointense character. The CT-assisted myelography disc herniation) lead to radiculopathy and myelopathies.
demonstrates partial or complete subarachnoid block. The discogenic radiculitis have acute onset, the pain in
The radiography can establish the enlargement of the tensifies in movement and disappears at rest. The spinal
spinal canal and scoliotic deformation in slowly grow arteriovenous malformations and spinal multiple sclero
ing tumors in children. sis can have pseudotumor course.
The treatment of the tumors of the spinal cord and
III. Spinal column tumors the spinal column is surgically. It aims to remove the
The primary tumors of the spinal columa are very tumor, decompression of the spinal cord and roots and
rare - osteoma, osteoblastoma, osteogenic sarcoma, histological verification of the tumor. In malignant tu
chondroma, chondrosarcoma, fibroma, fibrosarcoma. mors is applied radiotherapy. Dexamethazone is applied
The metastatic carcinoma is the most common neo for reduction of the edema and spinal compression.
plasm of the spinal column - 5-30% of the patients with
carcinoma develop spinal metastases. The neoplasms of
the lung, breast, kidney, prostate, and thyroid most of 15.3. IDIOPATHIC INTRACRANIA L
ten give metastases. In 10% of patients the origin of the HYPERTENSION (IIH)
primary tumor remains unclear. In 30% of the cases is
affected more than one vertebra. The metastases affect The disease is characterized by symptoms of
vertebrae in heamatogenous way or in proximity. Most space-occupying process (headache, vomiting, papil-
often they are localized in the lumbar and thoracic ver loedema) in the absence of cerebral space-occupying
tebrae. The metastases develop in the body and arches process. In the past this syndrome was termed “Pseu
of the vertebrae and can invade the epidural space and dotumor cerebri”. Currently, this term also includes
paraspinal tissues. Sometimes they lead to pathological cases of clear etiology (secondary intracranial hyper
fracture with or without dislocation of the vertebrae and tension). The incidence of IIH is approximately 1-2 /
cause acute spinal compression. In adults, the vertebrae 100 000 and is higher in young women - 12/100 000.
are often affected by multiple myeloma. In obese women the frequency reaches 21/100 000. The
Clinical picture. The first symptom is pain with lo disease is observed in the age range 11-58 years.
cal or radicular character. It results of neoplastic infil The etiology is unclear. It is assumed that the dis
tration of the bone substance and the posterior roots. ease is due to hormonal dysfunction, which is support
The pains with bone character appear even at rest and ed by the more frequent involvement of young obese
often are stronger at night unlike the discogenic pain women in period of menarche and menopause. Several
that increase in movement of the spine and weaken at pathogenetic mechanisms are discussed: 1) impaired
rest. Thereafter compression of the dural sac and con CSF absorption at the level of arachnoid granulation;
15. Tumors of the nerv ous system 123
2) increased venous pressure in the dural sinuses with The diagnosis is established by exclusion of diseases
secondary intracranial hypertension; 3) development of which induce intracranial hypertension such as brain tu
cytotoxic brain edema, etc. Secondary intracranial hy mors, abscesses, venous and dural thrombosis. The exam
pertension can occur when taking certain medications ination does not detected focal neurological symptoms; the
(penicillin, tetracycline, vitamin A, oral contraceptives, neuroimaging studies, including MRI with angiography and
indomethacin, ketoprofen, amiodarone, corticoster venography, and CSF examination are normal. The fundus-
oids), in thrombosis of cerebral sinuses, and endocrine copy reveals papilloedema but the visual acuity is normal.
diseases. ' The treatment is performes w ith carbonic anhydrase
Clinical picture. The most common symptom is inhibitor Acetazolamide (Diamox), which suppresses
headache which is diffuse, sometimes stronger in the the secretion CSF, with Furosemide, and Mannitol. The
frontal and occipital region or in the retro-orbital area. therapy with Mcthylprednisolon reduces congestive pa
It increases in coughing and is stronger on awakening. pilla, but with the discontinuation of the steroid ther
Often is accompanied by nausea and vomiting. In 2/3 apy the intracranial pressure can increase again. The
of patients are observed transient visual disturbances - lumbar punctures reduce the intracranial pressure and
blurred vision or blackout for less than 30 s. In some of headaches. In rapid progression is resorting to decom
the patients occur lesions of the VI cranial nerve as a pression by ventricular peritoneal / atrial shunt because
result of the increased intracranial pressure. of the risk of secondary atrophy of the papilla.
124 / Clinical Neurology
They are the most common cause of disability and concussion. The prognosis is less favorable when the
death in young adulthood. In most cases, they are due to loss of consciousness exceeds 6 hours. In more severe
accidents and less on domestic or criminal injuries. In cases the complaints continue few weeks and develop
our country the traumas registered annually are about the clinical picture of the post-concussion syndrome
150/100 000 population. According to their localization (traumatic cerebrasthenia).
are differentiated traumas of the brain, spinal cord and The treatment requires strict bed rest; avoiding of
peripheral nerves. The cerebral traumas are divided to mental and other stress such as reading, working on a
open (in damaged soft tissue of the skull) and closed. computer, alcohol intake. Analgesics, nootropic (Pi
The open cranial-cerebral trauma depending on the racetam, Ginko biloba), anti-vertigo medications and
integrity of dura mater is divided into penetrating and hypnotics are prescribed. In more severe cases anti-ede
non-penetrating. The closed traumas are divided into ma agents (Mannitol, Furosemide) are applied.
two main groups - concussion (commotio cerebri) and
cerebral contusion (contusio cerebri). In the course of
the cranial-cerebral traumas can occur early and late 16.2. C ER EBRA L CONTUSION
complications. The early complications include: trau (CONTUSIO CEREBRI)
matic hemorrhage, carotid-cavernous fistula, inflamma
tory complications (meningitis, abscess), and CSF fistu The cerebral contusion is a severe cranial-cerebral
las. The late complications are of two groups: traumatic trauma, wherein the pathomorphological changes occur
cerebrasthenia (cerebrasthenia traumatica) and traumat in the brain, which give rise to general cerebral and fo
ic encephalopathy (encephalopathia traumatica). cal neurological symptoms. As a result of the direct me
chanical impact (coup) over the head develops traumatic
necrosis of the parenchyma, imbued with blood. Sim
16.1. CER EBRA L CONCUSSION ilar contusion focus develops on the contralateral side
(COMMOTIO CEREBRI) (contra coup) as a result of inertia and brain trauma in
unevenness of the skull and the edges of the dura mater.
The cerebral concussion is the mildest closed cra Thus, in a blow to the occipital region is obtained direct
nial-cerebral trauma that occurs only with brief loss injury to the occipital lobe and indirect contra coup in
of consciousness and without morphological changes jury to the frontal lobe. The contusion foci are single or
in the brain. The pathogenesis is associated with func multiple imbued with blood - from confluent petechi
tional impairment of the brainstem ascending reticular al hemorrhages to formation of hematoma in the brain
activating formation with temporarily inhibiting or dis parenchyma. In the following hours develop cerebral
continuation of its activating effect on the cerebral cor edema - vasogenic due to the damage of the vascular
tex as a result of the vibration in trauma. In mild cases wall and cytotoxic resulting from dysfunction of cellu
no morphological changes are observed. In more severe lar membrane. The intracranial pressure increases, dis
cases transient cerebral edema is detected. location of brain parenchyma with the risk of herniation
Clinical features. As a result of the trauma occurs and compression of the brainstem occurs. The activat
transient loss of consciousness from a few seconds ing function of the reticular formation of the brainstem
up to 20-30 minutes, sometimes more than 1 hour. In is impaired with long-lasting loss of consciousness. In
mild cases there is only obnubilatio with disorienta the recovery stage develops gliosis and encephalomalat-
tion of time and place. After regaining consciousness, ic traumatic cysts.
presence of amnesia is established - memory loss for The clinical picture is dominated by general cer
events around or shortly after the trauma. In the follow ebral and focal neurological symptoms. The general
ing hours and days the patient complains of headache, cerebral symptoms are due to the intracranial hyperten
compounded by mental and physical stress. A variety sion and manifest themselves with quantitative changes
of accompanying symptoms is observed - nausea, vom of consciousness, headache, and vomiting. The period
iting, dizziness, fainting when standing and various of unconsciousness is longer - more than 6 hours and
autonomous disorders - bradycardia or tachycardia, in apallic syndrome persists for days and months. The
postural hypotension, sweating, flushing or pallor. At depth of coma is followed by Glasgow-Liege scale: 13-
neurological examination are not detected focal symp 15 points - mild cranial-cerebral trauma (CCT), 8-12
toms except sometimes anisocoria, nystagmus, anisore- points - moderate CCT, below 8 points - severe CCT.
flexia. The neuroimaging methods - CT and MRI do not Exceptionally, in injury in the so-called “deaf zones” of
reveal pathological changes. The course of the disease the brain the loss of consciousness can be absent. After
is favorable with full recovery within a few days in mild regaining consciousness memory disorders are estab
16. Traumas of the nervous system / 125
lished - traumatic amnesia for events before the injury of CSF trom the ear (otorrhea). Л damage of the cranial
(retrograde amnesia), during the trauma (congrade am nerves in the pontocerebellar angle can be observed -
nesia) and after the trauma (anterograde amnesia). Very facial, auditory and vestibular, less often trigeminal. In
typical is the Korsakov’s syndrome - anterograde amne fracture ot the posterior cranial fossa the occipital bone
sia and filling the gaps in the minds with confabulations is atfected and the fracture can reach foramen occipital
(fiction). The headache, sometimes accompanied with magnum. There is a risk of bulbar nerves affection w ith
vomiting is prolonged, and increases in tension. Other development of bulbar palsy and of brainstem affection
accompanying symptoms are fatigue, somnolence, and with an unfavorable outcome.
disturbances in attention. Tfie focal cerebral symptoms The diagnosis of fractures of the skull is based on
depend on the location of the injury - hemiparesis to the target radiographs. With CT and MRI are visualized
hemiplegia, hemihypoesthesia, hemianopsia, aphasia, contusion foci, cerebral edema and traumatic hemor
damage to the cranial nerves, ataxia, vertigo, seizures, rhages.
severe mental disorders, etc. The prognosis of recovery of linear fractures is good,
Examinations. With X-ray a skull fracture can be and those of the compression fractures can be serious
detected. CT and MRT identify the location and size because of underlying cerebral injury and development
of contusion foci, traumatic hemorrhages, cerebral ede of traumatic hematoma. In CSF fistula exists risk of
ma and dislocations. EEG reveals depression in alpha recurrent meningitis, which requires surgical closure
rhythm and generalized or focal slow wave activity. of the defect. In some of the cases develops traumatic
With EEG the comatose state is monitored and brain arachnoiditis.
death is diagnosed. The funduscopy in intracranial hy
pertension detects papilloedema. The CSF examination
demonstrates erytrocyterachia and xantochromia. 16.4. EARLY TRAUMATIC
The prognosis depends on the size and location of COMPLICATIONS
the injury and the extent of cerebral edema. In severe
cases of traumatic hematoma, with dislocation and com The traumatic hemorrhages in the brain parenchyma
pression of the brainstem the mortality rate is high. The or meninges are more common. They can be combined
cerebral contusion survivors remain with various mani with fractures of the skull, concussion or cerebral con
festations of traumatic encephalopathy. tusion. The other possible non-vascular complications
The treatment is carried out in an intensive care include posttraumatic meningitis and meningoenceph
unit with monitoring of the vital functions, artificial alitis, brain abscesses, epiduritis, subdural or epidural
ventilation with moderate hyperventilation to reduce empyema, CSF fistulas, pneumocele, arachnoid cysts,
the intracranial pressure, anti-edema therapy (Mannitol, and carotid-cavernous fistula.
Furosemide, Dexamethasone), nootropic (Piracetam),
antiepileptic and other symptomatic agents. 16.4.1. Epidural hematoma
The subdural hematoma results of cranial-cerebral The traumatic subarachnoid hemorrhage results of
trauma or develop spontaneously after a minor head in rupture of the blood vessels in the meninges and most
jury in adult alcoholics, cachectic patients, in patients frequently accompanies the cerebral contusion. The
on anticoagulant therapy or with impaired hemostasis. blood is poured into the subarachnoid space, causing
Often patients do not remember the spent trauma. The meningeal irritation with headache, vomiting, neck
hemorrhage is venous and is due to rupture of the pialic stiffness, positive symptoms of Kernig and Brudzinski.
veins before their penetration into the venous sinuses. Diagnosis is based on the CSF examination and CT.
The blood is poured into the subdural space (between
the dura mater and arachnoidea), which is well-defined 16.4.4. Traumatic intracerebral hematomas
and hematoma reaches larger size - to 300 ml. Since
venous pressure is low, the hemorrhage develops more They develop in 5-25% of the cases with severe
slowly and upon reaching a larger volume it presses the cranial-cerebral trauma on the side of the direct in
veins and bleeding discontinues. Thereafter the volume jury (coup) or on the contralateral side following the
of the hematoma increases due to the penetration of CSF mechanism of contra coup due to rupture of an arte
through arachnoidea due to higher osmotic pressure in rial or venous vessel. More often are localized in the
the hematoma. frontal and temporal lobe, and sometimes are multiple.
The clinical picture is similar to those of the epi They are combining with cerebral contusion and brain
dural hematoma, but the dynamics is slower. Accord edema. Besides the early traumatic hemorrhages, late
16. Traumas of the nervous system / 127
post-traumatic hemorrhages in traumatic rupture of the ment of the fixative memory (post-traumatic dementia)
aneurysm are also observed. The clinical picture is very is also possible. Sometimes Korsakov’s syndrome is ob
severe and depends on the size and location of the he served - anterograde amnesia with confabulations.
matoma - a disturbances of consciousness, hemiplegia, 4) Traumatic pachymeningitis and arachnoiditis.
and brainstem symptoms. In CT hematoma is visualized They are due to aseptic inflammation with formation
as a zone with high density, while the cerebral contusion of adhesions and arachnoid cysts. In convexity arach
and edema - with reduced density. In larger and more noiditis are observed epileptic seizures. In basal arach
superficial hematoma the treatment is surgical evacu noiditis are damaged cranial nerves and the CSF flow is
ation of the hematoma. In unresectable hematoma the desturbed. In arachnoiditis in the pontocerebellar an
treatment is conservative with anti-edema, hemostatics gle are affected VII and VIII cranial nerves, while in
and symptomatic agents. The prognosis is poor in 2/3 optochiasmal arachnoiditis the visual acuity and visual
of patients. fields are impaired.
5) Normal pressure hydrocephalus. The condition
is due to impaired CSF absorption at the level of the
16.5. DELAYED TRAUMATIC COM arachnoid granulation. It develops weeks and months
PLICATIONS after the injury and is manifested by the triad: progres
sive dementia, ataxia and incontinence. The treatment is
Traumatic cerebrasthenia (Cerebasthenia tra u with ventricle-peritoneal or ventricle-atrial shunt.
matica). It develops in about half of patients with cer
ebral concussion. The complaints have neurotic-like
character - headache that increases during physical and 16.6. SPINAL CORD TRAUMAS
mental stress, nausea, fatigue, reduced performance,
sleep and memory disturbances, increased irritabili The spinal cord traumas are observed mainly in
ty, emotional lability, dizziness and instability when young and middle age due to road accidents, sporting,
walking. Common are the autonomous disorders - or professional and household injuries. Their annual fre
thostatic hypotension, tachycardia, sweating, flushing quency is about 5/100 000. Men are affected three times
or pallor. The neurological examination does not reveal more often. The spinal trauma is the most common
focal symptoms and the neuroimaging tests are normal. cause of paraplegia and has great social importance be
The prognosis is good and the complaints disappear for cause of serious and permanent disability.
several months. The treatment is carried out with a noo The spinal cord is the most severely injured in trau
tropic, analgesics, anti-vertigo agents and anxiolytics. matic vertebral fracture with dislocation and compres
Traumatic encephalopathy (Encephalopathia sion of the spinal cord to the point of complete anatom
traum atica). It arises from more severe injuries - cer ical interruption. Mostly are affected the transitional
ebral contusion, post-traumatic hemorrhage, cranial areas - cervical-thoracic and thoracic-lumbar. The spi
compression fractures. In the brain are observed patho- nal cord is damaged also in a sudden strong rotation,
morphological changes that are demonstrated on CT hyperextension (whiplash in the cervical area) or hyper
and MRI - contusion lesions, glial scars, arachnoiditis, flexion. Pathomorphologically are established contusion
arachnoid and cerebral cysts, hydrocephalus, etc. Paral lesions, myelomalacia, hematomyelia and in later stages
lel to the cerebrastenic symptoms focal cerebral symp - posttraumatic cysts, gliosis, and arachnoid adhesions.
toms are available: Concussion of the spinal cord (Commotio medul-
1) Negative neurological syndromes - hemiparesis, lae spinalis). Morphological changes are absent and the
hemihypoesthsia, hemianopsia, aphasia, ataxia, crani clinical symptoms are transient. Most often develops
al nerve lesions. In subcortical involvement develops spinal shock with flaccid paresis, areflexia, conducting
post-traumatic Parkinson’s syndrome and hypotha anesthesia below the level of trauma, and sphincter dis
lamic syndrome (diabetes insipidus, obesity, impaired orders. In upper cervical injuries transient loss of con
thermoregulation). The damage to the limbic structures sciousness can be observed. Complete recovery occurs
occurs with memory disorders (affection of the fixative in a few days.
memory with anterograde amnesia); Spinal cord contusion (Contusio medullae spi
2) Post-traumatic epilepsy - often develops after nalis). The spinal cord contusion is most often a con
open cranial-cerebral trauma and damage of the hip sequence of fracture and dislocation of the vertebra,
pocampus. Typically occurs in the first year after the wherein the contusion necrosis, petechial hemorrhages
injury, but it is possible the onset to be after 10 and more and regional edema of the spinal cord develops. The
years. The epileptic seizures are partial or complex with clinical picture is of spinal shock with plegia and anes
secondary generalization. thesia below the level of the injury and with sphincter
3) Cognitive and psychiatric disorders. The so- disorders. The upper cervical injury is complicated by
called posttraumatic personality change develops - the swelling of medulla oblongata, development of bulbar
patients become circumstantial, emotionally unsta palsy and death. The stage of the spinal shock is fol
ble, dysphoric, sometimes aggressive, more rarely de lowed by spinal automatism - spastic para- or quadriple-
pressed. Permanent cognitive impairment with involve gia, reflexes of spinal automatism, conducting anesthe
128 / Clinical Neurology
sia, bladder automatism, and development of decubitus. Partial or complete interruption of the spinal
Epidural hematoma. It develops a few hours after cord. It is followed by a definitive negative symptoms.
the trauma and clinical onset is with radicular pain, fol In complete interruption is observed paraplegia or
lowed by a progressive compressive transverse lesion of quadriplegia, paraanesthesia, spinal and sphincter au
the spinal cord. tomatism.
Subdural hematoma. It develops more slowly - for The accurate diagnosis and severity of the spinal
a few days to a few weeks with signs of slowly progres cord injury is established by MRI. The prognosis de
sive paraplegia, parahypoesthesia and pelvic reservoir pends on the proper behavior immediately after the in
disorders. jury with complete immobilization, placing a cervical
Hematomyelia is a result of hemorrhage into the pa collar and careful transportation. The therapy in most
renchyma of the spinal cord at the time of the injury. cases is surgical - decompression of the spinal cord,
The clinical picture is of spinal shock with complete or reposition and stabilization of the spinal column. There
partial lesion of the spinal cord. after a continuous neurorehabilitation is recommended.
17. Epilepsy / 129
17. EPILEPSY
Epilepsy is a chronic brain disease of various etiol I he classification of the epileptic seizures, accord
ogies, which is characterized by recurrent paroxysmal ing to the International League Against Epilepsy (1981)
attacks, often with loss of consciousness. It results of is as follows:
abnormal neuronal discharges of a certain population of 1. Partial (focal) seizures.
neurons. Epilepsy has a relatively high incidence - the 1.1. Simple partial seizures (without change of con
morbidity is - 5-10 / 1000. About 50% of cases start be sciousness, the epileptic focus is in the primary cortical
fore the age of 10 and 75% - before the age of 20 years. fields):
The etiology of epilepsy is different in the differ • with motor symptoms;
ent age periods (fig. 17.1). In the neonatal period most • with somatosensory or specific sensory symp
common cause is the prenatal damage, perinatal hypox toms;
ia and ischemia, intracranial hemorrhage and trauma, • with autonomous symptoms;
acute brain infection, metabolic disorders (hypoglyce 1.2. Complex partial seizures (with disturbances of
mia, hypocalcemia, and pyridoxine deficiency), con consciousness, the epileptic focus is in the associative
genital abnormalities. In childhood potential causes are fields):
infections, trauma, developmental disorders, genetic • starting as simple partial seizures and pro
factors, etc. In adults brain trauma, tumors, vascular gressing with change of consciousness;
malformations and diseases, metabolic disorders can • with impaired consciousness from the onset.
cause epileptic seizures. The formation of the epilepto 1.3. Partial seizures with secondary generalization.
genic focus is a result of the interaction of genetic fac 2. Primary generalized seizures (bilaterally syn
tors (idiopathic epilepsy) and acquired factors (symp chronous and symmetrical clinical and EEG manifesta
tomatic epilepsy). Epilepsy is divided to lesioning and tions with disturbance of consciousness due to a sudden
non-lesioning depending on whether there is or there is and simultaneous cortical engagement of epileptic focus
.no macroscopic cerebral lesion. in the synchronization structures (thalamus, reticular
Pathogenesis. In partial epilepsies the epileptogenic formation).
focus is localized in the cerebral cortex or amygdala and 2.1. Absences;
hippocampus. In generalized epilepsy the localization Atypical absences;
is in the oscillating cortico-talamo-cortical circuit. The 2.2. Myoclonic seizures;
epileptogenic focus consists of a population of neurons 2.3. Clonic seizures;
with abnormal membrane excitability due to a prima 2.4. Tonic seizures
ry defect in the neuronal membrane and ion channels 2.5. Tonic-clonic seizures;
with instability of membrane potentials, membrane 2.6. Atonic seizures;
ATP-dependent ion transport, GABA inhibitor systems, 3. Unclassified epileptic seizures.
of receptors of the excitatory neurotransmission (glu
tamate, aspartate), etc. The epileptic seizure occurs in Clinic picture of the partial (focal) seizures.
abmnormal synchronous neuronal depolarization in the
epileptogenic focus with subsequent propagation ot the 1. The simple partial seizures have diverse pres
epileptic activity. entation: motor, sensory, olfactory, gustatory, audito
ry, vestibular, visual, and psychic. The epileptic focus
is in the primary cortical fields and the consciousness
Idiopathic
is preserved. In epileptogenic focus in gyrus precen
Febrile tralis (area 4) are observed clonic seizures in the con
Birth injury tralateral half of the body, starting more often from
Metabolic the face and upper limb. The motor Jacksonian sei
Infection zure can progress to secondarily generalized sei
Trauma zure. Sometimes after the seizure remains transient
postictal paresis. In epileptogenic focus localized in
Tumor
gyrus postcentralis occur seizures with paresthesia
Stroke
and dysesthesia in the contralateral half of the body
(sensory Jacksonian seizure). Sometimes the sensory
Jacksonian seizure progresses into motor and gener
age / years
alized seizure. The adversive seizures result of excita
F ig . 17.1. Causes of seizures as a function of age at onset tory focus in the premotor cortex (areas 6 and 8 of the
(Aminoff) frontal lobe) and are characterized by a tonic deviation
130 / Clinical Neurology
of the eyes, head and sometimes the whole body to the A tonic contraction of all muscles including the respira
opposite side. Olfactory seizures are due to a focus in tory muscles occurs - dorsal extension of the head, ex
the temporal lobe or in the basal part of the frontal tension of the limbs, trismus with a biting of the tongue,
lobe, while in the gustatory seizures the focus is in the sudden loud scream (ictal cry), apnea with cyanosis,
opercular area. The auditory seizures are divided into emptying of the pelvic reservoirs, increasing of the
simple (sounds, noises) or complex (melodies, voices, sympathetic tone (tachycardia, hypertension, mydria
sometimes frightful) and are due to a focus in the tem sis). The tonic phase lasts 15-20 s, followed by a deep
poral lobe. The vestibular seizures (dizziness, feelings and noisy breathing, clonic convulsions in the limbs,
of frustration, flying) are also associated with focus bloody foam at the mouth, and sweating (fig. 17.2). The
in the temporal lobe. In focus in occipital lobe are ob clonic phase lasts up to a minute and is followed by
served simple (light points, lines, scotoma) or complex postictal phase, which has varying duration - gradual
visual hallucinations (images, pictures, visions, some exit from coma, confusion with psychomotor agitation,
times frightful). In damage to the limbic system (gyrus somnolence, and lethargy. EEG in all leads during the
cinguli) are observed autonomous seizures - redness, tonic phase registers growing fast rhythms (spikes), and
pallor, hot flashes, unpleasant epigastric feeling that during the clonic phase - bilaterally synchronous spike-
rises to the throat, salivation, etc. The seizures with slow wave or multiple spikes-slow wave and in the pos
psychic events (dreamy state) are due to a focus in the tictal phase - diffuse slow delta waves or isoelectric
temporal or frontal lobe - attacks of sudden fear, vi line. Therefore, typical for grand mal are: a sudden loss
olent thoughts, unreal perception of the surrounding of consciousness, falling to the ground, injuries, biting
world (derealisation) or of own body (depersonaliza of the tongue, incontinence, mydriasis, pupil areflexia
tion), deja vu (already seen) or jam ais vu (never un and lack of memory (amnesia).
seen), macropsia or micropsia (objects are perceived as • Generalized myoclonic seizures. The general
larger or smaller), macromelia and micromelia (body ized myoclonic seizures are manifested by a short and
parts are perceived as larger or smaller), aphasia, etc. fast massive muscle contractions of the limbs and body,
Simple partial seizures can progress into complex par not always accompanied by consciousness disorders.
tial or generalized tonic-clonic seizures. Sometimes they are combined with tonic-clonical sei
2. In complex partial seizures are involved the zures or sudden loss of muscle tonis. Often are triggered
associative cortical fields (mainly of the temporal and by awakening and photo stimulation. EEG registers bi
frontal lobe). The consciousness is disturbed due to lateral synchronous paroxysms of spikes-slow wave.
the propagation of excitation to the contralateral hem • Generalized tonic seizures. The generalized
isphere and brainstem. The attack occurs suddenly or tonic seizures are characterized by tonic spasm of the
gradually, and its duration is from 30 s to 3 min. The muscles - extension of the head, body and legs with flex
consciousness is impaired, there is no memory of the ion of the arms, and duration 5-30 s. Most often occur at
seizure, after the seizure the patient is disorientated night and are observed in syndrome of Lenox-Gastaut.
for time and place. The seizure often begins with aura EEG detects generalized increasing rhythms (spikes).
(simple partial seizure), which is stereotyped in a pa • Generalized clonic seizures. They are symmet
tient. The early ictal phase is manifested with an abrupt rical, sometimes last longer and can cause falls. They
change in behavior, and an expressionless face. Motor are typically observed in children with febrile seizures.
automatisms (chewing, lip smacking, licking, swallow EEG registers bilaterally synchronous paroxysms of
ing, scratching, rubbing) or more complex meaningless spike-slow wave.
actions (probing, searching, undressing, tear
ing, urination, and aimlessly running) are ob
served.
3. Clinical picture of the generalized sei
zures. The generalized seizures are charac
terized by bilateral clinical and EEG manifes
tations without obvious focal onset and are a
result of excessive widely spread discharge of
the cortical neurons or of thalamus and retic
ular formation. Clinically are manifeted with
loss of consciousness, bilateral symmetrical
motor activities and autonomous disturbanc
es. EEG registers synchronous bilateral, sym
metrical, generalized paroxysms. The gener
alized seizures are divided into convulsive
and non-convulsive.
• Tonic-clonic seizures (grand mal).
They start with a sudden loss of conscious F i g . 17.2. Generalized tonic-clonic seizure, illustrating the appearance of
ness without aura, with a fall to the ground. the patient in tonic (stiffening) and clonic (shaking) phases (Aminoff)
17. Epilepsy / 131
• Generalized atonic seizures. They are due to a occurs with prolonged disturbance of consciousness,
sudden myoclonia and loss of muscle tone of the whole amnesia and automatisms. Epilepsia partialis continua
body with falling to the ground or loss of muscle tone (of Kozhevnikov) is characterized by clonic or tonic sei
of the neck muscles with a droop of the head. They are zures involving a part of the limb, often in combination
common in Lenox-Gastaut syndrome. with a motor deficit.
• Absence seizures (generalized non-convulsive Classification of epilepsy. According to their etiol
seizures). The absences are divided into typical (sim ogy the epilepsies are divided into symptomatic (40%)
ple and complex) and atypical. EEG in typical absenc - with proven etiology, cryptogenic (40%) - with sus
es showes bilateral synchronous paroxysms spike-slow pected but unproven etiology and idiopathic (20%) - no
wave 3 in a second with a sudden start and end, and proven reason other than hereditary. The symptomat
in atypical absence seizures - spike-slow wave 2.5 in ic epilepsy results of various reasons: vascular (15%),
a second with a gradual onset and end (fig. 17.3). The brain tumors (6%), alcoholism (6%), cranial trauma
typical simple absences are characterized by transient (3%), neuroinfections (3%), malformations, degenera
disturbance of consciousness (5-30 s), sudden onset and tive and metabolic diseases, etc (7%). The International
end, suspension of the committing action, fixation of Classification o f epilepsy (1989) is based on the etiolo
the eyes, lack of memory. Sometimes they reach high gy, age of onset, seizure type, EEG, triggers, evolution
frequency - up to 100 per day (picnolepsia). The typical and prognosis.
complex absences are often combined with automatisms
(continuation of initiated action), changes in the muscle I. Partial idiopathic (primary) epilepsies.
tone (decrease or increase), autonomous manifestations • Benign infantile onset epilepsy with centro-tem-
(redness of the face, rarely micturition). The atypical poral spikes (epilepsy with Rolandic paroxysms). The
absences have greater duration (60 s), gradually begin onset is between 3 and 13 years of age. It represents 20%
ning and end, and are often combined with automatisms of all epilepsies in the school age. The disease affects
and motor asymmetric events (clonic movements, droop boys more often and often has a pronounced family his
of the head), autonomous events (changes in breathing, tory (chromosome 15q 14). It is characterized by partial
micturition). They are typical for Lennox-Gastaut syn motor seizures (oro-buco-pharyngeal) during the day or
drome. night sleep, rarely tonic, clonic and hemicorporal sei
Status epilepticus. It is characterized by recurrent zures. It has a favorable prognosis. Carbamazepine and
seizures (more than 2-3) without recovery of conscious- valproate have good therapeutic efficacy. The benign in
mess between them and continuous epileptic activity fantile epilepsy with occipital paroxysms and the benign
on the EEG (more than 5-10 min). Generalized and psychomotor epilepsy have a similar evolution.
partial status epilepticus is distinguished. The gener
alized status is divided into convulsive (tonic-clonic, II. Partial symptomatic (secondary) epilepsies
tonic, clonic, myoclonic) and non-convulsive (absence). • Temporal epilepsy. The disease represents 60%
The generalized convulsive status is a life-threaten of all symptomatic epilepsies. The etiology is deter
ing condition because of the development of cerebral mined in 70% the cases with MRI - atrophy and scle
edema, hyperthermia, aspiration, and electrolyte dis rosis of the hippocampus and amygdala, posttraumatic
turbances. Status epilepticus with recurrent simple gliosis of the hippocampus, arterio-venous malforma
partial seizures is characterized by persistent negative tion, hamartoma, cavernoma, disembrional tumors, fo
focal symptoms between the seizures in preservation of cal dysplasia, etc. It is characterized by complex partial
consciousness. The complex partial status epilepticus seizures, often with secondary generalization - autono-
i АЛ л.
ft 'ж
шшшшт
нишаншит
Fig. 17.3. EEG o f a patient w ith typical absen ce (petit mal) seizures, showing a burst of generalized 3 Hx Plkc
that is sym m etric and bisyn ch ron ous (A m in o tt)
132 / Clinical Neurology
mous events, olfactory, auditory or vestibular hallucina The onset is between 3 and 7 months of age. The dis
tions, psychomotor and psychosensor events, motor and ease is more often observed in boys. Tonic flexion and
verbal automatisms, aphatic disorders. less often extension seizures are observed, lasting 1-15
• Frontal epilepsy. The disease is characterized s, sometimes in a series. The seizures are accompanied
by a large clinical polymorphism - Jacksonian motor by a cry or smile. In parallel, delay in the psychomotor
seizures, adversive seizures, discontinuation of speech development of the child is observed. The child stops
or vocalization, forced thoughts, axial clonic move smiling, cannot hold his head and sit. There are signs
ments. In orbital-frontal localization are observed ol of encephalopathy - quadriparesis or hemiparesis, mi
factory hallucinations, gestures and motor automatisms, crocephaly, etc. Generalized or partial seizures are also
autonomous events. possible. EEG reveals the so-called “hyipsarrhythmia”.
• Occipital epilepsy. It is characterized by sim The prognosis is unfavorable. The disease progresses
ple negative (amaurosis, scotoma, hemianopsia) or ex towards the syndrome of Lennox-Gastaut. The treat
citatory (bright dots, ribbons, stars) visual manifesta ment was performed with valproate, vigabatrin, predni
tions, visual hallucinations, macropsia and micropsia, solone, and ACTH.
perceptions of changing colors and lights, conjugated • Syndrome o f Lennox-Gastaut. The onset of the
deviation of the head and eyes. The rapid propagation encephalopathy is between 1 and 8 years old. The boys
forward in the other lobes and the secondary generali are more often affected. Frequently a family history is
zation is typical. present. The disease represents 5-10% of all epilepsies
in the childhood. The clinical course is with a variety of
III. Generalized idiopathic epilepsies. seizures - atypical absence, combined with motor ton
They are characterized by an absence of neurologi ic, atonic or myoclonic seizures, partial or generalized
cal and intellectual disorders, absence of changes on CT tonic-clonic seizures. In 75% of the patients is observed
and MRI, presence of genetic predisposition, general status epilepticus. In 90% of the patients is detected
ized clinical and EEG changes, onset of the seizures in psychomotor retardation. The prognosis is unfavorable.
a typical age. The treatment is performed with valproate and clonaz
• Infantile epilepsy with absences (petit mal, pic- epam.
nolepsia). Its onset is between 3 and 10 years and often a
family history is present. It is characterized by frequent V. Febrile seizures.
(up to 100 per day) typical absence seizures, simple or They are observed up to 5 years of age. The boys
complex, which are triggered by hyperpnoea. EEG re are more often affected. Frequently a family history is
cords typical bilateral synchronous paroxysms of spike- present. The seizures occur in the course of intercurrent
slow wave with frequency 3 per second. The disease febrile illness with hyperpyrexia above 38°C as gener
is easily controlled by valproate and ethosuximide. At alized tonic-clonic seizures or myoclonus with duration
puberty occurs recovery or occurrence of tonic-clonic up to 30 minutes. The prognosis for recovery is good.
seizures. The risk of subsequent epilepsy is between 2% and 5%
• Juvenile epilepsy with absences. The onset of cases. A prophylaxis with valproate is rarely neces
is between 10 and 15 years of age with rare and brief sary. During the seizure rectal diazepam is applied.
absences, sometimes combined with myoclonus of the Diagnosis of epilepsy. The medical history of the
limbs, head and body and generalized tonic-clonic sei patient and his relatives is very important. In simple par
zures. The seizures are provoked by tiredness, waking tial seizures due to a lack of amnesia, the medical his
up and menses. The patients respond well to valproate, tory of the patient reveals the nature of the seizures. In
but the treatment lasts for years, including into adult complex partial and generalized seizures due to distur
hood. bances of consciousness and lack of memory history of
• Juvenile myoclonus epilepsy (syndrome of relatives and eyewitnesses of the seizure is needed - the
Janz, petit mal impulsive). The onset is between 8 and onset of the seizure is specified, the availability of fall
26 years of age with a generalized myoclonus, usually ing, biting of the tongue, pelvic reservoirs disturbances,
in the morning upon awakening, provoked by sleep dep types of the seizures, changes in the diameter of pupils,
rivation, alcohol, and photo stimulation. The myoclonic and the postictal condition of the patient. The family
seizures are often combined with grand mal. Treatment history specifies the presence of genetic predisposition.
with valproate is effective, but lasts for years. Carba- The obtaining of information of prenatal, perinatal and
mazepine worsens the seizures. postnatal injury, cranial trauma, poisoning, infectious
• Epilepsy with generalized tonic-clonic seizures diseases, malformations, and tumors is necessary. The
on awakening. The onset is between 10 and 25 years of medical history specifies the provocative factors - sleep
age. The therapeutic response to valproate and carba- deprivation, hyperventilation, photo stimulation, fa
mazepine is good. tigue, intoxication, fever, concomitant disease, etc. The
findings of the neurological examination help to clarify
IV. Generalized cryptogenic or symptomatic epilep the etiology of the epileptic seizures.
sies EEG is a basic examination in epilepsy, which al
• Syndrome o f West. It was described in 1841. lows localization of the epileptic focus, determins the
17. Epilepsy / 133
type of epileptic discharges and seizures, distinguish served intermittent tonic-clonic seizures.
es the idiopathic from symptomatic epilepsies. Typical The psychogenic (hysterical) pseudo seizures are
findings are the spikes, sharp waves and their combi sometimes difficult for differentiation from general
nation with slow waves. The sudden and synchronous ized seizures. The onset is gradual, the consciousness
occurrence of these graphoelements in more than two is narrowed, the pupils react to light, the movements are
channels of EEG is termed paroxysmal activity. Gener scattered, theatrical, incontinence, tongue biting and
alized paroxysms occur in absence seizures, myoclonic, injuries are absent, the duration is longer and a partial
tonic-clonic, tonic, clonic and atonic seizures. The focal memory of the seizure is present.
paroxysms are more common than the generalized, and Narcolepsy (sudden attacks of sleep), cataplexy (sud
are divided to ictal with clinical manifestation and in- den loss ot muscle tone while awake) and parasomnias
terictal in the period between the seizures. In 20-30% fsomnambulismus, enuresis nocturna, pavor nocturnus,
of cases EEG remains normal. In these cases a provoc jactacio capitis) and physiological sleep myoclonus are
ative tests are applied - hyperventilation, photostimu often misinterpreted as epileptic seizures.
lation, and sleep deprivation. The diagnostic value of Differential diagnosis is also made with paroxys
EEG increases in computer analysis of the frequency mal myoplegia (periodic paralysis), tetanic seizures in
and amplitude of the waves, EEG mapping, video-EEG, hypocalcemia, hemifacial spasm, transient ischemic
Holter-EEG, and polysomnography. With EEG the ef attacks, gastroesophageal reflux disease, panic attacks,
fect of treatment is also monitored. etc.
CT and MRI are necessary for diagnosis of the lesion Treatment. The treatment is based on the precise
epilepsies - posttraumatic cysts, unilateral or bilateral clinical and EEG diagnosis of epileptic seizures, mon
gliosis of the Ammonium horn, heterotopia of ganglion otherapy with most efficient and least toxic product and
cells, vascular lesions, vascular malformations, tumors, polytherapy in refractory forms of epilepsy. Depending
etc. SPECT and PET contribute to clarification of the on the therapeutic spectrum antiepileptic drugs (AEDs;
epileptogenic focus. AEM) are divided into:
Differential diagnosis. The abortive grand mal and 1) AEM with the widest spectrum (they are effective
absences must be distinguished from syncope. In syn in all types of seizures including the typical absences):
cope is observed episodic loss of consciousness and valproate, benzodiazepines, Lamotrigine, Topiramate,
decrease of the postural tone due to cerebral hypop Levetiracetam.
erfusion. It can be provoked by strong emotion (see 2) AEM with a narrower spectrum (they are effec
ing blood, accident, intense fear, pain), stay in hot and tive in all types of seizures except for typical absences):
stuffy room, suddenly getting up from lying down, etc. Phenobarbital, Phenytoin, Primidone, Carbamazepine,
The loss of consciousness is with shorter duration and Oxcarbamazepine, Levetiracetam, Gabapentine, Vigab-
not as sudden as in an epileptic seizure, the postictal atrine, Tiagabine, Pregabalin, Lacosamid.
confusion is absent. The disorder is preceded by dark 3) AEM with most narrow spectrum (effective only
ening the eyes, ringing in the ears, faint feeling, nau in typical absences) - Ethosuximide, Trimethadione.
sea, pallor, sweating. The arterial hypotension is typical In view of the efficacy / risk ratio, and effects on
for syncope. In more severe syncope of cardiac origin cognitive function, drugs of first choice are Valproate
can be observed palpitations and arrhythmia, mild con and Carbamazepine. Therefore, treatment begins with
vulsions (convulsive syncope) and even incontinence. one of the two drugs and in the absence of satisfacto
In the syndrome of Morgani-Adams-Stokes (complete ry effect is switching to other drugs or to polytherapy.
atrioventricular block with heart rate below 40) are ob In polytherapy are possible drug interactions, enzyme
induction and increasing the risk of side effects. This treatment (lesionectomy, amigdalohipocampectomy,
requires monitoring of the plasma levels of some drugs, anterior temporal lobectomy, calosotomy).
monitoring of liver function, blood counts, creatinine Status epilepticus. At the incident site are taken the
level, etc. The new AEDs have more appropriate phar following measures: providing respiratory passage, pre
macokinetics and a lower risk of pharmacological inter venting the tongue from biting (placing a soft object be
actions and are indicated for combination use. The daily tween teeth or tongue holder), prevention of aspiration
dose of AED is determined by the body weight (mg / kg (placing the head sideways) and injuries, cleaning the
/ d), the patient’s age and clinical effect. Thus, the daily mouth, providing a venous pathway, application of Di
dose of valproate is 20 mg / kg for adults and 30 mg / azepam 10 mg i.v. or i.m. (Clonazepam 2 mg i.v. or i.m.)
kg in children, carbamazepine - respectively 15-20 mg and / or Phenobarbital 200 mg i.m. In the intensive care
/ kg and 20-25 mg / kg, phenytoin - 3-4 mg / kg and 4-8 unit is performed administration of oxygen, possibly
mg / kg, of clonazapem - 0,01-0,02 mg / kg, lamotrigi- intubation, monitoring of the ECG, respiration, blood
ne - 150-500 mg / 24h, topiramate - 200-400 mg / 24 h, pressure, heartbeat, laboratory tests. In the absence of
levetiracetam - up to 5000 mg / 24 h. The treatment is effect are administered Diazepam 20 mg i.v. (Clonaz
monitored clinically and with ECG and is discontinued epam 1 mg) slowly, Phenytoin 15 mg / kg i. v. infusion
gradually after a period of 2-5 years free of seizures of up to 50 mg / min or Depakine 15 mg / kg bolus, fol
and at normal EEG. Besides pharmacotherapy patients lowed by perfusion of 1 mg / kg / h for 5 h. Anti-edema
are advised to follow certain diet (low-salt, ketogenic), treatment is applied with Mannitol and Furosemide. The
to avoid provoking factors (sleep deprivation, alcohol water-electrolyte and acid-base balance are maintained.
consumption, photo stimulation, hyperventilation, etc.). In the absence of effect are administered Diazepam
In treatment resistant forms of epilepsy are applied (Clonazepam) and i.v. perfusion with Phenytoin 50 mg /
other methods - vagal nerve stimulation, and surgical min. The last drug of choice is Thiopental.
18. Headache / 135
18. HEADACHE
The headache can be an independent neurological tical hypoperlusion, which begins in the visual cortex
disease (primary headache) or a symptom of another and spreads forward with speed 2-3 mm / min, but the
disease (secondary headache). According to the Inter degree of hypoperfusion is not sufficient to induce fo
national Headache Society Classification (2004) the cal neurological symptoms. According to the neuronal
headache is divided into the following types: 1. Mi theory of migraine, the spreading forward cortical de
graine; 2. Tension headache; 3. Cluster headache and pression, caused by pre-existing metabolic activity with
chronic paroxysmal hemicrania; 4. Other primary head the release of potassium, is causing the migraine attack.
aches; 5. Headache in cranial and cervical injuries; 6. The electrical stimulation of nucl.raphe dorsalis in the
Headache in vascular diseases; 7. Headache in non-vas- upper brainstem causes migraine headache. The cere
cular intracranial diseases; 8. Headache caused by med bral blood flow in pons and midbrain is increased during
ications or their discontinuation; 9. Headache in intrac the migraine attacks, probably due to the increased neu
ranial or systemic infections; 10. Headache in metabolic ronal activity of nucl.raphe dorsalis and locus ceruleus.
disorders; 11. Headache or facial pain in diseases of the This brainstem generator of migraine is inhibited during
cranium, neck, eyes, ears, sinuses, teeth, jaws, and oral sleep and of some antimigraine medicaments. Seroton
cavity; 12. Headache in mental diseases; 13. Cranial ergic projections are established from nucl. raphe to the
neuralgias; 14. Not classified headache. In the group of cerebral arteries and visual centers that can determine
primary headaches are included: 1. Migraine; 2. Cluster the visual and circulatory disorders. According to the
headache; 3. Chronic paroxysmal hemicrania; 4. Ten trigeminal-vascular theory the activation of the cells in
sion headache; 5. Chronic daily headache. nucleus caudalis n.trigemini leads to release of vasoac
tive neuropeptides (substance P, calcitonin gene-relat
ed peptide) in the vascular terminals of the trigeminal
18.1. MIGRAINE nerve and to development of aseptic inflammation in the
vascular wall and meninges with activation of the noci
The migraine is a primary headache which is char ceptive trigeminal afferents. In the pathogenesis of mi
acterized by recurring attacks of unilateral pulsating graine is also involved serotonin (5-hydroxytriptamine).
headache, accompanied by nausea, vomiting, photo- Methysergide, the first medicament for prevention of
and phonophobia. Its onset is most often in puberty and migraine attacks, antagonizes the peripheral effects of
in the majority of cases family history of the disease 5-HT. The level of 5-HT in the platelets is decreased at
is present. The migraine is the most common cause of the onset of the headache. The drugs that release ser
headache - it affects 15% of women, 6% of men and 4% otonin, can cause migraine headache. Triptans, which
of children. are used to treat migraine attack, are agonists of 5HTlb,
Classification. The migraine is subdivided to mi 5HTld and 5HTlf receptors. The stimulation of 5HTlb
graine without aura (common migraine), which is ob receptors, which are localized in blood vessels and
served in 75% of the cases and migraine with aura (clas nerve terminals, has anti-migraine effect. A genetically
sical migraine) - in 25% of patients. Less common forms determined hypersensitivity of dopamine receptors, im
are basilar migraine, ophthalmoplegic migraine, retinal balance of neurotransmitters and impairment of central
migraine and familial hemiplegic migraine. In the child inhibitory mechanisms are also supposed.
hood are observed periodic syndromes that can precede Clinic. The onset of migraine is most common in ad
the onset of migraine or can be associated with it. olescence, but can also occur in childhood or in later age.
Pathogenesis. In most patients with migraines are The migraine attack occurs in four stages - prodromal,
available data for hereditary predisposition. Genetically aura, headache and post-headache phase. The prodromal
determined dysfunction of the ion channels is supposed phase is observed in one third of the patients. It occurs
- i.e. migraine belongs to the group channelopathies. In hours to days before the headache and is manifested by
the familial hemiplegic migraine in 19 chromosome is depression, euphoria, increased or decreased appetite,
detected defect in a gene that encodes a P / Q calcium thirst, fluid retention, oliguria, etc. In 15% of the patients
channel expressed only in the CNS and regulating cal the headache is preceded by an aura, which develops in
cium-induced release of neurotransmitters. According about 20 min and continues up to 1 h. The most typical
to the vascular theory of migraine, which is now aban is the visual aura - scintillating scotoma and photopsia
doned, the aura is due to intracranial vasoconstriction, that gradually grow and move from the center to the pe
and the pulsating headache - of extracranial vasodilata riphery of the visual field, sometimes distorted images
tion. The examinations of the cerebral blood flow have (metamorphopsias, micropsia, macropsia) and visual
shown that in the classical migraine exists relative cor hallucinations. Rarer is the sensory aura - paresthesias
136 / Clinical Neurology
in the contralateral upper limb, lips, tongue, sometimes Differential diagnosis is made with tension, cluster
with spreading in the contralateral half of the body. In and other more rare primary headaches, as well as sec
the familial hemiplegic migraine during the aura devel ondary, symptomatic headache, associated with brain
ops hemiparesis, which persists for more than one hour. tumor, neuroinfections, trauma, cerebrovascular dis
The symptoms observed in the basilar migraine are from ease, vascular malformation, vasculitis, sinusitis, glau
both occipital lobes and brainstem - visual disturbances, coma, etc. - Fig.18.1, Fig. 18.2. For exclusion of a sec
dizziness, tinnitus, diplopia, dysarthria, ataxia, some ondary headache are applied appropriate examinations
times impaired consciousness. These symptoms of the - CT, MRI, cerebral angiography, CSF examination, etc.
vertebral-basilar system are followed by occipital head Treatment. The treatment of the migraine attack is
ache. In some cases the aura is not followed by headache more effective in the early stages and is held in milder
- migraine aura without headache (migraine equivalent). cases with analgesics (Aspirin, Paracetamol) in com
Migraine headache starts gradually and grows rapidly, bination with Coffein, Codein, NSAIDs and antiemet
continuing 4-72 hours. The headache is unilateral (hemi- ics (Metoclopramide). Specific agents are 5-HTl-ago-
crania), intense, pulsating and most often localized in the nists - ergotamine and triptans. Ergotamine tartarate,
temporal lobe and the orbital area, with irradiation to the Dihydroergotamine (tabl., supp., amp., nasal spray)
frontal and occipital region. More rarely the headache is are non-selective serotonin agonists and are the oldest
bilateral or changes its side. The headache is accompa preparations. In our country is applied the combined
nied by nausea, vomiting, photophobia and phonophobia, preparation Coffergamin (100 mg coffein and 1 mg
infrequently by osmophobia, pallor, sweating, stiffness in ergotamin). Triptans (Sumatriptan, Eletriptan, Riza
the neck, and bulging temple blood vessels. In migraine triptan, Naratriptan, Zolmitriptan) are selective agonist
status the headache lasts more than 72 h, despite of the of the 5-HT1 inhibitory presynaptic serotonergic recep
ongoing therapy. Very rarely a cerebral infarction can de tors. They inhibit the serotonergic transmission and the
velop - the focal symptoms during aura persist for more neurogenic inflammation. In more severe attacks are ap
than 7 days and CT / MRI visualize ischemic zone. In plied Dexamethazone and inhalation of oxygen. Prophy
the ophthalmoplegic migraine the headache is followed lactic treatment is applied in more than 3 relapses per
by ptosis, diplopia, and mydriasis, which persist for 1-8 month, in side effects of the treatment and in hemiplegic
weeks. Most often it affects III, rarely IV and VI crani migraine. It is performed with beta blockers (Propran
al nerves. In the retinal migraine are observed recurrent olol), calcium antagonists (Flunarizine), tricyclic anti
transient monocular scotoma or blindness, lasting less depressants (Amitriptyline), anticonvulsants (valproate,
than an hour, after which occurs the migraine headache. Topiramate, Gabapentine).
The familial hemiplegic migraine begins with hemipare
sis, which lasts more than an hour (prolonged aura) and
may persist for up to a week.
Migraine headache is provoked by mental stress,
physical stress, menstruation, certain foods and drinks
(cheese, cocoa, chocolate, nuts, citrus juices, canned
food, red wine), drugs (nitroglycerin and nitrate prepa
rations, reserpine, levodopa, hypoglycaemic agents,
contraceptives ), changes in barometric pressure, etc.
The diagnosis is made in the presence of at least
two attacks of migraine with aura or 5 identical attacks
of headache without aura, unilateral location, pulsating
character, nausea, vomiting, photophobia, and phono-
phobia. The absence of a history and clinical data for 0 3 6 9 12
secondary (symptomatic) headache is obligatory. The Time (monts)
migraine attack can be provoked by administration of
nitroglycerin as a diagnostic test. Fig. 18.1. Temporal patterns of headache (Aminoff)
Pain
Ptosil, _
myosis
Tearing^
Nasal stiff—
iness and
dischar
cramp
Fig. 18.2. Differential diagnosis of headache (Aminoff): 1. Migraine, 2.Trigeminal neuralgia, 3. Postherpetic neuralgia, 4. Clus
ter headache, 5. Tension headache
18. Headache / 137
18.2. CLUSTER HEADACHE Differential diagnosis is made with a number of
diseases, migraine without aura, chronic paroxysmal
In the past this type of headache was recognized hemicrania, trigeminal neuralgia, and temporal arteritis
under different names: grouped headache, histamine of Horton, syndrome of Tolosa-Hunt, corneitis, scleritis,
headache, headache of Horton, migraine neuralgia, sinusitis, and dental diseases. The symptomatic cluster
Sluder’s neuralgia, Vidian’s neuralgia, eritromelalgia of headache can be observed in parasellar meningiomas,
the head, petrosal neuralgia, neuralgia sphenopalatina, pituitary adenoma, aneurysm of the anterior commu
hemicrania periodica neuralgiformis. It occurs much nicating artery, arteriovenous malformations near the
less frequently than migraine - 0.01 to 1.5%, and affects sinus cavernosus.
six times more often men. Usually starts in the third The treatment of the attack is conducted with
decade. The family history is very rare - in 5%. It is fast-acting drugs because of the transience of the at
divided to episodic, which is much more common and tacks - Summatriptan of 6 mg subcutaneous, Dihydroer-
chronic cluster headache. gotamine (sublingually, suppositories and nasal spray),
The pathogenesis is unclear. Disorders of the cir inhalation of 100% oxygen through a mask. For preven
cadian pacemaker due to hypothalamic dysfunction are tion of cluster’s period are applied corticosteroids, an
suspected. As a result of reduced inhibitory activity ticonvulsants (Valproate, Topiramate), calcium channel
of the periaqueductal gray nucleus and nucleus raphe blockers (Verapamil, Nimodipine, Nifedipine), Ergot
magnus the unilaterally trigeminal-vascular system is amine tartarat 2 mg orally two hours before the time of
activated with subsequent development of neurogenic the expected attack.
inflammation.
The clinical picture is characterized by attacks of
extremely strong burning and tearing unilateral pain in 18.3. CHRONIC PAROXISMAL HEMI
the orbital-frontal area. Sometimes the pain spreads to CRANIA
wards the ear, neck, upper jaw and the entire half of the
head. The cluster attacks are accompanied by at least This headache resembles the cluster, but is distin
one of the following symptoms: ipsilateral conjunctival guished by the dramatic response to indomethacin. Un
injection, lacrimation, nasal congestion, rhinorrhea, fa like cluster headache women suffer 7 times more often
cial hyperemia, sweating of the forehead and face, mio than men. It occurs much less frequently from cluster
sis, ptosis and eyelid edema. The pain attack lasts from headaches. The pathophysiology is unclear; the perio
15 to 180 minutes, on average 45 minutes and was re dicity implies a presence of central generator followed
peated 1 to 8 times daily. Attacks are grouped in series by an autonomic dysfunction. The clinical picture is
(cluster periods) lasting 1-2 months and are linked to similar to cluster headache - attacks of utnilateral se
the daily solar lighting and remissions last more than 14 vere headache, accompanied by nasal congestion, eye
days to months and years (fig 18.1, fig. 18.2). The attacks redness, lacrimation, ptosis and mild swelling of the
occurr about 90 minutes after falling asleep, during the eyelid. The attacks can be triggered by flexion and ro
first REM phase of sleep and less often daytime during tation of the neck. The headache continues for about 13
siesta. The attacks are provoked by small doses of al minutes and is repeated 11 times on average per day.
cohol, from vasodilators such as nitroglycerin and his It occurs episodically with remissions and more often
tamine, stress, heat, fever, gaps in nutrition, sleep until chronically without remissions. The diagnosis is con
late morning. Alcohol in a larger amount postpones the firmed by the typical response to indomethacin.
relapses. The differential diagnosis is as in cluster headache
Variants o f cluster headache are syndromes “clus and is performed with SUNCT - syndrome. The treat
ter-tic”, “cluster-migraine”, “cluster-vertigo” and ment is performed with Indomethacin to 200 mg per day
“SUNCT” syndrome (attacks of brief neuralgic-like or with Aspirin, which has a lesser effect.
headache, conjunctival injection, lacrimation, sweating
and rhinorrhea. The attacks last only 15-60 seconds, but
can be repeated 5-30 times in an hour).
18.4. TENSION HEADACHE
The diagnosis of cluster headache can be suspect
Previously it was termed neurotic, psychogenic,
ed from the history by the typical characteristics ol the
psychogenic-myogenic, muscle contractile, headache
headache - the presence of at least 5 attacks lasting 15-
in stress. This is the most common type of headache.
180 minutes, at least one accompanying symptom and
Mostly affects middle age, more often women. It is di
frequency 1-8 times daily. For confirmation of the diag
vided to episodic (covers up to 15 days per month or 180
nosis is applied provocation of the attack with 1 mg sub
days per year) and chronic (more than 15 days a month
lingual Nitroglycerin, histamine 0,3 mg subcutaneously
or more than 180 days a year). It is also divided into
or a small amount of alcohol. The diagnosis requires a
headache, associated with a tension of the pericranial
lack of history and clinical data for secondary head
muscles and headache without tension on these muscles.
aches. The neurological examination outside the attack
The pathogenesis is unknown. Although in some
reveals increased sensitivity in the area of pain, mild
patients there is a certain contraction of the pericranial
ptosis and miosis and the so-called “leonine tacies.’
138 / Clinical Neurology
muscles due to psychogenic stress, it is believed that this deine and barbiturates can result in drug abuse and in
prolonged muscle contraction, with subsequent muscu the conversion of episodic headache in chronic tension
lar ischemia and formation of algogenic substances has headache. In chronic headache are applied continuously
not been a leading cause of the headache. More likely tricyclic antidepressants (Amitriptuline, Nortriptiline)
it is connected with reduction of serotonin in the CNS and anticonvulsants (valproate).
with disturbance in the central mechanisms of pain con
trol and reduction of the pain threshold.
Clinical picture. The first presentation of the head 18.5. CHRONIC DAILY HEADACHE
ache is after the age of 20 years with onset of headache
in stressful situations. The headache later can become This is a type of the tension headache or a combina
chronic, particularly in abuse of analgesics. The head tion of migraine without aura and tension headache. The
ache covers diffusely the entire head as a hat, some most common reason for the transformation of the epi
times is more strongly in the temples and neck and can sodic headache is chronic abuse of analgesics and com
spread to the shoulders. The headache is tightening, bination products containing caffeine, codeine, barbitu
clamping, low to moderate and is not accompanied by rates, ergotamine, and opioids. The pathogenesis is as
photophobia and phonophobia, nausea and vomiting. sociated with impairment of the antinociceptive system
In episodic headache the duration averages 12 hours. and reduced level of opioid peptides. The classification
In chronic cases upon awakening the patient has head of this headache is the following: 1) primarily migraine
ache or it appears shortly thereafter and gradually in type; 2) mostly tension-type; 3) transformed migraine.
creases at the end of the day along with the increasing Clinical picture. The headache is usually bilateral,
stress during the day. It is possible that the headache is slight, and covering the entire head and neck. The head
accompanied by nausea and photophobia. In 1/4 of the ache often increases relapsing; covers half head and be
patients occurs migraine. Often are observed symp comes stabbing and pulsating and can be accompanied
toms of depression, anxiety, psychosomatic symptoms by nausea and vomiting. This migraine-like headache is
(chest pain and low back pain), sleep disturbances, de caused by the same factors that trigger migraine. It lasts
creased performance. 2-3 days and is replaced by a weaker diffuse headache.
The diagnosis is made when the following crite The most common type of the chronic daily headache is
ria are present: 1) at least 10 episodes of headache that the transformed migraine. In most cases it is a menstru
lasts from 30 minutes to 7 days; 2) clamping or shrink al migraine without aura that after 15 years becomes
ing non-pulsating pain; 3) a weak to moderate pain in chronic and transformed into a tension headache.
tensity; 4) bilateral location; 5) lack of increased pain The diagnosis can be difficult and requires applica
during exercise; 6) absence of nausea and vomiting; tion of neuroimaging techniques (CT, MRI) to exclude
7) separately photophobia and phonophobia can occur; symptomatic headache.
8) exclusion of secondary headache. It is necessary to The treatment requires stopping the abuse of an
seek the cause of the headache - psychological stress, algesics, treatment of withdrawal syndrome (NSAIDs,
anxiety, depression, somatoform disorder, temporoman anxiolytics, antiemetics, water-saline infusions, etc.),
dibular dysfunction, chronic abuse of analgesics, mus application of antidepressants, beta blockers, NSAIDs.
cle strain and fatigue, work in awkward posture, etc.
The examination can reveal stereotypical posture with
raised shoulders and head bent forward, deleted tooth 18.6. SECONDARY HEADACHES
enamel due to bruxism, reinforced physiological tremor,
soreness and tightness of the frontal, temporal, occipi Sensitive to pain are the following cranial struc
tal, paravertibral and masticatory muscles. tures: skin, subcutaneous tissues, muscles, extracrani
I he differential diagnosis is made with migraine al arteries, the periosteum of the skull, the tissues of
without aura, headache in vascular diseases, increased the eyes, ears, nose, sinuses, intracranial venous sinus,
intracranial pressure (brain tumors and cerebral pseu dura, especially at the base, proximal portions of the
dotumor, chronic subdural hematoma, chronic venous intracranial arteries, the optic, oculomotor, trigemi
thrombosis), and chronic sphenoidal sinusitis. The alco nal, glossopharyngeal, vagal nerve and the first three
hol suppresses tension headaches and worsens migraine. cervical nerves. Brain parenchyma, ependima, choroi
I he treatment of episodic tension-type headache is dal plexus, a greater portion of the leptomeninges are
conducted with NSAIDs and analgesics (Paracetamol, painless. Various processes that affect these structures
Acetysal, Metamizole, and Ibuprofen), muscle relax- (traumas, vascular, inflammatory, neoplasms, etc.) can
ants (Tetrazepam, Tizanidine, Baclofen). The misuse cause secondary, symptomatic headache for the diagno
of combination preparations containing caffeine, co sis of which are required additional examinations.
19. Degenerative disorders o f the nervous system / 139
mentia), schizophrenia. tive (1-3 years). In the earliest stage is observed the so-
Most common dementias are Alzheimer’s disease called mild cognitive impairment (MCI) - memory dis
(60-70%), vascular (15-20%) and mixed dementia (10- orders that are more pronounced than expected for the
15%). Following are the disease with diffuse Lewy bod age and educational level, but they do not impair dai
ies (10-15%), frontotemporal dementia (5-10%), and the ly functioning. The objective examinations with Mini
sub-cortical degenerative dementias (5%). The dementia Mental State Examination (MMSE) shows 24-27 points
in alcoholism is relatively common. About 10% of the (normal ranges 28-30 points), as the deficit is manifested
dementias are potentially reversible upon removal of the in the verbal episodic memory (new information) and
underlying cause. with slight anomie (difficulty in naming) but the other
cognitive functions (speech, praxis, gnosis) are intact. In
19.1.1. ALZHEIMER’S DISEASE the next stage in addition to the short-term memory, the
long-term memory is also affected with the clinical pic
Alzheimer’s disease (AD) and the triad of patholog ture of severe memory deficits and disorientation. In the
ical brain changes were described in 51-year-old patient early stages MMSE reveals mild (19-23 points.) to mod
by Alois Alzheimer in 1907. The onset of AD is more erate cognitive deficit (12-18 points). In the later stages
frequently after the age of 65 (senile dementia, AD with the cognitive deficit is severe (less than 12 points). Be
late onset) and more rarely before 65 years (presenile sides the memory functions, a progressive worsening in
dementia, early onset AD). AD affects 2-8% of the pop other cognitive functions is detected also: speech (with
ulation of 65-80 years and 20-30% - 80-95 years. Risk the development of aphasia, alexia, agraphia), percep
factors for AD are advanced age, positive family histo tion and recognition (agnosia), motor skills (apraxia),
ry, female gender, low education levels, spent cranial and executive functions. The professional, social and
trauma, smoking, Down’s syndrome in first-degree rel daily functioning of the patients is impaired with need
atives. The use of NSAIDs and estrogens in menopause for external care of feeding, dressing and toilet. Psychi
reduces the risk. atric symptoms such as depression, insomnia, paranoia,
The pathomorphological changes are most pro psychotic behavior, delirium, delusions, hallucinations,
nounced in hippocampus, entorhinal cortex in the tem sexual disorders are manifested, and in more advanced
poral lobe and cholinergic basal nucleus of Meynert. stages - weight loss, neurological symptoms such as in
In these regions, and then more diffusely in neocortex creased muscle tone, bradykinesia, myoclonus, difficult
appear extracellular amyloid deposits (amyloid plaques) walking, and epileptic seizures are observed. Finally,
and intracellular neurofibrillary degenerations with the patients are bedridden, incontinent, and unable to
subsequent loss of neurons and synapses and brain atro communicate. The average duration of AD is 8-10 years.
phy. The amyloid plaques are composed of A0 amyloid, The diagnosis.is based on a certain clinical criteria:
proteoglycans, Apolipoprotein-e4 and other proteins. progressive deterioration of the cognitive functions in
Ap amyloid protein is composed of 39-42 amino acids, absence of other diseases leading to dementia (for ex
which is derived by proteolysis (of P- and y-secretases) ample, hypothyroidism, and cerebral-vascular disease).
of the transmembrane amyloid precursor protein (APP). Specific changes in the routine laboratory tests are ab
Ap amyloid is deposited in the cerebral arterioles and sent, but they are necessary for exclusion of symptomat
lead to the development of amyloid angiopathy. The ic dementia. The examination of the cerebrospinal fluid
neurofibrillary degenerations in the neuronal cytoplasm detects increased tau-protein levels and reduced A042
are composed of pairs of helical filaments, containing peptide levels. EEG reveals generalized slow wave ac
abnormally phosphorylated tau protein. This protein tivity. The neuropsychological examination (Alzheim
normally is involved in the formation and stabilization er’s disease Assessment Scale, ADAS) is the most im
of the neuronal microtubules. Due to the degeneration portant for early diagnosis and progression follow-up
of the basal nucleus of Meynert, the activity of choline test. CT and MRI demonstrate enlargement of the later
acetyltransferase and acetylcholine levels in the cere al ventricles and diffuse brain atrophy, especially of the
bral cortex, particularly in hippocampus and associa temporal lobe. The volumetric studies detect selective
tive cortex is reduced. The decrease of norepinephrine atrophy of the hippocampus and entorhinal cortex. The
is due to the degeneration of locus ceruleus in the brain functional imaging techniques - SPECT, PET and fMRI
stem. In recent years, genetic defects are found in the reveal hypoperfusion and hypometabolism in the pos
APP gene on chromosome 21, more than 50 mutations terior temporal-parietal cortex. PET with a radioligand
in the Presenilin-1 gene in chromosome 14, and in the is used to visualize the extent of amyloid deposition in
Presenilin-2 gene on chromosome 1 in the early auto the brain.
somal-dominant AD. In Caucasians is established as The differential diagnosis of AD includes other de
sociation of sporadic and familial late AD with Apo- generative and symptomatic dementias.
lipoprotein-£4 allele encoded on chromosome 19. The The treatm ent is performed with cholinesterase in
family AD represents about 5% of the cases with AD hibitors (Tacrin, Donepezil, Rivastigmine, Galantam-
and usually starts at an earlier age. ine), NMDA-antagonists (Memantine), a-Tokopherole
The clinical picture is characterized by three stages (vitamin E), and Ginco biloba. The effectiveness of
- amnesic (1-3 years), demented (1-7 years) and vegeta beta- and gamma-secretase inhibitors that block the
19. Degenerative disorders o f the nervous system / 141
formation of amyloid protein and of the monoclonal palsy, supranuclear gaze palsy, extrapyramidal rigidi
antibodies against the amyloid protein is still under re ty, gait ataxia and dementia and established atrophy of
search. the midbrain and pons with neuronal degeneration and
neurofibrillary changes. PSP is a sporadic neurodegen-
19.1.2. Frontotemporal dementia erative disease referred to the taupathies. The disease
starts in 6-7 decade and progresses more rapidly than
The onset of the frontotemporal dementia (FTD) is Parkinson’s disease. It constitutes 4-10% of the cases
at an earlier age than in Alzheimer’s disease - between with Parkinsonism. It is characterized pathomorpho-
45 and 65 years of age and affects both sexes equally. logical by accumulation of neurofibrillary degeneration
It is subdivided to sporadic and familial forms. In fa positive for tau and negative for amyloid and a-synucle-
milial cases with autosomal-dominant inheritance are in. The degenerative changes affect the midbrain, basal
identified mutations in the tau-gene on chromosome 17. ganglia, brainstem nuclei, and frontal cortex.
In other families are detected mutations in the 3 and 9 The clinical picture is manifested by frequent falls,
chromosome. The degenerative changes affect most the vertical gaze paralysis, symmetrical rigidity, bradykin-
anterior frontal and temporal lobe. The atrophy can be esia and progressive dementia. The gaze disturbances
asymmetric. Gliosis and neuronal loss is detected mi are supranuclear, as the reflex ocular movements are
croscopically. The neurons and glial cells contain cy preserved. The symmetrical muscle rigidity in the axial
toplasmic inclusions composed of pathological tau-pro- extensor muscles with hyperextension of the head is a
tein. Similar tau-aggregates can be found in progressive typical observation. The falls, mainly backward are due
supranuclear palsy (PSP) and corticobasal degeneration to the axial rigidity, gaze paresis in downward direc
(CBD). In P ick’s disease, which is also characterized by tion and postural instability. The dementia is similar to
a progressive atrophy of the anterior frontal and tempo those in FTD - apathy, frontal executive dysfunction,
ral lobe, are observed intracellular inclusions (Pick bod delayed thought processes, impaired verbal fluency, and
ies), containing tau-protein. Currently, Pick’s disease is difficulties in carrying out successive operations. Py
regarded as a variant of the FTD. ramidal signs and pseudobulbar palsy with emotional
The clinical picture of FTD is markedly heteroge incontinence, dysphagia and dysarthria, rarely cerebel
neous. The major symptoms are as follows: 1) behavioral lar events can be observed.
disorders (disinhibition with hypersexuality, hyperactiv The diagnosis is difficult in the absence at the be
ity, impulsiveness, aggressiveness, bulimia with obesity, ginning of a vertical gaze paresis. MRI reveals atrophy
compulsiveness, euphoria or apathy, neglect of personal of the midbrain - colliculi superiores, PET detects -
hygiene), 2) impairment of executive functions (planning, frontal and striatal hypometabolism. Anti-Parkinson’s
judgment) and 3) disturbances of the speech (echolalia, medications are less effective due to the deficiency of
perseveration to mutism). In predominantly left hemi dopamine and dopamine receptors.
sphere damage is observed primary progressive aphasia. PSP must be differentiated from Parkinson’s disease
In more severe involvement of the left frontal lobe de in the later stages of which can develop dementia and
velops non-fluent aphasia with impaired verbal expres postural instability.
sion, which progresses to mutism. In left temporal lobe
involvement develops fluent aphasia with impaired com 19.1.4. Corticobasal degeneration (CBD)
prehension and naming (semantic dementia). In predom
inantly right hemisphere damage disinhibition and anti CBD belongs to the group of sporadic taupathies. It
social behavior occur, while the memory, language and is characterized by asymmetric involvement of the pa
visual-spatial functions are relatively preserved. Apart rietal and frontal cortex and basal ganglia. The disease
from the typical frontal syndromes (apathetic-abulic and onset is after the age of 60 years. The combination of
Moria) also frontal ataxia, grasping reflex and pseudob motor, cognitive and perceptual disorders is typical. The
ulbar syndrome are observed. Combined manifestations motor disorders consist of a combination of akinetic-rig
of FTD, PSP, CBD, akinetic-rigid Parkinsonism and mo id asymmetric Parkinsonism, not responding to lcvodopa
toneuron symptoms are also possible. treatment, and focal dystonia, starting asymmetrically in
The diagnosis is confirmed by detection of fron one hand. Postural and action tremor in combination with
tal-temporal atrophy, which can be asymmetric, thin action myoclonus are gradually added. The perceptual
ning of corpus callosum and enlargement ot the lateral disturbances occur as cortical sensory disorders w ith im
ventricles. possible recognition of the objects by palpation (stereoag
Treatment. The behavioral disorders respond well nosia). The syndrome of the “foreign hand’’ (alien limb),
to serotonin-modifying antidepressants and atypical in which limb is not recognized as own is very typical.
Kinetic apraxia with inability to perform fine movements
anxiolytics.
with the fingers develops. Fronto-subcortical dementia
19.1.3. Progressive supranuclear palsy with impairment of gestures gradually develops. MRT
establishes asymmetric atrophy of gyrus frontalis supe
Steele, Richardson and Olszhewski (1964) described rior and gyrus parietalis superior. PET reveals hypome
the findings at autopsy of patients with pseudobulbar tabolism in the mentioned areas.
142 / Clinical Neurology
19.1.5. Disease with diffuse Lewy bodies cerebellar type of the disease and of hyperintensity of
putamen and globus pallidus in the striato-nigral type
The bodies of Lewy are intraneuronal cytoplasmic by MRI.
inclusions containing a-synuclein and ubiquitin. They
were described in 1912 by Lewy. They are typical find
ings in Parkinson’s disease and are identified mainly 19.2. PARK IN SO N’S DISEASE
in substantia nigra and other brainstem nuclei. In 1980
were described patients with predominantly diffuse Parkinson’s disease (PD) is described by the Eng
cortical location of the Lewy bodies. This finding leads lish GP James Parkinson in 1817. The mean age of onset
to differentiation of two separate diseases - dementia is 55 years. The disease affects 1% of the population
with Lewi bodies and Parkinsonism. The dementia with over the age of 55. The incidence increases with age.
Lewi bodies is characterized by the following triad - The morbidity is 20/100 000 and at the age of 70 years
fluctuations in the cognitive deficits, Parkinsonism, and reaches 120/100 000. The sporadic or idiopathic PD rep
hallucinations. The fluctuations in the memory impair resents 75-80% of cases with Parkinsonism, the famil
ment, attention and alertness are accompanied by ep ial PD - 5%, and the remaining cases are of secondary,
isodic delirium and visual hallucinations that initially symptomatic Parkinsonism. When the onset is before
appear in the admistration of levodopa and neuroleptics. the age 20 years the disease is termed juvenile Parkin
The disturbances of attention, frontal-subortical skills, sonism, between 20-40 years of age - early onset Par
verbal fluency and visual-spatial orientation are typi kinsonism. The risk factors for PD are Caucasian race,
cal findings. Parkinson’s syndrome is characterized by positive family history, male gender (male: female = 3:
bradykinesia, rigidity, postural disorders, slight tremor 2), cranial-cerebral traumas, exposure to pesticides, and
and poor response to levodopa. drinking of well-water. Factors that reduce the risk are
coffee consumption, smoking, NSAIDs, and estrogen
19.1.6. Multisystem atrophy (MSA) replacement therapy in menopause.
The etiology and pathogenesis of PD is unclear. It
MSA is a sporadic disease from the group of syn- is assumed that PD results of genetic predisposition and
ucleinopathies, characterized by parkinsonism, cere influence of exogenous toxic factors with age-related
bellar, pyramidal and autonomous disorders. Intracyto- penetrance. It is known that V* of sufferers have a family
plasmic inclusions positive for a-synuclein and ubiqui history of PD and the risk of inheritance is higher when
tin are observed pathomorphological in the neurons and the father is deseased. The first described form is those
glia of the basal ganglia, brainstem nuclei, cerebellum, of the autosomal-dominant PD with PARK1 mutations
intermediolateral nucleus and the anterior horns of the in the gene for a-synuclein in the fourth chromosome.
spinal cord. The first symptoms are observed around the The second described form is the autosomal-recessive
age of 50 years and have a progressive course within form with PARK2 mutations in the gene for parkin pro
6-9 years. Completely developed clinical picture is ob tein in the sixth chromosome. Currently, more than 15
served in 1/3 of the patients, while in the rest predomi genetically determined forms of PD are described. They
nate two subtypes of the disease: striato-nigral degen are with a clarified biochemical defect that character
eration with leading akinetic-rigid Parkinsonism (80%) izes the earlier onset. The mutation in LRRK2 gene,
and olivo-ponto-cerebellar atrophy with main symptom which is found in both familial and sporadic cases of
cerebellar ataxia (20%). Parkinson’s syndrome occurs PD are of special interest. The discovery that the chem
with akinesia, rigidity, postural instability, and rarely ical substance MPTP (l-methyl-4-phenyl-l,2,3,6-tet-
postural tremor. The falls are not as frequent as in pro rahydropyridine) and the pesticide rotenone can in
gressive supranuclear palsy. The heavily bented forward duce parkinsonism gave the reason for discussion the
posture with touching the chin to the chest is typical. role of exogenous toxins or endogenous formed similar
In the beginning when the neostriatum is less affect substances in genetically susceptible individuals. It is
ed patients respond to the treatment with levodopa. The assumed that the oxidative stress plays a main role in
cerebellar disorders predominate in approximately 20% the neuronal degeneration due to the increased forma
of the patients - gait ataxia, instability and falls, inten tion of oxygen radicals and/or reduced antioxidant pro
tion tremor and muscular hypotension. The autonomous tection (genetically determined or acquired), which is
disorders occur early in the course of disease in 40% of supported by the decrease of the reduced glutathione
the patients, and later are present in all patients - ortho in substantia nigra. The reduced activity of complex I
static hypotension, impotence, incontinence or urinary in mitochondria confirms the importance of the mito
retention, constipation, inspiratory stridor, impaired chondrial dysfunction in apoptosis and neuronal degen
thermoregulation. The combination of autonomous dis eration. The accumulation of abnormal proteins in the
orders and Parkinsonism was referred to as syndrome cytoplasm results of defective function of the UPS (the
of Shy-Drager in the past. The pyramidal signs are less ubiquitin-proteosome system).
pronounced - hiperreflexia, pseudobulbar palsy, spastic The most characteristic pathomorphological finding
ity in the legs. The diagnosis is confirmed by the detec is the fading of pars compacta of substantia nigra due
tion of atrophy of the cerebellum, pons and oliva in the to degeneration of the dopaminergic melanin-contain
19. Degenerative disorders of the nervous system / 143
ing neurons. The eosinophilic intracytoplasmic inclu The gait is slow, with small steps; particularly difficult
sions (Lewy bodies), containing a-synuclein are typical are the initiation of the movement and the changing of
finding. Similar degenerative changes are found in the direction. In more advanced cases, the bradykinesia and
noradrenergic nucleus locus coeruleus, the serotonin- rigidity hamper the everyday activities - washing, feed
ergic nucleus raphe, the cholinergic nucleus basalis of ing, and dressing. The pulsion phenomena are very typ
Meynert, nucleus dorsalis n.vagi, frontal cortex, etc. As ical - if the patient is pushed forward he speeds up his
a result of degeneration of the nigrostrial neurons oc steps in attempt to keep balances and hardly stops with
curs denervation of striatum, which is more pronounced risk of falling (anteropulsio). Especially disabling in
in putamen compared to nucleus caudatus. Clinical more advanced cases, is the phenomenon of “freezing”
symptoms are observed in degeneration of 60% of the - acute onset inability to move and freezing in place.
nigrostrial neurons. Biochemical consequence of this Sometimes kinesia paradoxa is observed - sudden re
is a reduction in the level of dopamine in striatum and covery of the normal mobility, but for a short period of
impaired balance between the excitatory direct pathway time. The postural instability is characteristic of the lat
(starting from D1 receptors) and the inhibitory indirect er stages and is cause of falls. This is due to the loss of
pathway (starting from D2 receptors), increased gluta- the postural reflexes, akinesia, rigidity, flexion posture.
matergic activity of nucleus subthalamicus, increased Besides the described motor symptoms, non-motor
GABA-ergic activity of the basal nuclei (internal pal symptoms are observed in approximately 50% of the pa
lidum and s.nigra pars reticularis), increased suppres tients - depression, cognitive disorders, and disorders of
sion of the ventral-lateral thalamic nuclei and reduced the autonomic nervous system. Depression can be the
activity of the excitatory motor talamocortical pathway first manifestation of the disease and is found in more
to the cortical motor neurons - fig.5.4; fig. 5.5. Due to the than 50% of patients. Typical finding early in the course
reduction of dopamine in striatum the balance with ace of the disease is the reduction of smell (hyposmia). Cog
tylcholine is also impaired with increased cholinergic nitive disorders with developing of dementia occur in
activity. The balance between the excitatory glutama- the later stages in 20-30% of the patients. The autono
tergic mediation and the inhibitory dopaminergic medi mous disorders are usually mild - orthostatic hypoten
ation is also impaired with glutamatergic hyperactivity, sion, constipation, sphincter disorders, seborrhea, and
which leads to excitotoxicity and neurodegeneration. impaired thermoregulation.
Clinical picture. The disease onset is slowly and The diagnosis is based on the clinical symptoms
imperceptibly with nonspecific symptoms such as pain - presence of two of the three cardinal manifestations
and feeling of “tightness” in the shoulders and neck (tremor, rigidity and bradykinesia) and asymmetric in
and mild clumsiness in the affected limbs. The major volvement of the limbs. The diagnosis is more difficult
symptoms of the disease are gradually exhibited - trem in patients with tremor onset. The tremor detected by
or, rigidity and bradykinesia. The asymmetric onset EMG is with alternating pattern in agonists and antag
most often from the arm and then involving the epon onists and has a frequency of 4 to 6 Hz. The therapeu
ymous leg and later the limbs on the opposite side is tic response of the symptoms to levodopa (DOPA test)
typical finding. The tremor at rest (static tremor) is in confirms the diagnosis. Neuroimaging studies (CT and
itial symptom in 70% of patients. It begins unilateral MRI) are necessary to exclude symptomatic Parkin
ly; most often involving fingers of the one hand or lips, sonism (vascular, in normal pressure hydrocephalus,
tongue and jaw, but the head is speared. The tremor is Parkinson-plus syndromes). The diagnosis is verified
static, at rest; it disappears in movement and increases by DaTSCAN - SPECT, which visualizes the decreased
in stress. The frequency of tremor is 4-6 Hz. The muscle density of the dopamine transporters expressed on the
rigidity results of the increased muscle tone. It is estab nigrostriar terminals in striatum. In symmetrical start,
lished as a constant resistance throughout the passive the absence of tremor, the presence of pyramid, cere
movement or as a stepwise discontinuation of the re bellar, autonomous and other symptoms, early develop
sistance (a cogwheel phenomenon). The muscle rigidity ment of dementia and postural instability and negative
is more pronounced in the flexor muscle groups, which DOPA-test give rise to suspicion in the diagnosis and
determines the typical hunched posture of the patients. require differential diagnosis with secondary, sympto
Bradykinesia occurs with slowdown of all movements, matic Parkinsonism and Parkinsonism in other extrapy-
hypokinesia - with extreme poverty of the movements. ramidal disorders (atypical Parkinsonism).
There is delayed in the start and execution of the move Differential diagnosis. The differential diagnosis in
ments, they are reduced in amplitude and speed and this patients with isolated tremor onset is performed with
affects the external appearance of the patient. The tace essential tremor. It is more common than PD, has pos
is expressionless, mask-like, with rarely blinking; the tural character, synchronous pattern of EMG and affects
gaze is stared (hypomimia). The speech is quiet, monot more symmetrical upper limbs. The essential tremor can
onous (hypophonia). The writing becomes difficult; the affect head and larynx also and has a higher frequency
handwriting is with small letters, especially at the end 8-10 Hz. Typically decreases in alcohol consumption
of the line (micrographia). The spontaneous movements and propranolol intake. The variants of essential trem
are reduced. Typical is the reduction and disappearance or with a lower frequency and alternating pattern on
of the normal synkinesis in upper limbs while walking. EMG are more difficult for differentiation. A number of
144 / Clinical Neurology
increases in tension, in precise volitional activity and 100 000. HC is due to a genetic defect in the short arm
decreases in alcohol consumption. The disease pro of chromosome 4 —the number of trinucleotide CAG
gresses slowly, over time its amplitude increases, and (cytosine-adenine-guanine) repeats in the HD gene is
its frequency decreases. The tremor disturbs daily ac increased above 40 at a normal range less than 25. The
tivities such as writing, eating, and professional activ gene encodes a cytoplasmic neuronal protein called
ity. EMG identifies tremor with frequency 6-8 Hz and huntingtin. Neuronal loss, gliosis and atrophy of stri
synchronous (type A) or alternating (type B) muscle ac atum most pronounced in the head of nucleus caudatus
tivity. The synchronous type A tremor responds well to and cerebral cortex are observed. The degeneration of
the therapy with Propranolol, and the alternating type GABA-ergic medium spiny neurons that have inhibitory
B - with Primidone. In disabling character of the tremor projections to the neurons to external pallidum, blocks
are undertaken thalamotomia or deep brain stimulation the indirect pathway and excites the direct pathway. The
of the ventral intermediate nucleus of thalamus. cause of the hyperkinesis is the reduced inhibitory ac
Differential diagnosis is made with Parkinson’s tivity of the striatum.
tremor, as well as the following types of tremor: Clinical picture. The choreic hyperkinesias are
• Enhanced physiological tremor. It is observed fast, rhythmic, transitory involuntary movements that
in neurotic conditions, anxiety, distress, thyrotoxico resemble voluntary movements. At the beginning they
sis, withdrawal, intake of beta-adrenergic agonists, are limited in the distal parts of the extremities and face
methylxanthines, corticosteroids, etc. It is due to in and resemble nervousness and mugging. Over time they
creased sensitivity of the peripheral beta 2 receptors, increase in amplitude and involve the proximal and axi
which causes oscillations in the stretch reflex of the al muscles and disturb the gait (“dancing gait”), speech,
muscle. EMG registers low-amplitude discharges of swallowing, and even breathing. The muscle tone in
synchronous activity with a frequency of 8-12 Hz. the extremities is reduced. The choreic movements are
• Primary orthostatic tremor. It is presented in sometimes combined with slower dystonic movements
getting up and standing only and reduces and disap (choreoathetosis). Over time the hypotonic-hyperkinetic
pears in walking and sitting. It has high frequency (14- syndrome is replaced by hypertonic-hypokinetic (Par
18 Hz) and amplitude and an alternating character. Fam kinson’s) syndrome, postural disorders are developing,
ily history is not present; the tremor does not respond progressive behavioral and cognitive disorders (subcor
to alcohol consumption and beta blockers intake. The tical dementia) are observed. The onset of HC is some
tremor responds well to treatment with Clonazepam, times with mental and cognitive disorders. The disease
Phenobarbital and Primidone. is subdivided into two forms: with childhood onset (un
• Cerebellar tremor. It is due to involvement of der the age of 15-20 years) - a variant of Westphal and
the afferent and efferent cerebellar pathways and occurs with late onset - 35-40 years. The course of the child
on the ipsilateral side. It is accompanied by other cer hood onset form is w ith a clinical picture of Parkinson’s
ebellar symptoms - muscle hypotonia, dysmetria, adi- syndrome, seizures, mental retardation, ataxia and fast
adohokinesia, asinergia. The tremor is postural, action er progression. For the adult onset form are characteris
and intention, with synchronous nature and frequency tic the chorea and progressive dementia.
of 3-8 Hz. The diagnosis is easy if choreic hyperkinesias, pro
• Rubral (mesencephalic) tremor. It results of gressive dementia and family history are present. CT
damage to the upper brainstem - nucleus ruber, upper and MRI reveal atrophy of nucleus caudatus, increased
cerebellar peduncle, mesencephalic tegmentum, there bi-caudat index and cortical atrophy. The diagnosis is
by damaging the rubral-olivar-cerebellaral-rubral path more difficult in the absence of family history and debut
way and rubral-tegmental-spinal fibers. The tremor with mental symptoms. In these cases genetic testing is
is observed at rest, increases in maintaining posture, recommended.
movement and especially in intention. It is irregular, Differential diagnosis is performed with other
with a frequency of 2-5 Hz, affects the more proximal neurodegenerative diseases (neuroacanthocytosis, den-
parts of the limbs and body and has disabling character. to-rubral-palidal-luisian atrophy), benign hereditary
• Psychogenic tremor. It has variable character chorea, senile chorea, tardive oral-buccal-lingual dys
and represents a combination of static, postural, kinetic kinesias (treatment with neuroleptics), chorea grav
and intention tremor, with varying frequency and am idarum, chorea in systemic lupus erythematodes and
plitude depending on the psychic condition. antiphospholipid syndrome. The syndrome of hemicho-
rea / hemiballismus most often is due to a stroke with
involvement of the contralateral subthalamic nucleus.
19.4. HUNTINGTON’S CHOREA Choreic syndromes are described in hyperthyroidism,
hypo- or hyperglycemia, hypo- or hypernatraemia, hy
Huntington’s chorea (HC) was described in 1872 by pocalcemia, hypomagnesemia, polycythemia, medica
G. Huntington as an autosomal-dominant disease with tion (amphetamine, dopaminergic, anticholinergic, an-
complete penetrance and onset between 35-45 years ol tihistaminic, tricyclic antidepressants, etc.).
age. The disease occurs earlier if the pathological gene The treatment of hyperkinesias and psychotic man
is inherited from the father. The morbidity is 5-10 / ifestations is performed with neuroleptics (Haloperidol,
146 / Clinical Neurology
onset). The pathologic studies reveal degeneration of the the familial cases with autosomal- dominant transmis
pyramidal tract, while in the complicated forms in ad sion is detected mutation of Cu-Zn SOD (superoxide
dition to the pyramidal damage is observed involvement dismutase) in 21q22.11 chromosome. Later are found
of funiculus gracilis, spinocerebellar tracts, etc. other genetic defects with autosomal-dominant (1, 2, 9,
Clinical picture. Slowly and progressively devel 17, 18, 20 chromosomes) and autosomal-recessive (2, 5,
ops central lower paraparesis with spastically increased 12, 15 chromosomes) transmission. The gene products
muscle tone and muscle weakness in lower limbs, lead are also identified (alsin, senataxin, tau, dynactin, hex
ing to difficulties in walking in the following decades. osaminidase, kinesin, etc.).
Very characteristic of the disease is the pronounced The pathogenesis is disputable - genetic predispo
spasticity and only slightly manifested muscle weak sition and influence of unclear exogenous factors that
ness in lower limbs. The neurological examination re trigger the pathogenetic mechanisms are supposed - ox
veals pyramidal signs in the 4 limbs - pathological hy- idative stress due to deficiency of SOD, mitochondrial
perreflexia and clonus in preservation for a long period dysfunction, glutamate excitotoxicity, apoptosis, deficit
of the abdominal reflexes. In later stages spasticity and of trophic and growth factors, etc. The pathomorpholog-
weakness in upper limbs can be observed. In the clinical ical studies establish a selective degeneration of the py
forms with isolated involvement of the pyramidal tracts ramidal cells in layer 5 of the motor cortex, the pyramid
sensory and sphincter disorders are not detected. Two pathway, motor nuclei of the bulbar nerves and anterior
major clinical forms are distinguished - hereditary para horn motor neurons of the spinal cord. In the degenerat
paresis without associated symptoms (pure form) and ed motor neurons accumulate lipofuscin, ubiquitin and
hereditary paraparesis - plus syndromes (complicated neurofilaments.
form): spinocerebellar (spinal and cerebellar ataxia, in Clinical picture. ALS begins most often between 50
tention tremor), extrapyramidal (Parkinsonism, tremor, and 70 years of age, in 10% - before the age of 40 and in
dystonia, athetosis), ocular (optic atrophy, macular de 5% before the age of 30 years. The initial manifestations
generation, gaze paresis), hypoacusis, mental retarda can be either from the upper or from the lower motor
tion, dementia, neuropathy, amyotrophy, epilepsy, etc. neuron, but soon both motor neurons are involved. The
The autosomal-recessive syndrome of Sjogren-Larsson, most common initial symptom is muscle weakness in
starting in childhood include spastic paraparesis, men upper limbs, at the beginning asymmetrical. More rare
tal retardation and ichthyosis. ly, the initial symptom is weakness in lower extremities
The diagnosis is based on the clinical and family or both upper and lower extremities. More often the dis
data. MRT sometimes detects changes in the white cere tal muscle groups of the arms, particularly extensors are
bral matter and thinning of corpus callosum. The motor affected. Other initial symptoms include tightness and
and somatosensory evoked potentials reveal particu spasticity, mainly in the lower limbs or muscle twitch
lar changes. Differential diagnosis is performed with ing in the limbs or trunk. Along with increasing of the
multiple sclerosis, cervical myelopathy, malformation muscle weakness develops atrophy of the affected mus
of Arnold-Chiari, primary lateral sclerosis (a form of cles, especially pronounced in the small muscles of the
amyotrophic lateral sclerosis), spinal tumors, tropical wrist - the hand resembles a “monkey’s paw”. In 30%
spastic paraparesis, and chronic myelitis. of cases, ALS starts with bulbar palsy - difficulties in
Treatment with muscle relaxants such as Baclofene, chewing, swallowing (dysphagia) and talking (dyspho-
Tizanidine, and Dantrolene is administered. nia, and dysarthria). The feeding is disturbed, by mouth
ran saliva, fluids and food come out through the nose or
fall into the trachea. With the progression and generali
19.11. AMYOTROPHIC LATERAL zation of the disease the intercostal muscles and the dia
SCLEROSIS phragm are involved with development of dyspnea. The
death occurs due to aspiration and respiratory failure.
Amyotrophic lateral sclerosis (ALS, sclerosis later The different stages of the disease are dominated by
alis amyotrophica) is a progressive neurodegenerative manifestations of the upper or lower motor neuron, but
disease, involving the central and peripheral motor neu in most patients symptoms from both motor neurons
ron (Motor neuron disease), fatal for 3-5 years. The fre persist simultaneously. The neurological examination
quency of the disease is 3-5 / 100 000 and the annual establishes a combination of peripheral atrophic paresis
incidence - 1-3 / 100 000. Men are affected about twice (mostly in the upper limbs) and existence of pyramidal
as often. In some geographic regions (mainly Marianas signs (spasticity, exaggerated reflexes, and pathological
Guam, New Guinea and the Key Peninsula in Japan) the reflexes) mainly in the lower limbs. The combination
morbidity is much higher probably due to the consump of muscle atrophy and exaggerated reflexes is very typ
tion of food neurotoxins. ical. The tongue is atrophic with reduced mobility and
The etiology of ALS is not known - suspected risk presence of fasciculation, the soft palate is with reduced
factors are exposure to toxins (lead, mercury) smoking, or absent mobility, the pharyngeal reflexes are absent
foods high in glutamate, physical work, sports and oth (bulbar palsy). The mandibular reflex is brisk, and the
er injuries. ALS is primarily a sporadic disease, only refexes of oral automatism become positive (pseudob
5-10% of the cases are with family history. In 20% of ulbar palsy).
152 / Clinical Neurology
Clinical forms o f ALS: 1) Primary Lateral Sclerosis lopathy, syringomyelia, cervical intramedullary tumor,
- affects only the central motor neuron; 2) Primary mus carpal or ulnar canal syndrome, spinal vascular malfor
cular atrophy - clinical manifestations of lower motor mations.
neuron only; 3) Progressive bulbar palsy - selective The therapy is ineffective. The glutamate antago
ly affects the motor nuclei of the bulbar nerves, of the nists Riluzole and Gabapentin slow the progression with
trigeminal and facial nerve. The onset is between 30 and just a few months. Therapeutic attempts are being made
50 years of age with rapidly progressive dysphagia, dys- with neurotrophic factors, stem cells, etc. In respiratory
phonia, and dysarthria due to paralysis of the tongue, disorders are used artificial respiratory ventilation with
soft palate, pharynx, larynx, jaw and facial muscles. positive pressure, while in dysphagia is placed nasogas
The tongue is atrophic with fasciculation, motionless; tric tube or a percutaneous gastrostomy is performed.
3) Family form - the beginning is at an earlier age, af The prognosis is poor; the survival rate is 3-5 years.
fects both sexes equally, often beginning in the lower
limbs with involvement of the lower motor neuron; 4)
ALS plus - a combination of ALS with dementia and / 19.12. SPINAL M USC ULAR ATROPHY
or parkinsonism. This form is typical of Guam ALS; 5)
Syndrome o f Hirayama - muscular atrophy in one limb, The spinal muscular atrophy (SMA) is an inherited
which progresses for 1-2 years and then stations. disease with mainly autosomal-recessive mode of in
The diagnosis is based on the simultaneous presence heritance and degeneration of the motor neurons in the
of amyotrophic paresis and pyramidal symptoms, of bul anterior horns of the spinal cord and motor nuclei in
bar and pseudobulbar palsy, and the absence of sensory, the brainstem and development of muscle weakness and
oculomotor and sphincter disorders. The clinical diagno atrophy. The incidence is about 1/20 000.
sis is definite in affection of the upper and lower motor In 1995 was discovered defect in the so-called Sur
neuron in three regions (head, upper limbs, lower limbs vival Motor Neuron (SMN) gene on the long arm of the
and body). The diagnosis is possible in damage of both fifth chromosome (5qll-ql3). This gene has two copies
neurons in two regions; probable (damage of both neu - telomeric (SMN1) and centromeric (SMN2). The mu
rons in only one region or in central motor neuron dam tations in SMN1 gene are lethal in the absence of SMN2
age in two regions at least) and suspected (damage of the gene. The phenotypic variations of the disease depend
peripheral motor neuron in at least two regions). With on the number of copies of SMN2. In type I SMA is
EMG is demonstrated generalized anterior horn damage, present 1-2 copies of SMN2, in type II SMA - the copies
including in clinically unaffected muscle groups - fascic are 3, while in type III SMA - the copies are 3-4. The
ulation and fibrillation potentials, reduced motor action carriers of SMA have 50% of SMN-protein. In SMA
potentials that are polyphase with increased amplitude type I is present a deletion of SMN1, which can be de
and duration (“giant potentials”). The sensory fibers of tected by prenatal diagnosis. Therapeutic attempts with
peripheral nerves are preserved. With MRT is estab genetic therapy by increasing the expression of SMN2
lished thinning of the pyramid pathway and hyperintense are performed.
lesions along its course. The motor evoked potentials Several clinical forms of SMA depending on the age
are changed. In CSF there is a slight increase in protein. of onset and clinical course are described.
Some increase in creatine kinase due to the neurogenic 1. Infantile type o f Werdnig-Hoffman (SMA type I).
muscular atrophy can be observed. The frequency is 1/10 000 births. The disease is present
The differential diagnosis depends on the clinical at birth or occurs in the first 6 months. It may be suspect
form. ed even during the pregnancy by the weak movements
• In bulbar palsy: tumors of the brainstem and of the fetus. Newborns are floppy, subdued, with limited
the cranial base, chronic basal meningitis, myasthenia movements, in palpation the muscles are hypotonic and
gravis, syringobulbia and progressive bulbar palsy. atrophic, reflexes are absent, and the fasciculation are
• In pseudobulbar palsy: lacunar strokes in cere visible. The affected children cannot sit independently
bral vascular disease. and bend forward as a “pocket knife” or take a “frog
• In the syndrome of primary lateral sclerosis: posture”. In most cases bulbar symptoms are present
cervical myelopathy, multiple sclerosis, Strumpell’s dis with difficulty in sucking and swallowing. Breathing
ease, cervical compression, tropical spastic paraparesis, is also impaired due to involvement of the intercostal
vitamin B12 deficiency. muscles and diaphragm. Death occurs to the end of the
• In affection only of the peripheral motor neu second year.
ron: benign fasciculations and cramps, spinal muscular 2. Intermediate type o f Dubovitz (SMA type II). The
atrophy, multifocal motor neuropathy with block in con frequency of SMA type II is 1/10 000-1 / 20 000. The
ducting (with antibodies to Gm 1 ganglioside and MAG), onset is up to 2 years of age. The disease progresses
motor polyneuropathy, CIDP, lymphoproliferative dis more slowly and patients survive up to 20-30 years of
ease with paraneoplastic neuronopathy, motor polyneu age. They develop generalized muscle weakness, atro
ropathy, post-polio syndrome. phy and hypotonia, particularly in the proximal portions
• In spinal onset with manifestations from the of the lower limbs. The patients are able to sit, but the
cervical intumescense: spondilogenic cervical mye walking is impossible. Complications with contractures
19. Degenerative disorders o f the nervous system / 153
of the lower extremities, skoliosis and difficult breath quency is 1/50 000, men are affected only, and the dis
ing are observed. ease is transmitted by mothers. The onset is at the age
3. Juvenile type Wohlfahrt-Kugelberg-Welander of 30-40 years. The muscle atrophy affects the shoulder
(SMA type III). The frequency of SMA type III is 1/24 and pelvic girdle, hips and lower legs, trunk, facial and
000. This benign SMA affects the proximal muscles of bulbar muscles with dysphagia, dysarthria, dysphonia.
the extremities and resembles myopathy (pseudomyo- Fasciculations are present. In 50% of the patients gyne
pathic form of SMA). The onset of the disease is after comastia is observed.
2 years of age, usually between 5 and 15 years of age. 7. SMA in children and adolescents, in which the ge
The clinical manifestation is with muscle weakness and netic defect is not in chromosome 5qll:
atrophy of the proximal lower limbs muscles and the a) Progressive bulbar paralysis o f Fazio-Londe;
muscles of pelvic girdle. Sometimes pseudohypertro b) Skapuloperoneal SMA;
phy of m. triceps surae is observed. The patients have c) Facial-skapulo-peroneal SMA;
particular difficulties in climbing stairs and getting up d) SMA with hexosaminidase deficiency.
from a squatting position. The gait is myopathic (“wad Most of these types of SMA are with autosomal-re
dle”). The disease progresses very slowly. The paraspi- cessive mode of inheritance.
nal muscles are involved with development of lumbar The diagnosis of SMA is confirmed by the estab
hyperlordosis and scapulae alatae. The upper limbs and lishment of anterior horn damage by EMG - fascicula
bulbar muscles are rarely affected. The reflexes are di tions, fibrillation, reduced number of motor units. The
minished or absent. In approximately half of the patients motor action potentials have increased amplitude and
fasciculations are observed. duration (“giant potentials”). Data for myogenic or pe
4. SMA with late-onset (SMA type IV). The disease ripheral nerve damage are absent. The muscle biopsy re
is transmitted in an autosomal-dominant or autoso veals neurogenic grouped atrophy of the muscle fibers.
mal-recessive manner. The onset is after the age of 15. In some cases, creatine kinase is increased.
The disease progresses slowly and the clinical picture The differential diagnosis is performed depending
resembles SMA of Kugelberg-Welander. on the clinical form:
5. Distalform o f SMA (SMA of Aran-Duchenne). The • in the infantile form - with congenital myas
disease is autosomal-dominant with later onset in adult thenia, infantile form of myotonic dystrophy, congenital
hood or autosomal-recessive with an early start. The myopathies, glycogenosis, and some forms of cerebral
motor neurons of the cervical intumescence are most palsy.
affected. The muscle weakness and atrophy involve the • in the juvenile form - with progressive mus
distal parts of the upper limbs and progress proximally. cular atrophy type Duchenne, type Becker, type Em-
The progression is very slow and lower limbs are affect ery-Dreufus.
ed years later. • in the distal SMA - with neuropathy of Char-
6. X-recessive bulbospinal muscle atrophy with late cot-Marie-Tooth, distal myopathies, and ALS variants
onset (Kennedy’s disease). The disease is due to a ex with more benign course
pansion of trinucleotide repeats (CAG) in the gene for • in the bulbospinal form - ALS, and SMA type
the androgen receptor in Xqll-12 chromosome. The fre Kugelberg-Welander.
154 / Clinical Neurology
These disorders are observed in 3-5% of the new of the cases is in connection with a malformation of Ar
borns. They result of genetic defects and teratogenic nold-Chiary type I - protrusion of the cerebellar tonsils
factors during ontogenesis: radiation, infections (rubel into the cervical canal. Other reasons include syndrome
la, cytomegalovirus), diabetes mellitus and other meta of Dandy-Walker (agenesia of vermis with dilatation of
bolic diseases and intoxications of mother (alcoholism, the IV ventricle), basilar impression, atlanto-occipital
anticonvulsants), vitamin insufficiency states, etc. The fusion, obstructive lesions in the cranio-cervical area,
type of malformation depends on the embryonic period intramedullary spinal tumors, traumatic myelopathy
of impact of the pathogenic factor. and hematomyelia, spinal arachnoiditis and pachimen-
I. Defects in the formation of the neural tube (be ingitis. The majority of cases are idiopathic with un
tween the 25 and 29 gestational day). They are subdi clear etiology. Familial cases are described.
vided to: spinal (Spina bifida oculta, Spina bifida aper- The pathologic examination establishes longitudi
ta, Meningocele, Meningomyelocele, Diastemomyelia, nal cavities like a pencil (syrinx), the walls of which
Diplomyelia, Agenesia sacralis) and cranial (Encepha- consist of astrocyte glia and ependim cells. These cavi
locele, syndrome of Arnold-Chiary, Anencephalia). ties can be communicating or non-communicating with
II. Defects in the division and differentiation of the the central spinal canal. The enlargement of the central
neural tube (between the 20 and 30 gestational day): canal is called hydromyelia. The posterior horns in the
Holoprosencephalia, syndrome of Klipple-Feil. cervical-thoracic area are mostly affected; the lumbar
III. Disorders of the neural migration (between the intumescence is rarely involved. The cystic lesion can
50 and 115 gestational day) and gyrus formation (be involve medulla oblongata and pons (syringobulbia).
tween the 155 and 180 gestational day): Porencephalia, With the increase in the volume of the cystic formation
Schizencephalia, Lissencephalia, Macrogyria, Micro the posterior and lateral funiculi and the anterior horns
gyria, Agenesia of corpus callosum, Agenesis of cere become compressed. Developmental abnormalities are
bellum, syndrome of Dandy-Walker. often observed - malformation of Arnold-Chiary, plati-
IV. Disorders of the post-migration period: Hydro- basia, spinal dysraphism.
cephalia, Hydranencephalia, Microcephalia. The pathogenesis is unclear. According to the hydro-
V. Cranial malformations: Craniostenosis, Impressio dynamic theory the developmental anomalies of cranial
basilaris. basis disturb the CSF flow between spinal and intracra
VI. Residual syndromes - cerebral palsy (CP) nial subarachnoid space by ejection of CSF (during the
CP includes various residual syndromes that occur systole phase of the cardiac cycle) from the fourth ven
during the first year of life. They result from prenatal tricle to the central spinal canal and from the spinal sub
brain damage (diseases of the mother), perinatal brain arachnoid space to the spinal cord via the perivascular
damage (asphyxia and other injuries during birth) and spaces. According to another hypothesis in dysraphism
from damage in the neonatal period. Important risk fac or embryonic disturbances in the connection of the pos
tors are immaturity of the fetus and Rh and ABO in terior edge of the medullary plate undifferentiated glia
compatibility. The following clinical forms of cerebral is growing, followed by formation of cysts. The role of
palsy are differentiated: hemiplegic, diplegic, and dou the birth trauma is under discussion.
ble hemiplegic, extrapyramidal, cerebellar and mixed. Clinical picture. Syringomyelia can debut in the
VII. Phacomatoses: Neurofibromatosis (disease childhood and can cause scoliosis. More often the neu
of Recklinghausen), Tuberous sclerosis, syndrome of rological symptoms are manifested in adulthood. The
Sturge-Weber, Ataxia-telangiectasia (disease of Lou- disturbances of the superficial sensation for pain and
is-Bar). temperature in preservation of the deep sensation (fine
touch, joint-position and vibration sense) are typical
finding - syringomyelitic dissociation o f the sensation.
20.1. SYRINGOM YELIA The sensory abnormalities in the cervical- thoracic form
affect asymmetrically the upper trunk and the arm (as
Syringomyelia is a rare disease (incidence 8/100 a tunic) and later become bilateral. The painless burns
000), which is characterized by forming a longitudinal and frost damages are very typical. Upon an increase in
tubular cavities in the spinal cord and brainstem, with volume of the cavity and compression of the adjacent
typical dissociated sensory disorder. The etiology in 2/3 posterior and lateral funiculi and anterior horns the fol
20. Developmental disorders of the nervous system. Residual syndromes. Phacomatoses / 155
lowing symptoms are developing: pyramidal symptoms The diagnosis is confirmed by MRT - visualization
in the lower limbs (hyperreflexia, spastic paraparesis), of cavity w ith a density of CSF along the length of the
loss of fine touch and proprioceptive sensation, segmen spinal cord in cervical-thoracic and more rarely in lum
tal atrophy of the muscles of the wrist and hand (bra bosacral region or in brainstem. Various malformations
chial amyotrophies), degenerative arthropathy, painless can be established - of Arnold-Chiary, of Dandy-Walk
ulcers, and Horner’s syndrome (due to involvement er, platibasia, spinal dysraphism. The CSF protein can
centrum ciliospinale in C8-Thl segments). In lumbosa be increased in subarachnoid block of the CSF flow.
cral syringomyelia the clinical symptoms affect lower Differential diagnosis is necessary with intramed
limbs in asymmetrical way. Sphincter disorders are also ullary tumor, hematomyelia, spondilogenic cervical
observed. In syringobulbia due to involvement of the myelopathy, cerebral dysraphism, platibasia, basilar im
lateral tegmentum of brainstem initially facial syringo- pression, etc.
myelitic dissociation in the dermatomes of Zolder is de The treatmept consists in drainage of the cavity or
tected, and then the nuclei of bulbar nerves are affected placing a special catheter that guides the cerebrospinal
with dysphonia, dysarthria, dysphagia, atrophy of the fluid in the pleural or peritoneal cavity. In case of mal
tongue, facial palsy, nystagmus, and ataxia. The senso formation of Arnold-Chiary surgical treatment is rec
ry and bulbar disorders should be taken into account in ommended.
dental procedures.
156 / Clinical Neurology
21.1. MYASTHENIA GRAVIS (lymphoepitelioma), which can invade the pleura and
pericardium and to metastasize in the thoracic cavity.
Myasthenia gravis pseudoparalytica (MG) is an au In 50% of patients lymphocytic infiltrates in the mus
toimmune disease which is due to impaired transmis cles are identified. The immunocytochemical studies
sion of nerve impulses in the neuromuscular synapses reveal deposition of IgG and complement 2 and 9 on the
as a result of reduction of the acetylcholine receptors. It postsynaptic membrane with changes in the structure -
is characterized by fatigue and muscle weakness. a decrease in the number of the postsynaptic folds and
The autoimmune pathogenesis of the disease is evi enlargement of the synaptic space.
denced by: 1) the detection of antibodies to acetylcholine Epidemiology. The incidence of the disease is 4-8 /
receptors in the serum and the postsynaptic membrane; 100 000, and in some countries reaches 14/100 000. The
2) the creation of experimental myasthenia in animals onset of MG is most often between 20 and 30 years of
by injection of acetylcholine receptors; 3) transfer of the age. The disease is 2-3 times more common in women.
disease via immunoglobulins from a patient; 4) trans A second peak of the disease is detected after the age of
placental transfer of antibodies with development of ne 50 years, when men suffer more often.
onatal myasthenia; 5) the therapeutic effect of plasma Clinical picture. MG is characterized by the follow
exchange, thymectomy and immunosuppressants. The ing features - diurnal variations in severity of the symp
antibodies to acetylcholine receptors are blocking the toms, worsening after physical exertion and selective
binding sites of the receptors for acetylcholine, while affection of particular muscle groups. The disease starts
other antibodies induce complement-mediated damage slowly and imperceptibly, sometimes sharply - after in
of the postsynaptic membrane with decreased density termittent illness, surgery, stress, and certain medica
of the receptors (fig. 21.1). As a result, the amplitude of tions. The first clinical signs are most often from the oc
postsynaptic action potential is reduced and the neuro ular muscles - unilateral or bilateral asymmetric partial
muscular transmission is disturbed. The cause of auto fall of the eyelid (ptosis) and double vision (diplopia).
immune process is unclear. Abnormalities in the thy The ptosis and diplopia are differently expressed during
mus gland are observed, the number of the lymphoid the day. The neurological examination reveals in addi
follicles with germinal centers is increased (hyperplasia tion some weakness of m. orbicularis oculi. The absence
of the thymus), and in 15% of the patients tumors of the of pupil disorders and the preserved pupil reaction are
thymus - thymomas are detected. typical for MG. The diagnosis of isolated ocular myas
It is believed that the autoimmune process occurs thenia is assumed, when 2-3 year after the disease onset
in the abnormal thymus, in which antigen-presenting generalization is missing. This form of the disease is
cells (expressing HLA-class II molecules), T-helper observed in 15-20% of the cases.
cells (recognizing antigen), B cells
(synthesizing antibodies) and the Presynaptic nerve
terminal
so-called “myoid” cells (expressing
acetylcholine receptors and other
muscle antigens) are present. The Lambert-Eaton
microbial or viral infections via the syndrome
mechanism of molecular mimicry or Voltage-gated / (antibody)
calcium channels ^ Synaptic
superantigens can activate the po e y Aminoglicoside
c vesicle
tentially autoreactive CD4 + T cells. Ca2+- antibiotics
J ^ n ° ° 0 ’ 0 0 O n Act,ve
A genetic predisposition associat .Q o.0o .° O ° ° ° zonep Botulism (toxin)
ed with HLA-haplotypes (B8, DR3, Synaptic cleft Myasthenia gravis
and DQB1) is established. In patients i'H?•••'choli ni\
antibody)
with myasthenia and their relatives
are more often observed other auto
immune diseases - thyroiditis, sys
temic lupus erythematosus, rheuma J’vnr —*- — —«
• 'V — - - k
toid arthritis, pernicious anemia, etc. —^ P r e c e p to r s ------ fv . . A
—;- | .*•• v•*Л,V*.* ••
Pathology. In 70% of the pa ..... 5 v. . 1 •
tients is observed hyperplasia of the __________________ ---- : — -------iz *
thymus (thymit) - an increase of the
lymphoid follicles with germinal
centers. In 15% is found thymoma Fig.21.1. Sites of involvement in disorders of neuromuscular transmission (Aminoff)
21. Muscular diseases / 157
The initial symptoms are more rarely of bulbar mus cular atrophies, sensory disturbances and changes in
cles involvement with impaired swallowing, speech and the reflexes are typical findings. With anticholinesterase
chewing. The speech becomes nasal (dysphonia) and tests (Edrophonium, Prostigmine) is reported clinical
not well articulated (dysarthria). Due to the weakness improvement that can by detected by EMG. In repeti
of the muscles of pharynx and soft palate the swallow tive electrical stimulation of the nerve at a frequency of
ing becomes difficult (dysphagia), fluids are coming out 3-4 Hz is reported decremental muscle response - my
through the nose, and there is a risk of aspiration. The asthenic reaction. In the serum are tested antibodies to
chewing becomes difficult, the jaw droops down. The acetylcholine receptor, which are at high titer in 90% of
facial muscles are involved with characteristic expres cases with a generalized myasthenia and in 60% with
sionless look on the face. The examination reveals di ocular myasthenia. Antibodies to MuSK, titin and other
minished pharyngeal reflexes, reduced mobility of the muscle antigens are also tested. CT or MRI of the ante
soft palate during phonation, and disturbed swallowing. rior mediastinum is necessary for establishing of hyper
The prognosis is unfavorable in cases with bulbar symp plasia of the thymus or thymoma.
toms disease onset. Differential diagnosis of the ocular myasthenia is
As the disease progresses the cervical muscles are performed with syndrome of Horner, thyrotoxic oph
affected, the head droops down, the proximal limb mus thalmopathy, diabetic and other cranial neuropathies,
cles are involved with fatigue in climbing up stairs and mitochondrial ocular myopathy, multiple sclerosis, or
getting up from a squatting position. In involvement of retroorbital parasellar tumor, cerebral aneurysm, etc. In
the respiratory muscles (intercostal muscles and dia bulbar myasthenia is required exclusion of amyotroph
phragm) respiratory failure is observed. The rapid de ic lateral sclerosis, neoplasms of the brainstem and the
terioration of the weakness in oropharyngeal and res cranial base, brainstem stroke, cranial polyneuritis, etc.
piratory muscles, which is life-threatening, is called Generalized myasthenia should be differentiated from
myasthenic crisis. The reasons for myasthenic crisis are botulism, myasthenic syndrome of Eaton-Lambert,
inadequate anticholinesterase therapy, intercurrent in congenital myasthenia, periodic paralysis, polymyosi
fections, physical exertion, stress, surgery, childbirth, tis, myopathy, acute inflammatory demyelinating pol
the implementation of certain drugs (muscle relax- yneuropathy, diphtheritic polyneuritis, intoxication by
ants, benzodiazepines, anesthetics, narcotic analgesics, pesticides, insecticides, snake bites, etc.
aminoglycoside antibiotic, anti-arrhythmic drugs, high The treatm ent is symptomatic with anticholinest
doses of glucocorticosteroids), etc. The myasthenic cri erase agents and pathogenic with immunosuppressive
sis is characterized by generalized muscle weakness, therapy and thymectomy. Anticholinesterase agents
bulbar and respiratory disorders. An overdose of the block reversibly acetylcholinesterase and reduce the
anticholinesterase preparations can lead to choliner hydrolysis of acetylcholine. The most commonly used
gic crisis, which is due to depolarization block in the are Pyridostigmine (Kalymin, Mestinon - tabl. 60 mg,
neuromuscular synapse. The myasthenic symptoms are amp. 1 and 5 mg), Prostigmine (Syntostigmin amp. 0,5
worsening, muscarinic side effects occur (sweating, mg). Thymectomy is recommended in established pa
salivation, lacrimation, nausea, vomiting, diarrhea, fre thology of the thymus, after puberty and up to 60 years
quent urination, bronchospasm, bradycardia, myalgia, of age. Immunosuppressive treatment is conducted with
myosis). In additional worsening of the crisis nicotine corticosteroids (Prednisolon in ascending or descend
type cholinergic manifestations occur - muscle twitch ing schemes), Azathioprine, cyclosporin A, Cyclophos
ing, convulsions, CNS manifestations. The differential phamide, newer Mycophenolate mofetil, Tacrolimus,
diagnosis between myasthenic and cholinergic crisis is Rituximab. In risk of myasthenic crisis is administered
sometimes difficult because in most cases the crises are plasmapheresis or high doses of immunoglobulins. The
of mixed character. In this connection, in respiratory prognosis before the introduction of immunosuppres
failure discontinuation of the anticholinesterase therapy sive treatment was poor with lethal outcome in 33%.
and respiratory resuscitation is recommended. In myasthenia are absolutely or relatively contrain
Neonatal myasthenia. The disorder is due to a pla dicated the following drugs: aminoglycoside antibiotics
cental transfer of antibodies and is observed in 10-20% (gentamicin, kanamycin, neomycin, streptomycin, etc.),
of children born to mothers with myasthenia. The symp antiarrhythmics, beta blockers, calcium antagonists,
toms occur in 3-72 hours after birth with muscle weak phenytoin, chloroquine, penicillamine, potassium-los
ness and hypotonia, difficulties in sucking and swallow ing diuretics, lithium, chlorpromazine, benzodiaze
ing, and breathing disorders. The recovery occurs in 3 pines, gabapentin, tubocurarine, succinylcholine and
-6 weeks. The therapeutic efficacy of anticholinesterase other neuromuscular blockers.
agents is good.
The diagnosis of MG is based on the typical clini 21.1.1. Other diseases with impaired
cal features of muscle weakness and fatigue with daily
neuromuscular transmission
fluctuations in the external eye muscles, oropharyngeal,
1. Botulism. The disease is due to the exotoxins of
proximal skeletal muscles and respiratory muscles. The
Clostridium botulinum, an anaerobic microorganism that
pathological muscle fatigue is proved by application ot
develops in poorly canned food. Botulinum toxin is ab
certain tests for muscular exertion. The absence of mus
158 / Clinical Neurology
sorbed from the gastrointestinal tract and in hematogenic cases are detected on the occasion of prolonged apnea
way reaches the cholinergic receptors (nicotinic and mus in surgery. The diagnosis is confirmed by EMG - low
carinic), binds to the presynaptic membrane and block the amplitude of the motor action potentials in response to
exocytosis of acetylcholine (fig.21.1). The clinical symp low frequency stimulation and increase in the amplitude
toms occur 12-36 hours after ingestion of infected food - in response to high frequency (20-50 Hz) stimulation
nausea, vomiting, abdominal pain, blurred vision, ptosis, - facilitation. The muscle weakness responds good to
reduced ocular movements, mydriasis and absent pupil treatment with 4-Aminopyridine, 3,4-Diaminopyridine,
reflexes, facial diplegia, dysphonia, dysphagia. The pro Guanidine, and the autoimmune process - to corticos
gressive muscle weakness descends and involes the cer teroids, immunoglobulins, plasma exchange and remov
vical muscles and muscles of the limbs and trunk, includ al of the tumor.
ing the respiratory muscles with development of acute
respiratory failure. The wound botulism in infection of
wounds and infantile botulism are more rarely observed. 21.2. MYOPATHIES
EMG detects the facilitating reaction. Differential diag
nosis is performed with the syndrome of Guillain-Barre, Depending on the etiology myopathies are divid
porphyry polyneuritis, brainstem stroke, encephalitis, ed into: hereditary (muscular dystrophy), congenital,
and poliomyelitis. The treatment includes gastric lavage, metabolic, immune and inflammatory, endocrine, and
respiratory resuscitation, injection of trivalent antitoxin, drug-induced.
guanidine, and 3,4-diaminopyridine. Progressive muscular dystrophy type Duchenne
2. Congenital Myasthenia. The disease is not auto This is a X-recessive myopathy with an incidence of
immune, but hereditary. It is inherited most often in an 1: 3500 and affects most often the boys. About 70% of
autosomal-recessive manner, rarely in autosomal-dom mutations are inherited from the mother and the remain
inant way or is sporadic. It may be due to: 1) a presyn ing 30% are new mutations. Xp21 gene is responsible
aptic defect (abnormal re-synthesis and packaging of for the production of dystrophin membrane protein that
acetylcholine, decrease of the synaptic vesicles, reduced maintains the integrity of muscle cell in contraction and
quantum separation); 2) synaptic defect (deficiency of relaxation. In the absence of dystrophin occurs intracel
acetylcholinesterase) and most commonly 3) postsynap- lular influx of calcium and necrosis of the muscle cell.
tic defect (the slow channel syndrome, syndrome of the The clinical picture develops before the age of 5 years
fast channel and other defects in the kinetics of acetyl - difficulty in learning to crawl, myopathic gait, diffi
choline receptors, a reduction in the number of recep culties climbing up stairs and getting up from a squat
tors, etc.). More than 15 forms of congenital myasthenia ting position. The pelvic, hip and paraspinal muscles
have been described. The best studied is familial infan become atrophic with development hyperlordosis, sko-
tile myasthenia which is transmitted in autosomal-re liosis, and contractures. The respiratory capacity is re
cessive mode, with genetic locus on chromosome 17B duced, cardiomyopathy develops. The pseudohypertro
for epsilon subunit of the acetylcholine receptor. Con phy of m. triceps surae is typical. Later in the course of
genital Myasthenia is usually manifested in the new disease the muscles of the shoulder girdle are affected.
born or later in the childhood. The symptoms of the The independent gait becomes impossible before the age
external eye muscles or proximal muscles (limb-girdle of 13 years and death occurs up to 25 years of respira
form) are dominating in the clinical picture. Sometimes tory and heart failure. The diagnosis is confirmed by:
facial dysmorphism is observed. The symptoms usually 1) EMG data for myogenic damage (polyphase poten
do not progress. Antibodies in maternal serum and in tials with low amplitude); 2) strong increase in creatine
the serum of the diseased child are not present. phosphokinase (CPK) more than 10 times; 3) the almost
3. Syndrome of Lambert-Eaton. This is an au complete absence of dystrophin in the immunohisto-
toimmune disease which is due to antibodies to volt chemical examination of muscle biopsy; 4) DNA analy
age-gated calcium channels (VGCC) of the nerve ter sis for prenatal diagnosis. Treatment with Prednisolone
minals (fig.21.1). As a result the presynaptic release of 0,75 mg / kg slows the disease progression.
acetylcholine in the nicotinic receptors in muscle and in
muscarinic receptors of the parasympathetic ganglia is Progressive muscular dystrophy type Becker
disturbed. The antibodies targeting the VGCC of the ne The inheritance is X-recessive. The genetic defect in
oplastic cells in small cell lung cancer and neuroendo Xp21 results in abnormal or decreased dystrophin. The
crine tumors are cross-reacting with the VGCC on the onset is after the age of 7 years with muscle weakness
presynaptic membrane. The neurological symptoms oc and hypotrophy of the hip muscles with pseudohyper
cur before the diagnosis of the primary tumor. The dis trophy of the lower leg muscles. The weakness in m.
ease begins with muscle weakness in the proximal parts quadriceps femoris and muscle cramps can be the only
of extremities and is combined with areflexia, muscular manifestation. The muscles of the shoulder girdle are
atrophy and autonomous symptoms - dry mouth, consti involved later. The progression is slower, contractures
pation, lack of pupil reaction to light, etcs. Ocular and and cardiomyopathy develop less frequently and the in
bulbar symptoms are not usually observed. The patients dependent gait is preserved until the age of 16 years.
are abnormally sensitive to muscle relaxants and some CPK is increased more than 5 times.
21. Muscular diseases / 159
M uscular dystrophy type Emery-Dreifuss affected asymmetrically. Sometimes associated symp
The disease results of mutations in two different toms are observed (sensoryneural deafness, conductive
genes - for emerin on Xq28 chromosome (X-recessive cardiac disorders, retinal angiopathy). The diagnosis is
mode of inheritance) and lamin on lq21 (inherited in performed by DNA analysis.
an autosomal-dominant manner). These mutations lead
to a deficit of nuclear proteins emerin and lamin that Distal myopathies
form a common complex and no matter which of the two The distal myopathies are clinically and genetical
proteins is reduced the clinical phenotype is the same. ly heterogeneous group of disorders. Until now more
Disease onset is around the age of 5 years with simul than 15 different forms have been described. In most
taneous development of contractures and humeral-per of them are detected the so-called rimmed vacuoles.
oneal muscle weakness and atrophy. The involvement The distal myopathy Wellander type is transmitted in
of the conduction system of the heart is typical and at an autosomal-dominant manner (2pl3 chromosome).
the age of 30 often the need of pacemaker arises. CPK It starts after the age of 40 years with initial involving
is slightly increased. The deficit of emerin or lamin is of the fingers extensors. The evolution is slow. Later in
detected by immunohistochemical studies of muscle, the course of the disease are involved the extensors of
leukocytes and skin. A precise diagnosis is obtained by the feet. The distal myopathy Markesbery-Griggs-Udd
DNA analysis. type is also an autosomal-dominant disorder (muta
tions in titin gene on chromosome 2q31). The onset is
Limb-girdle muscular dystrophies (LGMD) after the age of 40 years in heterozygotes and in child
LGMD are genetically and clinically heterogeneous hood in homozygotes. The initial symptoms are mus
group of disorders. Up to now six different forms have cle weakness and atrophy of the anterior tibial muscles
been described with autosomal-dominant inheritance and development of steppage gait. The distal myopathy
(with genetic defects of muscle proteins myofilin, lamin Miyoshi type is an autosomal-recessive disorder with
A / C, caveolin-3) and 11 forms with autosomal-reces genetic defect of dysferlin on 2pl3 chromosome. The
sive inheritance (with deficit in calpain-3, dysferlin, a, onset is between 15 and 30 years old age in the posterior
P, y and 5-sarcoglycan, telethonin, titin). The recessive group of muscles of the lower leg. Identical mutations
forms are more common, their onset is earlier and the can cause phenotypically different myopathies - distal,
course is severe. In LGMD type 2A (kalpainopathy) limb-girdle 2B, distal with involvement of the anteri
have been described over 100 mutations in the calpain or tibial muscle. Unlike the other distal myopathies in
3 gene (15ql5) with an enzyme deficiency of the mus this myopathy the level of CPK is highly increased up to
cle-specific calcium-activated neutral protease calpain 100 times above normal. The distal myopathy Nonaka
3. The first symptoms occur between 2 and 45 years of type is an autosomal- recessive (9pl2-pll) disease with
age and severity vary from asymptomatic elevations a characteristic feature - presence of inclusion bodies. It
of CPK to severe muscle weakness of hip and later of starts at the age of 20-30 years with involvement of the
the shoulder girdle and trunk muscles. LGMD type 2C feet extensors. Other distal myopathies have also been
(gamma-sarkoglicanopaty) is due to mutations in the described: oculopharingeal myopathy; distal myopathy
gamma-sarcoglycan gene on chromosome 13q 12. Gam- with respiratory failure; distal myopathy with dementia
ma-sarkoglican is a transmembrane protein involved and Paget’s disease.
in the construction of dystrophin-associated glycopro
tein complex responsible for the stabilization of sar- Myotonic muscular dystrophy (Steinert’s disease)
colemma. The onset is between 3 and 10 years of age This is the most common muscular dystrophy (13.5
with weakness and atrophy of the pelvic and shoulder / 100,000 births). It is transmitted in autosomal- domi
muscles with initial involvement of flexors and a rel nant mode with variability in the age of onset and clini
ative preservation of extensors. Alpha-, beta- and del- cal course. The anticipation is typical (the symptoms in
ta-sarkoglicanopathies are more rare diseases. the subsequent generations occur earlier and are more
severe). The disease is due to the amplification of the
Facioscapulohumeral muscular dystrophy (FSHD) trinucleotide (CTG) repeats in the DMPK gene on chro
FSHD is the third-frequency muscular dystrophy af mosome 19ql3.2. As a larger is the number of repeats
ter DMD and myotonic dystrophy. The inheritance is the onset of the disease is earlier and the course is more
autosomal-dominant. The disease results of deletions severe. The distribution of muscle atrophies is typical
on chromosome 4q35. The age of onset for larger dele - orbicular muscles (ptosis), pharyngeal and laryngeal
tions is in the younger age. Congenital forms have also (dysphagia, dysphonia), chewing (sunken cheeks and
been described. The first manifestations vary from 0 temples), m.sternocleidomastoideus (“swan’s neck”)
to 50 years. The facial muscles are initially atlected, and distal muscles of the extremities. The involvement
followed by the periscapular muscles (scapulae allatae of the respiratory muscles leads to daytime sleepiness
are typical) at a relative preservation of mm. deltoid- and hypersensitivity to general anesthesia. The muscle
ei. The extensors of the foot and pelvic girdle muscles atrophy is combined with myotonic phenomenon (diffi
with impaired gait are affected later in the course of cult relaxation of the muscle after a strong contraction),
the disease. The muscles of the shoulder girdle can be which is detected on EMG. Cataracts, early balding,
160 / Clinical Neurology
cardiomyopathy, infertility, cognitive deficits are other es. The treatment of myotonia is performed with mem
typical symptoms. Besides the classical form are also brane stabilizers - Procainamid, Chinin, Phenytoin,
described: 1) congenital form with generalized muscle and Mexiletine. The application of depolarizing muscle
weakness and hypotonia; 2) early form and 3) proximal relaxants (Suxamethonium) is prohibited due to risk of
myotonic myopathy (3q21 chromosome). Myotonia re life-threatening muscle spasms.
sponds well to the treatment with Procainamid, Chin-
ine, Mexiletine, Phenytoin, Carbamazepin and other Periodic muscle paralyses
anticonvulsants. Periodic paralysis (PP) is a genetically heterogene
ous group of disorders - the hypokalemic PP is due to a
Myotonic disorders genetic defect of calcium channel ( lq31-32), and hyper
Myotonia is characterized by delayed muscle relax kalemic - of sodium channel (17q23). The normokalemic
ation after muscle contraction. The myotonic diseases PP is a variant of the hyperkalemic PP.
are divided into dystrophic (myotonic dystrophy) and The hypokalemic PP is transmitted in an autoso
non-dystrophic. The non-dystrophic myotonic diseas mal-dominant mode with reduced penetrance in wom
es and periodic paralysis are attributed to the group of en. The most severe cases begin in childhood and the
channelopathies. Channelopathies are due to a genetic milder cases - in the third decade. The episodes of mus
defect of voltage-dependent ion channels in the mus cle weakness occur at night or in the morning and are
cle membrane. In defects of the chlorine channel (7q32 triggered by carbohydrate-rich foods or rest after phys
chromosome) the membrane conductance for chloride ical exertion. The muscle weakness ranges from mild in
anions is reduced with prolonged depolarization and re limited muscle groups to generalized, sometimes with
petitive generation of muscle action potentials. Myoto involvement of the oropharyngeal and respiratory mus
nia of Thomsen and the generalized myotonia of Becker cles. During the attack the level of the serum potassium
are attributed to the chlorine channelopathies, while is reduced, the muscle reflexes are absent, ECG registers
paramiotoniya congenita and potassium-dependent my flat T waves. The attack lasts for several hours to three
otonia - to the sodium channelopathies. days. The intervals between the attacks vary from sev
Myotonia congenita (Thomsen’s disease) is an au eral days to several months. In 30% of the patients prox
tosomal-dominant disease with a complete penetrance. imal myopathy develops over the years. The diagnosis
The first symptoms occur after birth or in early child can be confirmed with provocative tests with glucose
hood. The myotonia is most pronounced after rest and or insulin. The treatment is performed with adminis
decreases after exercise. The musculature is well devel tered orally potassium or with potassium infusion. For
oped and the patients have athletic habitus. The muscle prevention - potassium-sparing diuretics (Triamterene,
strength is not reduced and is even increased. Myotonia Spironolactone), Acetazolamide are prescribed
decreases over the years. The hyperkalemic PP is transmitted in an autoso
Generalized myotonia Becker type is more frequent mal-dominant mode with complete penetrance in both
ly observed. It is transmitted in an autosomal-recessive sexes. The first symptoms occur from early childhood
mode. The onset is later - in the first or second decade until the second decade. The disease proceeds with
of life. The myotonia is more pronounced, sometimes milder but more frequent attacks of muscle weakness.
combined with muscle weakness in upper limbs and hy They are provoked by a potassium rich diet, emotional
pertrophy of the muscles in the legs. stress, starvation, exposure to cold. Some patients have
Congenital paramiotoniya (Eulenburg disease) is an symptoms of myotonia or paramiotoniya. The serum
autosomal-dominant disease with complete penetrance. potassium is increased during the attack, ECG records
It is due to genetic defect of sodium channel (17q23). high T-wave. Treatment is performed by intravenous
The first symptoms occur at birth. Myotonia worsens infusion of Glucosa, Insulin, Calcium gluconicum, in
after repeated muscle contractions (paradoxical myoto haled Salbutamol. For prevention is administered Ac
nia) and cold, and can be followed by muscle weakness. etazolamide, thiazide diuretics, diet low in potassium
It is sometimes combined with hyperkalemic periodic and high in carbohydrates.
paralysis (which is also sodium channelopathy). The normokalemic PP is a variant of the hyper
Potassium-dependent myotonia is an autoso kalemic PP.
mal-dominant disease which is due to mutations in the
gene for sodium channels. Two main forms are observed Metabolic myopathies
- fluctuating and permanent. The fluctuating myotonia They are due to disorders of carbohydrate and lipid
is provoked by exercise and potassium, which does not metabolism and mitochondrial dysfunction. The dis
cause muscle weakness as in hyperkalemic periodic pa turbances in the carbohydrate metabolism (muscle gly
ralysis. The permanent myotonia is generalized and is cogenosis) occur with impaired tolerance in physical ex
accompanied by muscle hypertrophy of shoulders and ertion (muscle pain, weakness, cramps, myoglobinuria)
neck. The attacks of severe myotonia of the thoracic or with permanent muscle weakness, including cardio
muscles are life-threatening. myopathy. They result of enzyme defects - of phospho-
The diagnosis of the latent myotonia is based on rylase (disease McArdle), an acid maltase (disease Pom-
the EMG findings, which recordes myotonic discharg pe), etc. The lipid myopathies are most commonly as
21. Muscular diseases / 161
sociated with the metabolism of carnitine, which trans weakness and muscle pain. The proximal muscles,
ports long-chain fatty acids through the mitochondrial sometimes cervical, pharyngeal, esophageal and res
membrane. They are manifested by muscle weakness, piratory muscles are involved. It can be combined with
myalgia and rhabdomyolysis after activities requiring interstitial pulmonary fibrosis, cardiomyopathy, Ray
oxidation of fatty acids (long exercise, exposure to cold, naud’s syndrome, arthralgias, vasculitis and connec
a diet high in fat and low in carbohydrates). tive tissue diseases.
Mitochondrial myopathies. They are due to muta Dermatomyositis is observed in children and
tions in mitochondrial DNA, resulting in disturbances adults, more common in women. Sometimes is asso
of the oxidative phosphorylation in mitochondria and ciated with malignant neoplasms. In addition to the
inclusion in the metabolism of anaerobic mechanisms proximal muscle weakness the purple skin rash and
with increased lactate and pyruvate in the serum. They facial edema are typical.
are manifest in early childhood with the picture of con Inclusion bodies myositis. The disease is observed
genital myopathy syndrome, Kerns-Sayre’s syndrome, in men after the age of 50 years. The distal muscle
progressive external ophthalmoplegia, cardiomyopa are mainly affected, the weakness of the long flexor
thy, and endocrinopathy, MELAS (mitochondrial en of the fingers is often observed. Typical are the intrat-
cephalopathy, lactic acidosis and strokelike episodes), cytoplasmal and intranuclear. There are inflammatory
MERRF (myoclonic epilepsy and ragged red fibers). changes as opposed to the distal myopathies.
The family history of inheritance from the mother is Inflammatory myopathies are observed in toxo
important for the diagnosis. plasmosis, trichinosis, cysticercosis, influenza, Cox-
Congenital muscular dystrophies. They are trans sackie and ECHO- virus, HIV.
mitted in an autosomal-recessive mode and are associ Endocrine myopathies. In hyperthyroidism the
ated with a deficiency of merosin. They are manifest at following complications are observed: 1) chronic thy
birth with muscle weakness and hypotonia, sometimes roid myopathy; 2) thyroid-associated ophthalmopathy;
arthrogryposis with contractures of the joints. The dis 3) thyrotoxic periodic paralysis. In hypofunction is
orders are non-progressive or slowly progressive. observed hypothyroid myopathy. In hyperglycocorti-
Congenital myopathies. They are classified on the cism and in prolonged treatment with corticosteroids
basis of the structural changes in muscle fibers estab proximal myopathy develops. An acute steroid myopa
lished by histochemical methods. Myotubular (centro- thy evoked by treatment with high doses of corticos
nuclear) myopathy is with X-linked, AR or AD inher teroids is described. In adrenocortical insufficiency
itance. Nemalinic myopathy is transmitted in AD, AR is observed generalized muscle weakness. In adrenal
mode or is sporadic. “Central core” congenital myo adenoma with hyperproduction of aldosterone develop
pathy is transmitted in AD or AR mode and is charac muscle weakness or hypokalemic periodic paralysis.
terized by a tendency to malignant hyperthermia dur Toxic and drug-induced myopathies. Muscle ne
ing administration of inhaled anesthetics. crosis with rhabdomyolysis and myoglobinuria can
develop in acute alcohol intoxication. Subacute and
Immune and inflammatory myopathies chronic alcoholic myopathy with proximal muscle
Polymyositis is an autoimmune disease in which weakness and atrophy are described. The drug-induced
muscles are infiltrated with CD8 lymphocytes and in myopathy is caused by a number of agents: fibrates,
serum are detected anti-Jo-1 antibodies. It is observed statins, corticosteroids, colchicine, vincristine, cyclo
after the age of 35 years with symptoms of muscle sporine, zidovudine, certain antibiotics, etc.
PSYCHIATRY
GEORGIONCHEV
22. Introduction in psychiatry / 165
This textbook has the ambition to present psychia not only offer knowledge but also has to create skills and
try to non-psychiatrists in brief and simple way. Need attitudes, i.e. to contribute to interiorization and practi
of basic knowledge and skills in mental health area for cal application of the learnt material.
doctors, dentists, nurses and other health and social care
professionals stems from the essence of clinical job. SCOPE OF MENTAL HEALTH
Each human interaction during treatment or other pro PROBLEMS
cess of helping requires contact with inner experiences
of someone else. Insufficient student curriculum in psychiatry is in
sharp contrast with the scope of mental health prob
lems (Table 1). One in four persons worldwide develop
SUBJECT some mental disorder at some moment in their lifespan,
and 24% of all patients in general out-patient care have
Subject of psychiatry has two aspects. One of them mental disorders. Most common among them are de
encompasses scope, etiology, pathogenesis, clinics, pression, anxiety disorders, and abuse with alcohol and
course, and treatment of mental disorders. The other other substances. Four from the ten leading causes of
one refers to illness behavior and embraces variety of disability in the world as well as 12% of the so-called
problems in every illness: from recognition of symp global burden of diseases, are due to mental disorders.
toms and the meaning attributed to them, to acceptance
of patient’s role and cooperation for treatment. Precisely ETHICAL FRAMEWORK
this second aspect is closely related to practice of med
icine in general of and to the necessity of basic training True understanding of mental health problems is not
in identification and intervention in most common men attainable out of the ethical context of the clinical job.
tal health problems in general medical practice. Individual autonomy and informed consent constitute
the core of bioethics. All other deontological principles
Teaching psychiatry meets also more general needs are superimposed on these basic norms and evolve from
related to practice of health professions, remaining how them. They may be neglected only according to clear
ever at least partially neglected in our medical education rules, e.g. when applying coercion against a patient with
which is predominantly technologically oriented. Such dangerous behaviour. Affiliation with certain diagnos
are the skills for interviewing and for creating thera tic group (e.g., schizophrenia) may not serve as a reason
peutic relation, unconditional respect for patient’s per in itself for deprivation of any rights (like the right of
sonality and recognition of his/her experience, adopting education, for instance) or of any capacities (like the ca
non-authoritarian clinical style and basic ethical norms pacity for informed consent). Such deprivation ground
of healing. In order to fulfill these aims teaching must ed solely on group’s affiliation is at the root of preju-
Lifetime prevalence (whenever in lifespan) in the general population in the world------------------ 25.0%
Ten leading causes of loss of full value years of life due to disability (Y LI)s) Part of the gen
eral disability
in the world
11.9%
Unipolar depression
4.6%
Hearing loss, adult onset
4.5%
Iron-deficiency anaemia
3.3%
Chronic obstructive pulmonaruy disease
3.1%
Alcohol use disorders
3.0%
Osteoarthritis
2.8%
Schizophrenia
2.8%
Falls 2.5%
Bipolar affective disorder
2.1%
A s t h m a _______________ _____________________________________________
166 / Psychiatry
dices against mentally ill people denoted as stigma. The key for understanding psychopathology is rec
Deprivation of any right or capacity is acceptable not in ognition of its inner experience essence, and its quan
principle but only after individual assessment. titative difference from normal human experience.
For this reason, basic characteristic of the good clini
Other basic ethical principles include: unconditional cal worker is his/her specific skill to try to understand
loyalty to the patient - with priority over loyalty to oth someone else’s experience. Mental disorders are pre
ers, confidentiality, self-determination, private space, sented shortly with their manifestations, practical rules
treatment with the least restrictive treatment option, for their recognition and principles of treatment behav
right of competent help in lack of capacity for deci iour. Organization of the material allows quick refer
sion-making, etc. Coercion in psychiatric and forensic ences. A glossary of basic terms is attached at the end.
psychiatry may be thought of as reglamentation of the
exclusions from these principles. This textbook may be used not only by students in medi
cine and dentistry but also by ergotherapists, social workers,
Content nurses, medical assistants, and others in the area of health
In this textbook, contemporary knowledge in psy care. It presents the minimum of clinical facts needed by
chiatry is laconically presented, with emphasis on those health professional to meet mental health problems in prac
aspects that are most relevant to everyday clinical prac tice. However, wealth of ideas and clinical nuances are in
tice. Used terminology is internationally accepted and trinsic to psychiatry. A short list of additional literature is
psychiatry that is taught is “evidence based” rather than attached at the end of the textbook. Phenomena in mental
based on schools of thought. In the chapter for gener life are multi-faceted, and usually illumination of one aspect
al psychopathology, symptoms are presented briefly in leads to need of explanation on another level discovering thus
domains of psychological experience, as well as in syn new knowledge. This textbook would have fulfilled its aim if
dromes, along with basic questions from the interview it stimulates such interest. Knowledge of psyche in general,
for their assessment. no matter in what professional context, makes one’s view of
life much wider and develops abilities for critical thinking,
sustaining insecurity, and tolerance to others’ opinions.
Different understandings of “madness” reflecting or burning on stake, includingly). The first psychiatric
dominant ideology have existed ever since ancient time in-patient facility was opened in Baghdad in the VIII
in all cultures. In 1550 BC, depression was mentiond century, followed shortly by others in Cairo, Valencia,
in an old Egypt papyrus on medical topic. In the Bible, Saragosa, Toledo, Uppsala, Elbing, London, and else
reliable clinical conditions have been described, e.g. in where in Middle East and Western Europe. Ibn Sina (Av
King Saul and Nabuchadnezzar in the Old Testamen. icenna) (XI century) linked psychological experience
Generally, mental deviations have been attributed to ei with physiology and developed a system for detection
ther bodily causes, or to supernatural forces. Such di of emotional fluctuations by assessing the pulse rate. In
vision under various disguise continues for centuries. the Middle Ages, during the bloom of the Arab civiliza
Hippocrates (IV century BC) claimed that mental dis tion, attitude towards mentally ill in the chaliphates was
eases were rooted in physiological anomalies, while Pla more humane and based on rationality than in Europe,
to endorsed divine influence on part of them. In Roman where fears of demons were widespread. The advance
tribunals, a scheme for assessment of mentally ill citi of natural sciences during the Renaissance gave impe
zens on 11 axes was introduced (attributed to Cicerone, I tus to development of ideas about link between men
century BC), encompassing different aspects of somatic tal pathology and the brain. In Eastern Europe at that
helath, family, and course of life. One of the axes, called time, care of the mentally ill was undertaken mainly in
orationes, refers to form and content of talk, which is monasteries. Towards XVII century psychiatry already
analoguous to mental status taking in psychiatry. This emerged as medical discipline, although the term “psy
approach is prototype of modern multiaxial assessment. chiatry” was brought later (in 1808) by Reil.
Different concenpts of mental diseases have deter In 1794 the French clinician Pinel ordered liberating
mined historical variety of methods for their “treat mentally ill from their chains in the Paris hospital Bi-
ment”: from trepanations and venesection to prays and cetre, and later on in the Salpetriere, thus marking the
expelling evil spirits from oneself (by means of torture development of practice of minimal restraint as basis
23. Brief history of psychiatry / 167
for efficient treatment. In XIX century, huge evidence
about long-term follow-up of patients in the large, asy
lum type, psychiatric hospitals started to accumulate
forming traditions of institutional care. Usual treat
ment approach at that time was the so-called moral
therapy, while explaining paradigms reduced psychopa
thology to brain pathology with specific localization, or
to degeneration. Griesinger for the first time among his
contemporaries clearly separated mental disorders as
a separate, not-to-be-reduced-to-other-pathology, class
of diseases.
INTERVIEW: ESSENCE AND RULES cal interview remains so far the most reliable method for
finding out phenomena in psychopathology. Complete
In psychiatry, like in general medicine, correct di clinical assessment however requires balance between
agnosis depends on thorough history taking and on full what patient has said and other sources of information:
clinical assessment. The main tool to achieve this goal observation, information from relatives, other persons,
is the interview. In psychiatry the interview is used not and documentation.
only for history taking but also for clinical assessment,
i.e. it is essential part of the medical examination. Good clinical interview has its rules:
• Structuredness of the environment
Skills necessary for doing good interview are: active • Guiding through interview phases, and con
listening, accounting for non-verbal communication, trolling it
selection of key fragments of information to reach diag • Data gathering from different domains; asking
nostic decision, lack of prejudices and open-mindedness for examples
• O p e n - m i n d e d n e s s , f l e x i b i l i t y ; a d a p t in g th e d i
to signals which may be in contradiction with initial hy
pothesis. In the absence of para-clinical markers, clini r e c t io n o f a s k i n g q u e s t i o n s
168 / Psychiatry
• Transition from open to closed questions you are with low mood?). When dating is difficult, ref
• Attending to content and process erence personal time points are used, e.g. service in the
army, birth of child, etc. Another general rule is that the
STRUCTURE OF INTERVIEW trend of interrogation should follow the main problem, and
only after its clarification it has to shift to signs that may
As far as the circumstances allow for, interview should be connected with the main complaint, e.g. in complaint of
be held in premises with certain comfort and privacy. The low mood, one is asked about sleep, appetite, energy, guilt.
clinician should introduce him/herself, and specify briefly
interview’s framework (aim and consequences). A standard scheme for data gathering is presented below.
Despite the outline, a good interview is characterized also by
Opening phase. It is usually started with open ques flexible switch over topics and adapting questions to problems
tion about the reason for visit, e.g. What is the main prob emerging during its course. The areas for assessment are:
lem that brings you here? or How do you think, why do • Cause and way of referral
they refer you to this office?, and the patient is encouraged • Current episode: duration of problems or com
to talk freely on this. Meanwhile, the clinician attends to plaints (precise dating or poor demarcation in time), in
two main aspects of the narrative: what the patient says, tensity, frequency, impact on functioning (e.g., loss of job
and how it is said. and social contacts, withdrawal in home), time trend
• Past psychiatric episodes and illness: clinical pic
Body of the interview. The main task in this phase is ture, onset (with prodromes or acutely), duration, course
to obtain information about the symptoms, history of the (continuucly or in episodes), treatment, dangerous behav
illness and personal history, along with mental status as iour (aggression, suicide attempts)
sessment. It is impossible to cover such variety of problem • Family history of mental disorders or behavioural
domains (Table 2) without discipline in asking questions abnormalities in relatives
and control over the interview. At the beginning, open • Individual developmet: with emphasis on early
questions (with unlimited number of replies) are more ap development, especially on the so-called milestones o f de
propriate. When certain phenomenon however is clarified velopment - beginning to walk and to speak, control over
as a whole with some details only remaining to be speci excretory functions, communication with peers, problems
fied, closed questions (with limited number of replies, e.g. such as excessive timidnesss, difficult detachment from
“yes” or “no”) are preferred. mother, stammering, nightmares; coping in school, partic
ipation in games, interest to sex, masturbation, adolescent
Fluent conduct of the interview may be hindered by crises, experimenting with alcohol or drugs
plenty of reasons. The most common one is excessive anx • Premorbid personality: permanent individu
iety - which may be part of the symptomatology, or may al traits of the individual, manifesting out of the illness
be provoked by the interviewing itself. Or, the patient may context, in different circumstnaces, which are usually
not cooperate, or may be too loquacious (in mania), or mo described with words from everyday language, e.g. shy,
notonous. In such instances, it should be shortly explained vulnerable, distrustful, jealous, hot-tempered, callous, pe
that time is limited and topic or the last question should be dantic, insecure, haughty, etc.
reminded. Conversely, it is relevant to encourage talking • Social history: education, job, marriage or part
nonverbally (nodding, bowing forwards, interjections), or nership, level of functioning
verbally, e.g. by repeating last phrase of the reply to pre • Somatic diseases
vious question (“ ...you said you do not have desire to do • Current psycho-social situation: living alone or
anything ...?”). with others, way of earning one’s own living, friendships.
History taking, or anamnesis, is systematic informa • Mental status referes to current symptoms,
tion gathering about symptoms, their duration, last ep present at the time of the interview. Impression about them
isodes, onset and course of illness, family history, early is gained even while history taking, i.e. history taking
personal development, job, somatic diseases, social and and mental status partially coincide, particularly in the
family context, former therapies. Firstly, the nature of domains of emotions, psychotic and cognitive symptoms
signs and symtoms should be asked for - no matter wheth (Table 3). Specific questions for each domain in the pre
er they are current or from the past, - and only then their sented scheme for mental status assessment by domains
dating should be probed (When did this begin? Since when below are subject to the general rules for interviewing, and
T ab le 2. The in te rview in g process
Phase Content Process
Opening phase Examination’s framework Establishing contact and trust
Opening Open question
Body of the interview Anamnesis Attending to transfer and defences
Control over the interview Mental status Attempt to probe deeply
Closing phase Brief summarization Messages
Closing Planning of treatment and visits Expectations
Attitude to treatment
24. Interview and clinical assessment / 169
besides, asking for examples is always recommended. The are: Some people hear voices that others cannot hear. Have
domains are: you had similar experience? What precisely do you hear?
• Appearance, face expression, psycho-motorics: Do they comment you? Do they give you orders? How do
in this domain what is directly observed during the inter you explain these voices? Do you sense strange smells?
view rather than what is said, is being assessed, e.g. patient Do you see objects that others cannot see - o f people or
may look neglected, dirty, or with clothes with vivid col animals, for instance? Was this a vision, or was it real?
ours; with frightened expression, angry, or with reduced • Thought: assessment for disturbances in tem
mimic (to the extent of affective flattening); with changes po of the thought process, e.g. accelerated or retarded; in
in pose or in motor behaviour such as immobility, head form, e.g., associations by sounding, circumstantiality,
bent down, wild gesticulating; with catatonic phenomena incoherence; or in content, e.g. overvalued ideas, obses
such as freezing in poses, stereotypies, waxy flexibility; sions, and delusions. Exemplary questions for assessing
or with smacking (in late dyskinesias)-, along with social delusions are: Does it seem to you that you are watched?
behaviour during the interview, e.g. arrogant interrupting, Orfollowed? Or that your thoughts are read? Do you have
or self-absorption, a special mission? Do you think that you are a subject of
• Speech: tempo, e.g. accelerated or retarded; some experiment? Do you receive messages by radio or by
quantity, e.g., excessive talking in mania or in anxiety; the TV? Does it appear to you that some o f your thoughts
lexical and topical scope, e.g. poor speech or unusual use are not yours? Or that they are under alien control?
of words; articulation and intonation, e.g. murmuring, • Consciousness and cognitive functions: assess
monotony, whispering; disturbances in the flow of speech, ment for clearness of consciousness or for its qualitative
e.g. abrupt change of topic, unusual association making, alteration (like confusion), orientation (e.g., in time and
sudden interruptions. space), passive and active attention (e.g., poor concentra
• Emotions: including both assessment of more tion), memory (e.g., short-term memory), intellect (e.g., in
permanent emotional background (mood) and transitory, calculation tasks), and abstract thinking (e.g., in interpret
outwardly manifested emotional response (affect). Exem ing proverbs). Exemplary questions in this domain are:
plary questions for assessing emotions are: How is your How old are you? Do you know where are you? What time
mood? Do you feel low or desperate? Can you experience o f the day is now? What is today's date? Which month?
joy? Do you think life has lost any sense? (for depression); Season? Day o f the week? Who is the Prime Minister now?
or: Do you worry about different things more than usu Could you count from l to 10, please? And now, in the re
al? Do you feel tense, unable to relax? Have you experi verse order, from 10 to l?
enced sudden powerful fear? (for anxiety); or: How is your • Insight: assessment about degree of awareness of
self-confidence? Do the others like you? Do they find you presence of mental disorder, causes to which it is attribut
amusing and charming? Have you become more witty? ed and need for treatment. Exemplary questions for this
Do you think more clearly andfastly? (for mania). Besides assessment are: How do you think, what is the reason for
nature of experienced emotions, also their intensity, dura this? Could it be a psychological or nervous problem? Or
tion, shifts between extremities, scope and congruence to a disease?
the topic (e.g., revealing an aggressive drive with blunted • Closing phase. It is advisable that the interview
affect) are to be assessed. should finish with short smmarization (closing) and with
focus on treatment plan and future visits.
• Perceptions: presence of hallucinations or illu
sions frequently becomes evident in the course of the in CONTENT AND PROCESS
terview even without asking questions; their elaboration
however is related to affected perception domain (modal While interviewing, the clinician listens not only to
ity), content, duration, frequency, impact on behaviour, the story but also to the way it is told. The plot (content)
insight, and their relationship with other symptoms (like goes along with a process of feelings exchange, a pecu
delusions). Exemplary questions for assessing perceptions liar “second level” of talk which is not always named and
Table 3. A n am n e sis and mental status - correspondence
Domains of anamnesis Domains of mental status
Consciousness and cognitive functions Consciousness and cognitive functions
e.g., getting lost out o f home, forgetfullness, losing ob e.g., impaired orientation, attention, memory, compre
jects hension
Perception and thought Perception and thought (form and content)
e.g., talking to oneself, frequently unintelligible, behav e.g., hallucinations, loose associations, delusions
iour influenced by persecutory ideas
Emotions Emotions
e.g., low mood in the last few weeks е p depressive affect during the interview
Somatic signs Appearance
e.g., changes in sleep and weight e.g.. skinny, neglected
Behaviour and functioning Observed behaviour
e.g., callousness, conflicts e.p.. arrogance, hostility
170 / Psychiatry
realized. Accounting for both levels of the interview is a ing for general brain symptomatology is of crucial
sign of good therapeutic attitude. It requires both skills importance. It is helpful also to assess higher cortex
for observation, as well as for self-observation. The term functions, such as speech (e.g., naming parts of ob
transfer denotes emotional responses and attitudes of ject), or praxis (e.g., making simple figure with match
the patient towards the clinician based in patient’s pre es), and in some cases also to look for micro-neurolog
vious experience with significant figures in his/her life. ical abnormalities.
Contra-transfer is emotional response of the clinician
towards patient’s attitudes. Observation of process and Para-clinical assessment. In psychiatry, there is no
conduct of the interview are not done separately but si one universal or mandatory para-clinical test. The need
multaneously, in parallel (see Table 2). This observation of additional investigations or consultations is subject to
is related to assessment of defence style of the patient - clinical judgement, e.g., EEG, serology, or neuro-imag
his/her specific tools for protecting oneself from anxiety ing methods. Intake of some antipsychotics requires li
and conflict topics. pids and blood glucose (for some - neutrocytes) tests and
ECG.
OTHER SOURCES OF INFORMATION
Clinical summarization. Written registration of
Other sources of data add to what the patient said, findings from the examination has medical, and in some
or may correct it. History from relative (taken in the cases legal, value. Documentation reflects anamnesis and
same order as in the interview with patient) is particu mental status according to the presented schemes, while
larly important. Data from relative may be very helpful summarization organizes information into more concise
when the patient does not provide realistic account of his/ record. Treatment plan is the link between diagnosis
her behaviour, either because of depreciation (mania), and treatment. Where plausible, it should be made with
forgetfullness (dementia), disguising (alcohol problem), patient’s participation.
or due to delusions. Medical documentation is addition
al valuable source of data. In all cases however, critical Structured instrumental clinical assessment.
weighing of the value and reliability of different sources Standardized instruments for clinical assessment are
is needed: what is said by somebody else, may be just a increasingly widely used in practice, besides, in many
different view point, and not by all means the truth. countries - predominantly by non-psychiatrists, e.g. by
nurses or social workers. Through instrumentalization,
Interview in specific situations various aspects of human behaviour and experiences
When interviewing in emergency situations, it is im can be assessed in a standardized and quantitative way.
perative to obtain brief information from other sources Standardization means that: a) normal range of replies is
before medical examination, along with prompt orien ascertaind in advance in healthy controls, with the replies
tation, about: a) leading signs and symptoms, b) level of the subjects that are assessed compared against them;
of lucidity of consciousness, and other signs of organic b) with limited options for reply (e.g., “yes” or “no”), the
causes or intoxication, c) imminent risk of aggression or reason for variation of replies is in the individual being
auto-aggression. Clarification of these three aspects of assessed, not in the instrument. There are different types
the clinical state has priority over gathering and assess of clinical instruments according to their content and
ment of other data. One should keep in mind that a state way of application. From the perspective of procedure,
of stupor may abruptly shift into a state of excitement, they are divided into standardized interviews, scales for
as well as that alcohol abuse and past aggressive acts do self-assessment, and scales for assessment by rater. In
increase current risk of aggression, regardless of the spe structured interview there is strict sequence and stand
cifics of clinical picture. When examining a dangerous ard formulations of questions applied verbatim, while in
patient, presence of staff or security is approprate, and semi-structured interview some flexibility is allowed.
when immobilization or involuntary medication are in
dicated, these individual coercive measures have to be Some of the widely recognized and validated struc
applied by trained staff according to established rules. tured interviews and assessment scales are: Composite
International Diagnostic Interview (CIDI), Brief Psychi
Other assessment and summarization atric Rating Scale (BPRS) - for general psychopathology,
Somatic status. It depends on specific clinical judge Mini-Mental State Examination (MMSE) - for cognitive
ment how detailed bodily examination in each case should status, Global Assessment of Functioning Scale (GAF)
be, however it should be kept in mind that bodily health - for assessment of level of functioning. Psychological
(dental health including) of psychiatric patients is gener tests provide additional information about intellect, neu-
ally neglected. In emergency and in states with unclear ro-psychological processes or personality profile. Their
clinical picture, it is appropriate to assess with priority results may be very useful with correct interpretation, es
vital signs, i.e. blood pressure, pulse, and temperature. pecially in cognitive impairment, personality deviations
or discrete (subtreshold) clinical manifestations. Tests
Neurological status. In clinical practice, when in may not however substitute clinical judgement, and may
doubt about organic causes or intoxication, examin not serve as main source for diagnoses.
25. General psychopathology 171
dromes. Grossly in this order relative specificity of the pening? Or, that you may get a stroke, or loose your
syndromes, as well as their weight in diagnostic hierar mind?
chy, increase.
DEPRESSION
ANXIETY
• Depression is disease syndrome characterized
Anxiety is universal human reaction to insecurity by:
and threat. When it is excessive and not corresponding • permanent low mood;
to its cause, anxiety may reach pathological dimensions • inability to experience pleasure (anhedonia)\
and affect everyday functioning, manifesting basically • lack of energy;
with three groups of symptoms: • loss of weight, sleep, appetite, and desire for
• mental: excessive worrying, anxious anticipa sex;
tion, fears; • motor retardation, or uneasiness, agitation;
• muscular, inability to relax, aches (back, neck, • low self-confidence or feeling of guilt;
and head); • retarded thinking, difficulties in concentration
• autonomous: sympathicus excitement (palpita or in decision making;
tion, mouth dryness, sweating, increased bowel motili • ideas for death or suicide.
ty, dizziness).
Normal prototypes of these manifestations are not
Anxiety is nonspecific syndrome accompanying a lot uncommon to most people. Clinical depression however
of somatic and mental illnesses, as well as many abnor is not trivial experience: unlike the latter, it is lasting
mal illness behaviour styles. When it does not accom (each day for at least two weeks), cannot be influenced
pany any other disease, excessive anxiety is at the root by outer stimuli, and has serious impact on coping with
of the so-called anxiety disorders manifesting in dif everyday activities. Low mood may have different nu
ferent forms: generalized anxiety, panic attacks, agora ances: sad, “dull”, cheerless, “flat”, empty, despondent.
phobia, specific phobias, social phobia, obsessions and Depressive syndrome may demonstrate in variety of
compulsions. Despite specific features of these different forms depending on various different combinations of
syndromes, there are common characteristics of all of symptoms. Low mood and loss of ability to experience
them: irrational fears, excessive autonomous reactivity, pleasure however are always present.
tension, “fainting” with feeling of shortness of breath
and expectation of bodily collapse. Somatic symptoms Depressive thinking is, as a rule, retarded, monoto
are frequent masks of anxiety. nous, with the feeling of unefficiency, or “dullness”, and its
content is concordant (or, congruent) to the emotions, and
The interview with anxious patient has some spe in a sense, due to them: with dominating ideas for failure,
cifics. Most of the anxious patients are too loquacious. loss of meaning, poverty, loss of perspective, exaggeration
Their excessive talking and complaining style are usu of one’s faults, and guilt. Sleep is usually disturbed in its
ally expression of resistance against introduction of later phase, with characteristic early awakening, and with
psychological topics. more painful experience in the morning. Drive oppression
leads to refusal to eat, wight loss, neglect of hygiene, or
Useful questions in the interview with anxious pa severe immobility (<depressive stupor). Combination with
tient are: anxiety results in marked uneasiness, to the extent of ag
□ Do you worry about different things more than itation - with inability to stay still, with noisy breathing
usual? Even about small matters? and sighing, and aimless fidgeting.
□ Do you feel tense, as if on your guards?
□ Do you feel tightening or pain in the neck? In In some atypical forms, there may be excessive eat
the head? ing (without however experiencing pleasure from food),
□ Have you become more irritable? weight gain, and sleepiness. The main risk in depression
□ Can you relax? is of suicide. Assessment for suicide risk by purposeful
□ Do you have dizziness, palpitation, sweating, interrogation is mandatory in depression. Severe de
tremor, nausea? pression may be complicated by psychotic symptoms,
□ Are you afraid to go out alone? Or, to travel most commonly by delusions for guilt (i.e., psychotic
alone with public transport? Or, to stay among others depression). Depression is basic syndrome of affective
(in queues, vehicles, crowds)? disorders, but may be present transitionally in other
□ Are you afraid of any objects or animals? mental disorders also.
□ Do you feel too much worries to talk in front of
other people? Useful questions in assessment of depression are:
□ Have you experienced a sudden attack of fear □ What is your mood? Do you feel sad? Low? Or,
without any reason? Feeling that something bad is hap desperate?
25. General psychopathology 173
□ Do you feel weak, without energy and strength? content: from unrealistic over-selfevaluation to mega-
□ Do you feel confident? lomanic delusions (for wealth, talents, celebrity, noble
□ Can you experience pleasure or joy? descent) in psychotic mania. When the syndrome is
□ Have you lost your usual desires or interests? severe, it may present with excitement, unintelligible
□ Do you feel that you think more slowly? That speech, and dangerous behaviour {confusedmania). Ma
you do everything more slowly? nia is basic syndrome of bipolar affective disorder.
□ Can you concentrate?
□ Do you feel you are useless? Empty? Stupid? Interviewing manic patients requires efforts for
Guilty? If guilty, what for? keeping up the timeframe and for holding their exces
□ Do you think that things will improve? Did you sive familiarity and talkativeness. Confrontation should
lose hope? * be avoided with angry patient, and interviewer’s behav
□ Has your appetite changed? Have you lost iour at the same time should be firm and demonstrating
weight? confidence and control.
□ How do you sleep? Do you wake up earlier than
the usual for you? Useful questions in the interview with manic patient are:
□ When do you feel worse - in the morning or in □ How would you estimate your mood? And,
the afternoon? your confidence?
□ Do you think that life has lost sense? That it is □ Does it seem to you that you could easily cope
not worth leaving? with everything?
□ Have you made plans to end your life? □ Do the others like you? Do they find you amus
□ By what means? When? ing; are they looking for your company?
□ Can you postpone it? □ How many hours do you spend in sleep? Do you
get tired?
□ Do you feel rush of energy? As if, being
MANIA “charged”?
□ Have you found recently that you have abilities
Mania is syndrome with increased drives, level of unsuspected so far? Talents?
vigility and activity manifesting with: □ Have you become more witty? Do you think
• permanently hightened (hyperthymia) or irrita more clearly, in more creative way?
ble mood; □ Do your thoughts flow more rapidly? So rapid
• increased initiatives and activity, with de ly, that you cannot control them?
creased tiredness and need for sleep; pressure o f speech; □ Can you think about several things at the same
• accelerated mental activity, to the extent of time?
flight o f ideas, extreme distractability; □ Can you stay passive, or you have to do some
• increased self-evaluation, to the extent of meg- thing all the time?
alomanic delusions;
• increased libido and familiarity;
• decreased insight. ACUTE PSYCHOSIS
Sometimes manic patients are viewed by others not Psychosis is a broad concept, applied to large group
as mentally ill, but rather as arrogant or intoxicated. of syndromes w ith altered (as compared to individual’s
Milder forms of the syndrome (known as hypomania) and cultural norm) attitude to reality, incorrigibility,
may in fact provide some selective advantage with in and presence of symptoms such as delusions, hallucina
creased sociability, libido, and energy. It is diificlut tions, and disorganized behaviour. Acuteness is a term
however to not recognize conspicuous manic syndrome. denoting both abruptness in onset (in terms of days),
It is assumed that the main symptoms have to be pres as well as pattern of symptom manifestation: stormy,
ent for at least a week to qualify for the diagnosis; as a emotionally intense, changeable. Signs o f acuteness are:
rule however, manic syndrome is longer lasting. Hight • delusional mood —perception of strange mean
ened emotionality is not concordant to outer events, and ing in otherwise unchanged surrounding, frequently
may be contagious and fun, or may manifest with anger puzzling or sinister, with hidden messages or hintings
(dysphoria). Behaviour is dominated by expressive psy- • disturbance in motor behaviour
cho-motorics, excessive spending, unreasonable initia • sleeplessness.
tives, increased sexuality, talkativeness (frequently with
hoarse voice), distractability, grandiose posture, some Besides these basic signs, at the onset of psychotic
times with a manner of powerfulness. episode frequently non-specific complaints, withdraw
al, or starrings are present also. They are followed by
Thinking and speech are accelerated, with rap rapid escalation, with intense affect (usually, of fear)
id shifts between topics, sometimes with associations and positive psychotic symptoms:
by sounding and punning, and with expansive ideas in • hallucinations such as hearing voices, or sens
174 / Psychiatry
ing strange smells or tastes; or delusional perceptions is also not recommended; the most relevant attitude is
- wrong interpretativeness with otherwise intact per neutral.
ception, e.g. receiving special messages;
• delusions - incorrigible ideas frequently de Useful questions in the interview with psychotic pa
veloping as “explanations” of other unusual experienc tient are:
es, with emotional intensity, not allowing for alterna □ Have you had unusual experiences recently?
tives (e.g., mere chance), such as conviction that one’s □ Can you read other people’s minds? Or can they
thoughts are recorded, that one is poisoned, followed, read yours?
watched, or is victim of experimentation; □ Do you think that others are talking behind
• the so-called first-rank symptoms for schizo your back? Plotting against you?
phrenia, characterized by experience of outer intrusion □ Have you noticed that you are being spyed on?
in one’s mental activities and loss of control over them: Or, followed? How do you explain this?
feeling of being revealed, thoughts “spoken aloud’’, □ Do you think your life is in danger? Have you
thought broadcasting, thought insertion, thought block looked for help?
ing, thought withdrawal (may be interpreted as stealing □ Do you have contact with supernatural power?
of thoughts), replacement of one’s volition, emotions Tell me something more about this.
and thoughts with stranger’s. □ Do you think that you are subject to some kind
• catatonic symptoms such as freezing in poses, of experiment?
catalepsy with waxy flexibility, stereotypies, imitative □ Some people hear voices that others cannot
acts or chaotic destructiveness; hear. Have you had similar experience?
• disorganized and impulsive behaviour; □ What precisely do you hear? Is it clear like my
• form al thought disorder such as peculiar turn voice now, or is it just inside your head?
of speech, unintelligibility. □ Do they talk to you, or between themselves? Do
they comment you?
These psychotic symptoms change individual’s be □ Do they give you orders?
haviour, sometimes beyond recognition, and directly □ Where these voices come from? How do you
control it. The psychotic individual may take measures explain them?
for self-defence, accuse, warn about imminent threat, □ Have you received messages from radio or TV?
transmit “coded” messages to and from “chasers” and □ Have you noticed something strange happening
“rescuers”. Basic aspect of assessment for psychosis is with your body?
with interrupted contact with reality and related in □ Have you noticed that some people look as if
corrigibility by logic and common sense reasons. The being changed? As if being dummies?
fact that one’s views are not shared by others, instead □ Have you sensed some strange smells?
of shaking his/her opinions, frequently rather increases □ Have you feeled that some thoughts are not
his/her conviction in a plot and in the necessity to de your own but rather are stranger’s ones?
fend onself. There are different types of delusions ac □ Have you experienced that your thoughts sud
cording to their topic and level of systematization (see denly disappeared?
Glossary at the end), while depending on which of the □ Or, that your thoughts are controlled by some
psychotic symptoms prevail in clinical picture psychot outer power?
ic syndrome may be: delusional, hallucinatory, delu □ Or, that they are sounding in your head? Can
sional-hallucinatory, catatonic, etc. Acute psychosis has others around you hear them?
low nosological specificity. It may occur in the course □ How do you think, what is the reason for all
of schizophrenia, affective disorders, schizo-affective these?
disorder, organic disorders, intoxications.
Chronic psychosis
In assessment it is mandatory to evaluate also level Chronic psychosis is characterized by the so-called
of predictability of behaviour and the risk of aggression negative psychotic symptoms:
or auto-aggression. In cases of lack of speech (mutism), • poor thinking (to the extent of alogia) and poor
clinical assessment is reduced to observation of psy- speech
cho-motorics: displayed emotions (e.g., fear, ecstasy), • hypobulia (to the extent of abulia)
trends in motor behaviour (e.g., hostility or withdrawal), • fla t affect and anhedonia
signs of intense hallucinating (lending or closing ears), • social withdrawal.
strange movements with symbolic meaning or chaotic
or jerky ones. Mutism is rarely full; more frequently it Positive symptoms are frequently also available, but
is partial and due to preoccupation with hallucinations, without the intensity and preoccupation in acute psy
hostility, or protest. The interview is usually feasible chosis. Functioning in chronic psychosis is dependent
when providing calm approach and atmosphere of se mainly on negative symptoms. Unlike positive ones with
curity and confidence. Generally, delusions should not their morbid “creativity”, there is loss of normal func
be confronted directly; however, agreement or approval tions with negative symptoms. As a result, patients are
_________________ 25. General psychopathology 175
indifferent, with low motivation and energy, apathetic and neuro-imaging testing. After delirium, recollection
expression, frequently with poor hygiene. Their every of events is incomplete; there may be recovery, but fre
day life is monotonous, and communication with others quently also worsening of cognitive functions (demen
is reduced and formal (autisation), without experienc tia) or impairment of memory fixation with filling mem-
ing torment or boredom about this. Cognitive decline, ory gaps with confabulations (Korsakoff syndrome) are
self-sufficiency, poverty, and dropping out of the social evident.
networks are present. Unlike acute psychosis which is
viewed as morbid condition even by laymen, chronic pa Useful questions in assessment of delirium are:
tients look more as if idle and neglected rather than ill. □ Where are you just now? How is this place
The syndrome occurs in late phases of schizophrenia, called?
but also in some other mental disorders with chronic □ Who are the people around? Are you afraid of
course, especially when combined with organic states them?
and institutionalism. Clinical assessment for chronic G Do you know me? Have you seen me before?
psychosis includes needs evaluation. Was I here yesterday?
□ What time is it now? Which part of the day,
Delirium approximately? Which day of the week?
Delirium is acute organic brain syndrome manifest □ Where have you been during the night? Did you
ing with: sleep here?
• qualitative disturbance of consciousness, with □ Did something unusual happen in the night?
altered perception and orientation, despite level of alert How would you explain this?
ness (vigility, i.e. the quantitative aspect of disturbance □ Do you see some images - of animals or peo
of consciousness); ple? Are you scared of them?
• misidentifications, illusions, and hallucina □ Was this a vision, or it really happened?
tions, mostly visual ones; □ Do you feel crawling, as if by insects?
• disturbed time and space orientation, with pre □ Is anybody hiding in your room? Under the
served self orientation; bed?
• sleeplessness, or severe disturbance of the □ Could it have been just a dream? Are you feel
sleep-wake cycle; ing puzzled? Helpless?
• motor uneasiness, usually limited within the □ Do you feel better when the light is on?
room;
• intense fear, less frequently ecstasy, or fluctua Dementia
tions between them; Dementia is chronic organic brain syndrome mani
• marked autonomous signs. festing with:
• memory failure, predominantly for recent
Delirious patient is commonly restless and fidgety. events, with realtively preserved, at least at the begin
His/her behaviour is excited, helpless, with scared look ning, reproduction of events from remote past;
ing round, starring, pointing at certain places, clinging • impairment of intellect, as well as of thinking,
to others, fidgeting, or constantly crushing up the linen judgement, calculation, orientation, learning, speech,
when in bed. Active attention and sleep are markedly comprehension;
disturbed, and diurnal fluctuations with exacerbation • loss of emotional control with frequent deprcs-
of symptoms in evenings are characteristic for the con sivity, apathy, emotional instability, or euphoria with
dition. Blushing face, sweating, coarse tremor, visual flat joking;
hallucinations, frequently with visions of animals, tac • decline of everyday habits, personal hygiene,
tile hallucinations (i.e., feeling of crawling), and intense and social behaviour.
fear are typical for alcohol delirium. Delirium causes
confusion: with misidentifications and disorientation to If delirium is not superimposed, consciousness is not
time and space, though with intact self orientation. The clouded. The main impairment is cognitive (in memory
latter is disturbed in more severe form of the syndrome and intellect, predominantly), but it is frequently masked
- amentia. Contact with delirious patient is difficult, by behavioral and emotional diturbances. The patient
while with the amentive one is impossible. may look uneasy, scared, or depressed, neglected, melt
ing, flatly joking, challenging, or insolent. With availa
Delirium is sign of organic brain damage, and may ble insignt, usually at early stage of dementia, patients
arise out of severe somatic diseases, intoxications, or may try to conceal their fading memory, or compensate
after general anaesthesia. In medical practice, deliriums it by aids, such as writing in a notebook. At later stage
are common in intensive care units, the most common however, difficulties in memory and judgement become
ones being alchol delirium and delirim in vascular dis conspicuous and may result in losing oneself, misiden
ease. Thorough assessment includes somatic and neu tifications, distortion of memories, impoverishment of
rological status (with special emphasis on symptoms of thought content, deterioration of habits and need of con
meningo-radicular irritation), additional para-clinical stant supervison and care.
176 / Psychiatry
Many systemic and brain diseases may cause de □ naming days of the week or months backwards;
mentia. The main types in adults are Alzheimer’s type □ subtracting 7 from 100, 7 from from the result
and vascular dementia. In Alzheimer’s type of demen ing number, etc. - up to 5 actions.
tia, memory deficits are at the foreground, course is
gradual, and there is frequently depression. In demen Useful questions in assessment of dementia are:
tia with predominantly vascular etiology, course is un □ What is your name? How old are you?
even, stepwise, with worsenings and improvements, □ Do you know where are you? Are you in your
with marked diurnal dynamics, and there is frequently home or in hospital?
emotional instability. Thorough somatic and neurolog □ What time of the day is now? What time is it?
ical assessment in dementia is mandatory. Looking for □ What is today’s date? Which month? What time
focal neurological symptoms, neuro-psychological as of the year? Which day of the week?
sessment, detailed history taking, and examinations for □ Who is the person behind you (caregiver, staff)?
possible curable causes of the syndrome, are imperative. And who is on this photograph?
□ When were you born?
In clinical assessment, some tasks informative about □ What was the name of your first teacher?
cognitive diturbance are: □ What is the name of your husband (wife)? Of
□ finding out similarities and differences be your childern? Of your granchildren?
tween apple and orange, dwarf and child, etc. □ I will tell you a name and an address. Can you
□ interpreting popular proverbs such as “The grass is repeat it. Try to remember it ...
always greener on the other side of the fence”, “Do not judge □ When did the First World War lasted? And the
the book by its cover”, “A rolling stone gathers no moss”, or Second one?
replying to questions such as “If the flag is waving to the □ Who is the Prime Minister? And the President?
west, from which direction is the wind blowing?”; □ Count from 1 to 10. Now try to count back
□ naming 3 objects, and repeating them after 2 wards, from 10 to 1.
and after 5 minutes; □ Can you repeat the name and the address that I
□ repeating of a sentence; told you just a moment ago?
ESSENCE AND AIMS OF PSYCHIATRIC embodiment of meaning of the word diagnosis: “learn in
DIAGNOSIS AND CLASSIFICATION depth" (Greek, diagignoskein), which goes beyond the
narrower meaning of identification of illness disorder and
Classifications satisfy our needs of predictability its differentiation from other entities (differential diag
and serve to organize knowledge in some area accord nosis). These two aspects reflect two approaches to diag
ing to certain rules. Classifications of living organisms nostic process. The first one ranks patient with group of
are known as taxonomies, while nosology denotes clas people with common pathology (nomothetic approach),
sifications of diseases in medicine. Basic aims of diag and the second one views him/her as unique human be
nosis and classification in psychiatry are identification ing (ideographic approach). While the first approach is
and description of cases, their assignment to categories, needed for summarizations and care standards, the oth
estimation of cost of care, communication between pro er one is useful for understanding unique characteristics
fessionals and institutions, planning of treatment, prog and for working out individual treatment plan.
nosis, and research.
Pathological states can be differentiated into catego
Diagnosis is central concept in psychiatry practical ries and dimensions. Categorial approach assign cases
ly defining its field. Diagnostics in psychiatry requires to groups with clear boundaries, while dimensional ap
two key skills The first of them is recognition of symp proach assign them to dimensions where they spread on
toms and morbid behaviours, their examination, and uninterrupted continuum (axis) from one extremity to
correct juxtaposition to criteria of diagnostic categories. another. Smooth transition between norm and pathology
The other skill is to examine patient in totality, to grasp is common, e.g. with hypertension and diabetes. At the
links of symptoms to his/her personality, life path and same time, pathology beyond certain threshold in this
circumstances accompanying illness, and thus to aquire transition is so clinically significant that it has to be dis
understanding of his/her individuality. This skill is sheer tinguished from milder cases.
26. Diagnosis and classification of mental disorders 177
Grouping of cases are subject to classification p rin havioural version) or in cognitive processes (cognitive
ciples which generally are etiological (according to version), and are erroneously learnt responses resulting
causing agent) or syndromological (according to clini from unfavourable events or from cognitive distortions
cal picture). Classification in medicine are based usually maintained by some supportive factors. Therapy is tar
on multifactorial determination, while purely etiolog geted at specific maladaptive behaviours and cognitive
ical approach is relatively rare. Contemporary psychi processes.
atric classifications are eclectic and based on different
principles in different parts, e.g. etiological with alcohol Each paradigm has own empirical evidence and bears
and drug use disorders, or syndromological with mood however its limitations with the efforts to ground all dis
disorders (Table 5). Certain rules of hierarchy also ex turbances in single certain level of impairment (know n as
ist, e.g. organic symptomatology dominates over other reductionism). The so-called biopsychosocial paradigm
levels of impairment, while anxiety level of symptoma is an attempt to overcome these limitations. According to
tology is the lowest and most unspecific one. it psychopathology is complex response to stress factors
acting on biological, psychological and social indibidual
The terms reliability and validity are related to the vulnerability, while symptoms are manifestation of un
infromative value of diagnostics. Reliabilty denotes re successful! coping with these factors. Modern psychia
producibility of diagnosis reflecting the degree of agree try is basically with integrative orientation. Helpful for
ment between psychiatrists when assessing same clini understanding clinical manifestations within integrative
cal condition. Large international studies have demon approach is the differentiation between predisposing
strated that reliability of contemporary psychiatric di (e.g., genetic vulnerability), precipitating (e.g., stress)
agnosis is generally high, even higher than reliability of and supporting illness (e.g., non-compliance) factors.
many instrumental examinations in medicine. Validity
denotes the extent to which diagnosis reflects underly
ing pathology. It is unsolved so far problem in psychi MENTAL AND BEHAVIORAL DISOR
atry, and the essence of underlying pathology depends DERS IN ICD-10, CHAPTER V
highly on paradigm's context in which it is viewed. For
this reason, in the absence of outer marker, clinical ap Contemporary internationally recognized and estab
proach still remains the most trustworthy way to know lished classifications are WHO’s International Classi-
in detail sick person and to assign him/her to a group ficaton of Diseases, 10th revision (ICD-10) and the Di
with similar pathology. agnostic and Statistical Manual of the American Psy
chiatric Association, 5th edition (DSM-5). They have
common characteristics, and although DSM is preferred
PARADIGMS ABOUT MENTAL DIS for reseach purposes, in Europe ICD-10 is approved
ORDER for daily clinical use. Mental disorders in ICD-10 are
described in Chapter V where diagnoses are indicated
Paradigms are theoretical models containing not by combined letter-digital code (from F00 to F99). The
only scientific but also ideological componenets, as well classification is not nosology but operational taxono
as criteria about what should be considered as scientific. my, i.e. diagnosis is based on descriptive signs and in
Test of a paradigm is its examination by empirical way. structions for checking them rather than on hypotheses
about possible causes (Table 4).
Biological paradigm dominates in clinical psychia The basic diagnostic entity is not disease but disor
try, and its traditions are traced back to Hippocrates and der defined as clinically recognized set of symptoms or
embodied in nosological views of Kraepelin. According
to it, mental disorders manifestations are results from Table 4. Basic principles o f contemporary psychiatric clas
disturbed physiological processes, and mental disorders sifications
themselves are diseases affecting brain, similarly to Taxonomies, not nosologies
other diseases in medicine affecting other organs and “Disorders” rather than diseases
systems. Treatment is targeted at compensation and re Eclectic and descriptive approach
covery of neurobiological disturbances. Based on evidence, not on theories or schools of
thought
According to psychodynamic paradigm psychopa High reliability but unknown validity
thology is result of unconscious intrapsychic processes: Hierarchical rules
conflicts, disturbances in development or defects in the Operationalization
so-called object relations. Therapy is oriented to con Quantitative criteria: intensity, duration, frequency of
flict resolution with Ego consolidation or to character manifestations
change. Assessment of degree of distress due to symptoms
Assessment of symptoms’ impact on functioning
With behavioural paradigm symptoms are viewed Possible multible diagnoses and comorbidity
as display of diturbances in behavioural models (be Multiaxial assessment_______ ___________________
178 / Psychiatry
T able 5. D iagn ostic se c tio n s in ICD-10 and cla ssification principles
Diagnostic sections - ICD-10, Chapter V (F) Principles
FO. Organic, including symptomatic, mental disorders, e.g. dementia in Alzheimer’s Etiological/ syndromolog-
disease, delirium ical
FI. Mental and behavioural disorders due to psychoactive substances use, e.g. harm Etiological
ful alcohol use, opioide dependence syndrome
F2. Schizophrenia, schizotypal and delusional disorders, e.g. paranoid schizophrenia, Syndromological
delusional disorder, acute polymorphic psychotic disorder
F3. Mood (affective) disorders, e.g. bipolar affective disorder, recurrent depression Syndromological
F4. Neurotic, stress-related and somatoform disorders, e.g. panic disorder, obses Syndromological
sive-compulsive disorder
F5. Behavioural syndromes associated with physiological disturbances and physical Syndromological/
factors, e.g. anorexia nervosa, erectile dysfunction etiological
F6. Disorders of adult personality and behaviour, e.g. paranoid personality, histrionic Syndromological/ affect
personality, transsexualism ed phase of development
F7. Mental retardation, e.g. mild mental retardation Affected phase of devel
opment
F8. Disorders of psychological development, e.g. childhood autism, specific disorder Affected phase of devel
of reading opment
F9. Behavioural and emotional disorders with onset usually occurring in childhood Affected phase of devel
and adolescence,e.g. attention deficit hyperkinetic disorder, tic disorder opment/ syndromological
behaviours which are usually associated with distress ing sense of and integration of clinician data includ
and functional impairment. Neurosis and psychosis are ing uniques case specifics, and not just its assignment
not explicitly defined and differentiated but denotions to diagnostic category. Ideographic (or personalized,
are preserved for descriptive convenience, e.g. the term unique) case formulation accounts for individual mean
psychosis as denoting presence of delusions, hallucina- ing of illness and is aimed at agreement with the pa
tios, catatonic phenomena. The classification is based tient for treatment purpose. It is based on comparison
on evidence, and on different principles in its different between patient’s, relatives’ and clinician’s viewpoints
parts (Table 5). Multiple diagnoses and comorbidity are about the clinical condition. Unlike medical diagnosis,
possible. The classification’s version for general prac case formulation assesses not only pathology but also
tice (see Recommended reading) is particularly useful healthy personality resources, i.e. positive factors with
for nonpsychiatrists. the patient such as skills, talents, sources of support.
27. SCHIZOPHRENIA
Paranoid form is the most common one (and more Clinical phases in the course of schizophrenia are:
common in developed countries) and is characterized by • Prodromal - gradual progression of yet pre-
predominance of delusions and hallucinations in clini morbidly present discrete cognitive, emotional or be
cal picture, without marked disorganization, affective havioural disturbances, onset of functional deteriora
disturbances or catatonia. tion, emergence of odd ideas and perceptions
• Acute - overt display of positive psychotic
In hebephrenic form, called also disorganized, symptoms
speech, social behaviour, drives and emotions are se • Chronic - prevalence of negative symptoms.
verely impaired. Positive symptoms are vague or absent.
Deterioration of habits, hygiene and cognitive functions Different courses of illness are possible:
prevail. Childish sillines may be present with early on • Single episode without deficit
set, and inadequate emotional response (parathymia) - • Recurrent episodes without or with minimal
with severe course. Disorganization manifests in behav deficit between them
iour (strange, aimless, impulsive) and in thought flow • Chronic course with exacerbations and con
(poor, unclear, with formal thought disturbances).~Clin- stant deficit between them
ical picture is complemented by negative symptoms and • Chronic course with exacerbations and pro
severe deficits in communication. gressing deficit between them.
In catatonic form catatonic symptoms such as stu Patients with schizophrenia are with poorer somatic
por, waxy flexibility, posing, negativism, mutism, or health (including dental health), lower life expectancy
odd movements and grimacing, excitement, echolalia rate and higher suicide risk, as compared to the general
and echopraxia prevail. This form is more frequent in population. In acute psychotic episode their behaviour
developing countries. Rare but potentially fatal is the may be unpredictable and dangerous, while in chronic
so-called febrile catatonia - catatonic syndrome or se phase it is prevailed by social deterioration, passiveness
verely disorganized behaviour combined with hyper and neglect. Despite this, in later stages of illness p a r
pyrexia, severe autonomous collapse and clouded con tial recovery is observed with approximately half of all
sciousness. patients.
The so-called simple form represents gradual devel Long-term prognosis depends on different factors.
opment of negative symptoms and behavioural oddities, Better outcome is usually associated with: presence
without positive symptoms and other manifestations of spouse or partner, friendships and relatives, female
characteristic for the other forms, however with pro gender, good premorbid functioning, higher education,
gressing deficits in communication and in functioning. presence of job, acute onset, later age of onset, precip
It is a rare form, not fully recognized and not accepted itating stress, florid but shorter episodes, absence of
by all clinicians. hydrocephalus internus or coarse neuro-psychological
impairment, absence of comorbid drug abuse, presence
Other forms are: undifferentiated - meeting criteria of good initial therapeutic response and compliance to
for schizophrenia however not matching any of the oth treatment.
major depressive or manic episodes, but at the expense being main rules of its management. With recurrent de
of this become chronic. Dysthymia is characterized pression and risk of relapse, treatment is continued dur
by constant or recurrent low mood for at least 2 years ing remission also, usually with the same antidepressant
not meeting criteria for major depression, and without with which improvement has been reached during the
hypomania. In cyclothymia, mood lability for at least illness episode.
2 years is present with episodes of mild depression or
hypomania not meeting criteria for major depression or Antidepressive response may be augmented by oth
mania, with or without euthymia between them. These er agents, e.g. thyroid medications or mood stabilizers,
conditions are more common among relatives of patients while with psychotic depression antipsychotics are add
with UD and BAD. Seemingly not overt, they worsen ed. Electro-convusive therapy (ECT) is very efficient in
quality of life and frequently remain untreated. severe or resistant depression, psychotic depression, or
with some co-occurring somatic or physiological (e.g.,
pregnancy) conditions. Other treatment options include
TREATMENT sleep deprivation, light therapy, n.vagus stimulation or
recurrent transcranial magnetic stimulation (rTMS).
Efficient treatments for affective disorders are avail With the so-called bipolar depression (depressive episode
able. Treatment is different in UD and BAD, as well as of BAD), mood stabilizers are added to antidepressants.
in disease illness episode and in remission. Biological
treatment is with priority, although cognitive-behav Treatment of mania, hypomania, mixed episode and
ioural and interpersonal therapies can by applied suc relapse prevention in remissions requires mandatory
cessfully in some depressions. Antidepressants are the temperament or mood stabilizers: lithium salts (lith
main group of medications for treatment of depression. ium carbonate), valproates, carbamazepine and lamo-
Those from older class like tricyclics are highly effi trigine. In treatment of mania mood stabilizers are com
cient but also with many side effects, and because of bined with antipsychotics, particularly with excitement
this nowadays antidepressants from newer classes, i.e. and psychotic symptoms, and with severe syndromes
SSRI (enhancing serotonine transmission) and SNRI and lack of efficacy - with ECT also.
(enhancing serotonine and norepinephrine transmis
sion), are more frequently used. They are more safe and With mania hospitalization is commonly imperative,
well tolerated, although not more efficient than tricy- while most depressions can be treated on out-patient ba
cics. Onset of antidepressants’ benevolent influence on sis. Treatment is long-term oriented and based on ade
symptoms becomes distinctly apparent after around 2 quate relapse prevention, psychoeducation, compliance,
weeks of medication intake, but treatment lasts for at partnership, e.g. in recognizing early signs ot relapse,
least 6 months, with consistency and adequate dosing and suicide risk monitoring.
184 / Psychiatry
spite volitional attempts to resist them. Most frequent ing stress reactions, i.e. loss, separation, diseases, mi
topics are associated with order, hygiene and check gration. Such events cause pathological reaction only
ing, e.g. unnecessary repeating washing hands, putting in some individuals, hence the priority of individual
clothes on and off in certain order, senseless counting. vulnerability. Response encompasses not only reaction
They are frequently accompnied by futile analysing to event but also adaptation to its consequences with
(“rumination”), general retardation, unefficiency and re-adjustment of way of life. Manifestation includes
disability. subjective distress, values crisis, emotional distur
bances (low mood, anxiety), helplessness, alcohol and
Acute stress reaction. This is pathological re drug abuse.
sponse to severe stress event, usually associated with
threat for survival, such as natural disaster or military Despite specific manifestations of different anxiety
calamity. The reaction supervenes event and lasts for disorders, their common characteristics include irra
hours or days. Manifestation usually include getting tional fears, excessive autonomous reactivity, general
stunned (sometimes to the extent of stupor), autonomic tension, somatic masks, avoidance of daily activities,
crises, panic, flight, anger, despair. impairment of functioning, and subjective discomfort.
EPIDEMIOLOGY AND SIGNIFICANCE Though being severely abnormal states, PDs are not
diseases in the strictest sense. Diseases are usually tem-
Most mental disorders are more common and have poraly demarcated from previous level of functioning,
more severe course when combined with personality while PDs characterize precisely the permanent individ
disorders (PDs). PDs affect around 10% of the general ual style.
population, and the proportion is higher among psychiat
ric patients. Their functioning is associated with higher
disease prevalence, criminal behaviour, disabilities, vio ETIOLOGY AND PATHOGENESIS
lence, unemployment and poverty. Social cost of PDs is
disproportionate to the focus this pathology receives in Personality pathology is final result of interaction
health care. between factors from different areas acting in differ
ent developmental stages. G enetic role in personality
formation is higher for normal traits than for PDs.
CONCEPTUALIZATION OF PERSON Summarized evidence from twin studies show hered
ALITY AND ITS PATHOLOGY itary contribution of between 35 and 50%, with the
reservation that general behavioural tendencies rather
The term personality refers to enduring individual’s than specific traits are genetically transm itted. These
traits manifesting in behaviour in different circumstanc tendencies do not coincide with diagnostic categories,
es. The word stems from per soriae, which denoted ac but reflect genetic links between different conditions
tor’s mask in ancient theatre. Personality encompasses a in the framework of larger spectrum s of pathology,
complex set of characteristics, most of them unconscious e.g. schizphrenia and schizotypal disorder.
and subject to little change in lifespan. Normalcy and
pathology in this area spread on continuum, and the line Biological m arkers also are not specific for dis
of their separataion is strongly influenced by cultural val crete PDs, but are characteristic for wider dimen
ues. Practically, a personality is normal with: a) absence sions, such as impulsivity, aggression, emotional in
of disabling diease; b) favourabe transition through de stability, anxiety, and others.
velopmental phases; c) absence of intolerable conflict or
fight for survival; d) ability to invest feelings and ideas in While biological factors determine disorder itself,
nonmaterial causes; e) skill for irony and self-irony. psychological ones determine its form and display.
According to psychodynam ic approach, conflicts
Despite its complex structure, basic personality com within abnormal personality are to be traced back to
ponents from clinical point of view are temperament early individual experience. Characterological dif
and character. C haracter refers to individual’s habitual ferences in PDs are associated not with the general
facade and his/her ways of interacting with others, while dynamic basis (real or symbolic fear of abandonment)
temperament presents inner trends of reaction that may but with the ways they cope with this threat.
be recognized outside of the context of communication.
Temperament is biologically determined, while character Cognitive theory views personality abnorm ali
to a larger extent depends on psychological factors. ty as learnt response to unfavourable events, which
is repeated and consolidated due to its efficiency to
Personality disorders (PDs) according to ICD-10 rep avoid anxiety, or to fulfil personal goals.The role of
resent deeply ingrained and enduring behavioural models socio-cultural factors is not clear in detail, but there
manifesting with rigid responses to a wide spectrum of per is evidence for influence of culture on personality
sonal and social situations. Onset of PDs is in adolescence, profile, both with pathogenic and protective factors.
with a trend towards ameloiration in older age. Their main
signs are persistence in time and problem functioning in
all spheres: family, work, friends, and others. CLINICAL M ANIFESTATION
PDs are extremities and caricatures of natural human Contemporary classifications are descriptive in
traits. Untrustfulness for instance is prototype of para nature and they divide PDs in discrete categories
noid personality, and shyness - of anxious personality. (Table 12), based on empirical evidence.
33. Personality disorders / 191
Table 12. Personality disorders in ICD-10 and other cla ssifi
es, emotional lability, chronic feeling of emptiness,
cations
stormy and unstable relationships, impulsive self inju
In ICD-10 Others ries. Bordeline personalities are with unstable identity
Paranoid Schizotypal and commonly they do not know who they are and what
Schizoid Narcisistic do they want. They may appear friendly, entertaining,
Dyssocial (antisocial) Passive-aggressive sentimental, or pompous, sarcastic, angry, bored, get
Emotionally unstable: border Depressive ting drunk impulsively and swinging between extrem
line and impulsive subtypes ities. The variety of manifestations attach a pattern of
Histrionic instability and unpredictability to them. Borderline
Anxious personality is vulnerable to affective disorders and
Dependent drug abuse.
Anankastic (obsessive-com
puslive) Histrionc personality. Main traits are grossly
Permanent personality change exaggerated expressivity to the extent of theatricali
Organic ty combined with shallow emotions, suggestibility,
looking for being at the centre of attention, sexual
Major step towards integrative approach to personality provocativeness in behaviour. Display of coquetry and
typology is setting it up in wider entities: eccentric - para vivid emotionality in females are socially acceptable,
noid, schizoid, and schizotypal PDs, dramatic - antisocial, and in most cultures encouraged. In overt abnormality
borderline, histrionic, and narcissistic PDs, and anxious - however these traits transform into their caricatures,
anxious, dependent, and anankastic PDs. subject to thirst for attention. Rapid change of dis
played emotions alternates with rapid recovery from
Paranoid personality. Main traits are suspiciousness, them, leaving the impression of playing. Tantrums of
resentfulness, tendency to accept neutral acts as hostile, rage, crying, pity, or admiration, along with emotional
groundless jealousy, “conspirative” explanations. The pro blackmailing with poses of abandonment or grief are
totype is usually middle aged male, appearing too serious, part of their repertoire. Histrionic personality is vul
secretive and touchy. Paranoid abnormality manifests with nerable to dissociative disorders and depression.
constant suspicion about others’ motives, conviction in one’s
own correctness, inclination for uncovering plots, writing Anankastic (obsessive-compuslive) personality.
reports and for litigation. They are frequently tyrannical and Main traits are doubts, excessive engagement with
live with a sense of mission and striving for power. details, rules, order, perfectionism, over conscien
tiousness, commitment to work, adherence to norms
Schizoid personality. Main traits are emotional cold with obstinacy that others should also adhere to his/
ness, rare experience of pleasure, limited ability to express her performance style. Anankastic individual is ped
feelings, low interest in sex, loneliness, excessive fantasies. ant who sticks to rules and schemes without flexibility
Milder variations may present as unostentatious and coy, and with aspiration for control. The trend for constant
while more severe abnormality is characterized by inacces doubt manifests in weighing “pro” and “con”, procras
sibility, asceticism and indifference to others. They may be tination, mental rumination and general retardation
vulnerable to depression and have nondemanding illness in activity. Anankastic personalities have difficulties
behavior. spending and throwing away unnecessary belongings,
and usually insist on perfect hygiene. They are vulner
Antisocial (dyssocial) personality. Main traits are dis able to depression.
regard to others, lack of respect to norms, low threshold of
aggression, inability for lasting relationships, experience of Anxious (avoidant) personality. Main traits are
guilt or for learning from experience. The prototype is usu permanent tension and apprehensions, unconfidence,
ally male who has tortured animals or children in his child reluctance to connection unless being liked, and avoid
hood, and can easily predispose others and cheat without re ance of communication. Anxious personality bothers
morse. The core of this pathology is not criminal behaviour about being embarrassed and feels uneasy among oth
but emotional deficits, like inability for loyalty and warm ers, this leading to flushing, sweating and excessive
feelings, and lack of any scruples. Traits of impulsivity and drinking. Exaggeration of risks of common activities
novelty seeking are associated with lowered excitability of causes avoiding behaviour, e.g. dating, examination,
sympathetic autonomous nervous system (as a result, anti or favourable offer if unsure. Difficulties in commu
social individual can stay cool even in extreme situations) nication are frequently surmounted at the cost of inner
- one of the few certain biological markers of psychiatric tension and regular doses of alcohol or tranqulizers.
pathology. Antisocial personality is vulnerable to alcohol or Anxious personality is vulnerable to depression, alco
other drug addictions and to affective disorders. hol abuse and anxiety disorders.
Borderline personality. Main traits are insecuri Dependent personality. Main traits are encourag
ty in self-image and in one’s own aims and preferenc ing others to make important decisions about one’s own
192 / Psychiatry
life, excessive conforming to others, wishes, helpless Comorbidity and relationship personality -
ness when alone, obsession by fears of abandonment, disease
limited ability to make decisions without advice or en There is high rate of overlap between PDs them
couragement. Dependent individual is usually viewed selves, e.g. between schizoid and schizotypal. Most
by others as weak-willed and submissive rather than frequent comorbidity of PDs is with depression and
as abnormal, allowing others to decide for him/herself alcohol and drug abuse. PDs also highten generally
and even acting to one’s detriment however with covert suicide behaviour risk. PDs and diseases do not only
benefit and manipulation. Illness behaviour is catching, combine between but they interact in different ways.
ostentatiously helpless and loaded with passive aggres Generally, PDs worsen manifestation and course of
sion. diseases - with earlier onset, higher suicide risk and
complicated course. PDs may also worsen comatic dis
Schizotypal personality. Main traits are odd beliefs eases, such as dermatoses, ischemic heart disease, and
and magic thinking (e.g., superstitions, clairvoyancy, others.
telepathy), reference ideas or suspiciousness, which are
not delusions, odd perceptions, odd speech (e.g., unclear, PDs affect also illness behaviour, or behaviour
metaphorical, overconcrete), narrowed emotions, unusu al aspects of illness beyond disease symptoms: dis
al behaviour and appearance, lack of friends, excessive play of complaints, looking for help, compliance for
anxiety in the presence of others. Schizotypal individual treatment, communication with staff, adherence to
is detached from others, with strange outlook and habits, regime. Abnormal personalities intensify transfer and
lack of intuition, uneven motorics and emotional cold contra-transfer. Attribution of emotions to the clini
ness. Ther is evidence for link between this personality cian with origin in patient’s past personal experience
pathology and schizophrenia in a common spectrum. {transfer), as well as emotional response to the patient
with origin in clinician’s past personal experience
Narcissistic personality. Main traits are grandiose {contra-trasfer) are not pathological in themselves,
feeling about one’s importance, fantasies for success, and are inherent to the therapeutic situation. Patients
power, love, belief in one’s uniqueness, need to be ad with PDs however have the ability to intensify them
mired and treated in special way, lack of empathy, ex in a way that commonly predeterminates outcome of
ploiting attitude, envy and arrogance to others. Narcis treatment. Expression of distress by actions, not by
sistic stance is haughty, demanding, sarcastic and cold words {acting out) is common for all PDs. Problems of
- frequently against the background of inner insecurity. illness behaviour with PDs are reinforced by the gen
In illness, narcissistic personality experiences shame and eral negative attitude of staff to them.
humiliation.
Clinical states include also (Table 13): pathological In cannabionoides (marijuana, hashish, ganja), ef
intoxication, memory gaps („blackouts”) without visible fects of intoxication depend to a large extent on pre
severe intoxication, Korsakoff's (amnestic) syndrome, liminary attitude, but commonly include coughing, in
pathological jealousy, alcohol hallucinosis (psychotic flamed eyes, bright perceptions. There is no overt with
state), Wernicke’s encephalopathy, dementia, person drawal syndrome, although mild transitory anxiety may
ality deteriorartion (residual state). Recently, evidence emerge when substance is discontinued, and cognitive
has been accumulated in support for differentiation be decline may develop with chronic use.
tween 4 types alcoholism in Lesch’s classification, with
distinct pathogenetic routes of development, prevailing In sedatives (benzodiazepines, barbiturates), intox
manifestations and prognosis: type 1 - with powerful ication is characterized by staggering (like drunk), dis
heredity and rapid escalation to dependence syndrome, orientation and nystagmus, while withdrawal is mani
type 2 - “anxyolitic” (self-medication of underlying fested with tremor, vomiting, insomnia, and may be
anxiety), type 3 - “antidepressive” (self-medication of complicated by convulsions, pyrexia and delirium.
underlying depressiveness), type 4 - “organic” (with
early neurodevelopmental problems or deficits and dete In cocaine, intoxication may lead to excitement and
rioration with cognitive decline and other organic stig may be complicated by tactile hallucinations (e.g., for-
mata). micatio, i.e., sensation of crawling under the skin) or
sudden heart arrest, while depression is common when
Severe clinical complications can be usually easily use is interrupted.
recognized. Much more common, and concealed, are
early signs of problem drinking. They are especially In other stim ulants (amphetamines, ice), intoxica
common in general practice, in medical examinations tion manifests with hyperactivity, dry mucoses and an
for other reasons, where significance of early identi orexia, and may be complicated by hyperthermia and
fication is directly associated with the possibility for ischemia, while transitory irritability and anergia may
early interventions. Such early signs are: secrecy and occur when discontinued.
elusiveness, especially if discussing drinking, mild au
tonomous changes (e.g., morning sweating), stereotypi- In haliucinogenes (LSD, mescaline), intoxication
sation of behaviour, covert storing of alcohol, grandios causes perception disturbances, panic, experience
ity, irritability, easy “alcoholic” humour, inclination for of time and space distortion, or distortion of global
cynical remarks, impaired concentration and memory, meaning, twisting of body image, hyperemia, without
sexual dysfunction, unexplainable traumas, gastritis, withdrawal syndrome but sometimes later brief re-ex
hypertension, elevated GGTR periences of intoxication symptoms („flashbacks”) sre
possible.
These signs are usually neglected, not in the last place
due to indulgent and ambivalent attitude of clinicians In phencyclidine (PCP), intoxication causes con
themselves. In hospitals, alcohol deliriums are common fusion, agitation and hypertension, and may be com
ly unintentionally iatrogenicly provoked - by abrupt dis plicated by seizure, rigidity, hyperthermia and stroke,
continuation of drinking and underestimation of warning without withdrawal syndrome though with low mood
signs during the first 1-2 days of hospitalization. Com sometimes, while chronic use causes aggression.
bination of insomnia, somatic disease, lack o f alcohol,
novel surroundings, male gender, and unclear history of In volatile substances (solvents, glue), intoxication
previous drinking increases the risk of delirium. manifests with lisping, staggering, confusion, bron-
____________ 34, Alcohol and drug use disorders / 195
chospasm, hyperemia and specific odour, while chronic vitamine B1 (thiamine), intravenous glucose and other
use causes psycho-organic syndrome. solutions, and anticonvulsants are applied. Alcohol de
lirium is treated as a rule in the hospital unit where it
has emerged. Detoxification in opioid addiction may be
TREATMENT done by different methods: abrupt withdrawal (“cold
turkey”), gradual dose reduction with substitution by
Some of the myths about addictions claim that they are opioid agonists (methadone) or opioid antagonists, and
incurable, withdrawal syndromes are dangerous and untol- others. In sedatives, the approach is gradual dose reduc
erable, and addicts are “victims” and do not bear responsi tion with substitution treatment.
bility. Despite its specifics, treatment of addictions has much
in common with treatment of other mental disorders: need Long-term treatment and rehabilitation programs
of early recognition and intervention, motivation workout, after detoxification include therapeutic communities,
partnership, phase orientation, and continuity of care. pharmacological treatment (methadone, disulfiram,
antidepressants, mood stabilizers), psychotherapy, self-
Detoxification denotes treatment of withdrawal syn help groups, e.g. Alcoholics Anonymous, harm reduc
drome after discontinuation of use. In alcohol withdraw tion programs providing free immunizations, needles,
al (including delirium), high doses benzodiazepines, condoms, etc., health education.
EPIDEMIOLOGY AND SIGNIFICANCE realtively more reliable: it refers to conditions with either
qualitative disturbances in consciousness (delirim ), or with
Acute confusion states (delirium) affect averagely be cognitive decline (dementia). Mechanisms of brain dam
tween 10 and 15% of all hospitalized patients regardless of age are various and depend on the noxious agent (Table
the reason for hospitalization. Dementia affects around 5% 14): hypoxia, blood supply impairment, parenchyma de
of all people above 65 and 30% of those above 85. Men generation, toxicosis, and others.
tal disorders secondary to bodily diseases emerge at some
point of the illness in approximately 25 - 40% of people
hospitalized globally. CLINICAL MANIFESTATION
In developed countries significance of the problem of Three groups of conditions are included here: acute or
dementia increases due to increase of the portion of elderly ganic brain syndromes (deliriums), chronic organic brain
people in general population, along with chronification of syndromes (dementias), and other mental syndromes, sec
most diseases. In developed countries organic conditions ondary to somatic diseases.
caused by exogenous factors, most commonly infectious
agents (e.g., cerebral malaria), are of higher significance. Delirium is always a sign of organic brain impairment,
and may emerge in the phase of decompensation of somat
ic diseases, intoxications and recovery from general anaes
ETIOLOGY AND PATHOGENESIS thesia. Delirium is particularly common in intensive care
units. Patient with delirium is confused, agitated, scared,
This group of diseases is one of the few in psychiatry with visual or tactile illusions and hallucinations, impaired
defined according to their etiology: evidence of brain active attention and sleep (see General psychopathology),
pathology, either with brain origin (organic in the nar and the severe stages of the syndrome (amentia) are char
row sense of the word, e.g. Alzheimer’s disease), or with acterized by extremely clouded consciuosness, incoher
origin in another organ, but affecting also brain (symp ent speech, self disorientation, severely damaged general
tomatic, e.g. hepatic encephalopathy). state, and uneasiness in bed with fidgeting fingers.
The designation “organic” is problematic because bi Alcohol and vascular deliriums are the most common
ological role is assumed for most of the other mental dis ones in general practice. Alcohol delirium (delirium tre
orders also, however on a more discrete, molecular level, mens) emerges as complication of alcohol withdrawal syn
without macroscopic lesions in brain, and without certain drome, frequently in hospitals, where patient is admitted for
markers so far. Clinical meaning of the term “organic” is other reason with subsequent unplanned discontinuation of
196 / Psychiatry
regular alcohol use. The so-called vascular delirium emerg Despite broad spectrum of causes of dementia as syn
es usually in late age, frequently superimposed on dementia, drome (Table 15), the main types in elderly are Alzheim
and after some intercurrent illness or general anaesthesia. er’s and vascular (multi infarct) types. Alzheimer’s type
Patients with dementia may develop delirium by minimal of dementia is characterized by overt memory deficits and
provocation, including change of common surrounding. Le gradual progressive course. In vascular dementia course is
thality of delirium is one of the quality of health care indi more uneven, stepwise, with marked diurnal fluctuations
cators. In developed countries, it does not exceed 10 - 15%. and emotional lability. Depression, irritability, psychotic
symptoms, and apathia may occur in both types of de
Delirium is characterized usually by diurnal dynam mentia. Suicide risk is high, especially when dementia is
ics - milder manifestation during the day, and coarse dis combined with severe somatic diseases and chronic pain.
orientation in the evening. Confabulations may also be Thorough clinical and para-clinical assessment is needed
present. Clinical assessment includes also monitoring of in search for potentially curable causes. Vascular demen
vital signs, neurological examination, and when indicated tia is usually accompanied by hypertension and ischemic
- lumbal puncture, complete blood count with biochemis heart disease, while Alzheimer’s type of dementia occurs
try, hepatic, renal and thyroid functions, urine drug test, usually in good health, or with diseases that have no rela
ECG, CAT of the head, and others. After delirium, recov tion to dementia.
ery may come, however frequently subacute organic brain
syndrome (Wernicke’s encephalopathy), Korsakoff's syn Other psychiatric syndromes secondary to bodily dis
drome or dementia may persist. eases, include psychosis, affective, amnestic, or anxiety
syndrome, sexual dysfunction, and personality change -
Table 14. M ain ca u se s for organic m ental disorders all due to underlying somatic disease.
Deliriums Dementias
Withdrawal Alzheimer’s disease
Vascular Vascular TREATMENT
Intoxication (drugs, carbon Chronic intoxications
monoxide, heavy metals) HIV infection Delirium is life threatening condition and is subject to
Endocrine dysfunctions Parkinson’s disease in-patient treatment, with two major treatment directions:
Hepatic encephalopathy Huntington’s disease symptom amelioration and preserving integrity of brain
Uremic encephalopathy Pick’s disease functions. Excitement is treated by benzodiazepines in
Post-surgical states Creutzfeldt-Jacob’s disease alcohol delirium, and by antipsychotics (e.g., haloperidol)
Defficiency states Neurolues in vascular and postoperative ones. Observation has to be
Electrolyte disbalance Endocrine and metabolic continuous, without change in surroundings and with fo
Systematic infections with diseases cus on safety measures, e.g. patient may be immobilized if
fever and sepsis Chronic hepatic encepha needed. Lights on during the night frequently may reduce
Epilepsy (postictal states) lopathy fears. Vital signs are monitored, metabolite and electro
Brain tumour Chronic uremic encepha lyte deviations are compensated, and vitamins from group
Head trauma (contusion) lopathy B, magnesium salts, and nootropics are frequently added.
Normal tension hydroceph Care about prevention of infections and consequence of
aly being bedridden, is also taken.
Brain tumour
Defficience states (e.g., of In dementia relatives are informed carefully but realis
vitamine B12, folic acid) tically, and in detail, about nature of the illness, needs of
Hepatolenticular degener care, and available programs and services. Re-adjustment
ation of life style is necessary for ensuring safety by accompany
Viral encephalitis ing patient when going out, for help for coping with every
day tasks, monitoring of somatic condition, eating, sleep
Dementia is chronic organic brain syndrome manifest and hygiene. Comorbid or underlying pathology should be
ing with decline of memory and intellect, loss of emotion treated, e.g. with vasodilators. Some medications stimulat
and drive control, and deterioration of habits and social be ing cholinergic neuromediation have moderate efficacy in
haviour (see General psychopathology). It develops usually some dementias. Treatment of behavioural and emotional
in late age, however there are also early onset dementias. disturbances requires caution against sedating medication,
At the beginning, behavioral and emotional disturbances preferably with low doses of antipsychotics rather than
my prevail over cognitive ones. benzodiazepines.
36. Mental disorders in childhood and adolescence / 197
EPIDEMIOLOGY AND SIGNIFICANCE These factors are different in different phases of devel
opment. In early childhood, such factors are intact CNS,
General prevalence of any mental diorder in child available (at least one) figure of attachment, and environ
hood and adolescence is around 15 - 20%. The signif ment providing nurture and food. Insecure attachment for
icance of this pathology is based in its utmost role for instance, reflects in later development of speech and com
future individual development. Children are not small munication - demonstrated in around 80% of children of de
adults and their psychpathology is not direct analogue pressive mothers. Milestone tasks in early age are to achieve
of adult’s psychopathology. They have specific needs in emotional security and control over one’s physiology, later
the different phases of their development. Clinical pic on - ability to play and communicate with peers, fantasy,
tures in this area are understandable only in the light of and motor dexterity, and in adolescence - ability to master
the knowledge what should be normally expected from one’s anxiety of rejection, self-reflexion, nuanced concepts
a child at certain age. What may be pathological for one about onself and others (identity), development of values.
age, is normal for another one. For instance, separation
anxiety during the first days in the kindergarden is fre The influence of heterogeneous factors in different phas
quently normal reaction; in later school years however, es determines whether developmental trajectory is normal
it is a sign of serious emotional problem. or deviant and delineates biopsychosocial framework of
health and illness. This framework includes biological vul
nerability, family, school, cultural expectations and roles, as
ETIOLOGY AND PATHOGNESIS well as nonspecific factors, such as poverty and maltreat
ment, that are influential in all phases and at all levels.
Biological factors have decisive role in retardation
and developmental deviations. They include genetic
anomalies, infectious and toxic agents (rubeola, herpes, CLINICAL MANIFESTATION
HIV, toxoplasmosis, cytomegalovirus, excessive alco
hol use), perinatal hypoxia. Individual clinical mani Clinical states in this age are summarized in Table 15.
festations in children are viewed by the systematic ap Main impairment in mental retardation is arrest of or
proach as inseparable from their connections with outer incomplete development of intellect (as measured by the
world, and by the developmental approach - as product so-called intellectual quotient, IQ), and related different
of interrelationship of different factors. degrees of retardation of motor, speech and social skills.
Depending on severity, only limited set of skills can be Age specifics make lots of phenomena understanda
aquired: some habits for self care and elementary speech ble. For instance, problems in attachment to the mother
in mild form, or understanding of simple instructions, in earliest age results in lack of basic trust, insecurity,
coarse motor skills, frequently with neurologic deficits loss of desire to explore surroundings, ambivalence or
and visible alterations of body and face morphology (in unconfidence, that may affect eating and sleeping. Fears
moderate and severe forms), or very poor understanding of strangers, separation, animals, darkness, etc., are
skills, limited mobility, incontinence, and impossible characteristic for early childhood age, while opposition,
survival without constant care {profound). anger, hyperactivity, poor impulse control - for later
age and adolescence. Psychoses and affective disorders
Developmental disorders can be specific, i.e. with rarely emerge before adolescence, and when they do
relatively isolated disturbances in some cognitive, mo so, they are usually related to developmental disorders.
tor and speech functions (e.g., speech or calculation), Typical features of depression are not characteristic for
or generalized, such as childhood autism or its milder children, but in adolescence its clinical manifestation
version, Asperger’s syndrome. Autism is characterized already resembles adults’ one, and the suicide risk in
by severe impairment of attachment and emotional crease (especially for teens between 15 and 19).
communication, non-participation in games, lack of
eye contact, poor deciphering of nonverbal signals, ab
sorption in inanimate objects and mechanical orders, TREATM ENT
“robotized” speech, stereotypies, odd preoccupations.
Basic principle of all treatment intervention is to
Clinical manifestations of behavioural and emo apply them only after detailed clinical assessment and
tional disoders with onset typical for childhood and wide case formulation accounting for family context.
adolescence, are frequently divided into internalized Differentiation between predisposing, precipitating
and externalized ones. Externalized ones can be di and supporting factors, and their examination and in
rectly observed - aggression, hyperactivity, opposi terpretation in the specific family “ecology” environ
tion, while internalized ones may not be evident, if not ment, is probably much more helpful in this area than in
interrogated about - low mood, generalized fears. Ex adult psychiatry. For instance, no treatment for anxiety
ternalized signs are, in general, behavioural in nature should be prescribed while physical abuse causing anx
and more common in boys, while internalized ones are iety is still going on.
emotional and more common in girls.
Once started, medication treatment has to be applied
Regardless of phenomenology, main criteria for with adequate duration and dosage, with particular cau
whether a behaviour is symptomatic or not, are impair tion in recommending antidepressants to adolescents.
ment of everyday functioning and impact on develop Psychotherapy is considered and adapted to age and phase
mental phase. Thus, brief episodes of anxiety and night of development. Combined approach usually implements
mares after traumatic events may be normal, but they “talking” therapeutic style with medication, e.g. in hy
become problematic if hindering relationship with peers perkinetic disorder with attention deficit. Each treatment
and school attendance. Similarly, in hyperkinetic disor approach has to be weighed against expected benefits,
der with attention deficit, not hyperactivity and attention side effects, and impact on learning and development.
deficit in themselves, but the extent to which they affect
behaviour and concentration in classes, are decisive.
37. Aggression and auto-aggression 199
self-injury to suicide, - is more closely related to mental experience: anger, hopelessness, guilt, anhedonia, and
disorders. Approximately 80% of all suicide attempts others. From the clinical factors, of utmost importance
are preceded by some psychopathological condition, for suicide attempts is depression, and for self-injuries
most frequently - depression. 15% of patients with se - personality disorders. Many people from those that
vere depression and 10% of all patients with schizophre have committed suicide attempts or self-injuries, re
nia end their lifes by suicide. peat their attempts, and one of the most powerful pre
dictors of auto-aggression is previous auto-aggressive
The ratio between attempts and successful suicides act. The role of early recognition and early interven
is 5 : 1, with attempts and self-injuries prevailing in tion is illustrated by evidence that most people com
women, and successful suicides - prevailing in men. mitting suicide attempts, have looked for medical help
Prevalence of suicides varies broadly, with highest or have communicated their intent to somebody else
rates in Belarus (35.1 in 100,000), Lithuania and Rus before the attempts.
sia (WHO data), and with lowest one in some Latin
American and island countries. In Bulgaria, it is 9.5 Table 17. Factors contributing to the risk of auto-aggression
in 100,000, which gravitates to the medium in Europe Biographic factors Family history of suicide
(Eurostat data). There is evidence for increase of the Physical or sexual abuse in
rate with decrease of number of hours of.sunshine childhood
yearly - northwards in the Northern hemisphere, and Previous suicide attempt or
southwards in the Southern one. The ratio between self-injury
men and women in those who have died because of su S o c io -d e m o g ra p h ic Female gender - for attempts,
icide is 3.5 : 1. Suicide is one of the 3 leading causes of factors male - for lethality
death in young people between 15 and 34 years of age. Northern country
The real prevalence of self-injuries is not known, but Adolescent and elderly age
there is data that it is particularly high in developed Alcohol abuse
countries and among adolescents. Mental disorders Depression, mixed affective
episode, schizophrenia, PTSD,
Etiology and pathogenesis. Self-injuries and sui borderline personality disorder,
cide attempts have multifactorial genesis. Family and eating disorders, addictions
biological factors (e.g., some polimorphisms in genes Other diseases Malignant diseases
responsible for serotonine transport) contribute to au- Psycho-social factors Separated, divorced, widowed
toaggressive behavior. Also, depression, sexual abuse Unfavourable life events
in childhood (increasing independently suicide risk 14 Low education
times in boys and 4 times in girls), alcohol abuse, lone Poverty
liness, poverty, poor coping skills, and others (Table
17), play important roles. Autoaggression has not only Treatment behavior. When in doubt about risk,
medical, but existential dimensions also. Besides risk thorough assessment is done by direct discussion of
factors, some protective ones are also known, such as the topic, that is frequently veiled by social taboo. The
social support and religiousness. prejudice that talk itself may push towards committing
suicide attempt is groundless, and incommensurable
Phenomenology and assessment. Autoaggressive with damage if the problem remains unrecognized. It
behaviours lay on a continuum from self-injuries with is clearly shown in practice that people usually share
out any visible signs to lethal methods for suicide (like their suicide intentions. It is relevant to work out some
hanging, jumping from high places, shooting). It is anti-suicide agreement for definite time period, with
imperative to assess intent and its connection to the establishing therapeutic relationship and plan for reg
lethality of methods. Self-injuries are usually not re ular visits during the period.
lated to death intent (parasuicide), however they may
increase suicide risk in the future, particularly when Hospitalization in inpatient facility with safety
repeated. In suicide behaviour, ideas, plan, prepara measures is organized, when the risk is marked and
tion, and execution of attempt are distinguished - with ability for (self-)control is impaired. Such measures
increasing degree of risk severity in this order. are needed also in some emergency and intensive care
units, e.g. video surveillance, temporarily deprivation
Auto-aggression risk assessment has to be made of some personal belongings that may be used for self-
in different clinical situations, and requires estimate harm, etc. Treatment behaviour after suicide attempt
of the risk factors (Table 17), with understanding of is oriented to long-term therapy of the underlying psy
their cumulative action. Assessment is made by in chopathology, to patient’s needs and current monitor
terview focused on thoughts, plan, methods, access ing of suicide risk. From medications, lithium salts and
to weapons, as well as on current stressors and inner clozapine have separate anti-suicidal action.
38. Treatment and rehabilitation of mental disorders/ 201
Some general prerequisists for success of treatm ent Psychopharmacology is the main biological treat
interventions in psychiatry are: ment. Major groups of psychopharmacological agents
• Voluntary way of treatment, informed consent, are presented in Table 18.
and partnership - and based on this, use of coercion as
last choice Antipsychotics are divided into conventional and
• Therapeutic relationship with long-term orien atypical ones.
tation
• Current assessment of aggression and auto-ag- Conventional antipsychotics, called also neurolep
gression risk tics, are efficient in positive psychotic symptoms and
• Complex approach: biological treatment, psy alterations in motor activity, their action being due to
chotherapy, rehabilitation dopamine (mainly D2 receptors) blockage. According
• Phase approach, e.g. active treatment in acute to their major effect they are predominantly sedating
episode, relapse prevention in remission (doses in hundreds of miligrams, e.g. chlorpromazine)
• Coordination with other institutions and potent, predominantly antipsychotic (doses in dec
• Orientation to patients’ needs, not just to symp ades of miligrams, e.g. haloperidol). The predominantly
toms sedating ones have anticholinergic side effects such as
Antidepressants
Tricyclic: amitriptyline, imipramine, Palpitation, dizziness, dryness in
clomipramine mouth, urine retention, sexual dys
SSRIs: fluoxetine, fluvoxamine, paroxetine, Depression function
sertraline, citalopram, escitalopram Anxiety
Others: maprotiline, venlafaxine, milnacip-
ram, trazodone, duloxetine, moclobemide, Headache, nausea, low appetite and
mianserin, agomelatine libido
Mood stabilizers
Lithium salts, valproates, carbamazepine, Affective episodes Lithium: poliuria, tremor, hypothy
lamotrigine Emotional instability roidism; others
202 / Psychiatry
hypotonia, dryness in the mouth, blurred vision. The Mood stabilizers act on temperamental lability,
potent ones have usually extrapyramidal side effects take part in treatment of affective episodes (e.g., by aug
(EPS) such as parkinsonoid, akathisia, dystonia, tardive menting antidepressive response when added to antide
(late) dyskinesias. Another group of side effects are pressants) and serve for relapse prevention in remissions
linked to hyperprolactinemia: galactorrhea, amenor of affective disorders. They include salts of lithium
rhea, gynecomastia, low testosteron. (lithium carbonate) and anticonvulsants, such as carba-
mazepine, valproates and lamotrigine. Lithium has also
Atypical antipsychotics have more varied receptor separate antisuicidal activity, along with however quite
profile as compared to conventionals (operative not only a few side affects that require plasma level monitoring.
on dopamine receptors), and are efficient not only with
positive, but at least partially also with negative symp Benzodiazepines are efficient in alcohol withdrawal
toms. They have less influence on extrapyramidal sys and alcohol delirium. Out of these conditions, benzodi
tem but higher risk of dysmetabolic syndrome: weight azepines do not have separate treatmnent role in psy
gain, hyperlipidemia, type 2 diabetes. There is also a chiatry and are used only as supplements to the main
risk of heart conduct disturbance (with prolongation of treatment. Major risk with them is that of addiction, and
the QT interval) with some of them. Clozapine is agent they are not recommended for long-term use.
of choice in poor treatment response and in aggressive
and auto-aggressive behavior that are not affected oth Side effects of psychopharmacological agents (Table
erwise, and requires white blood cell monitoring be 18) are frequently exggerated and subject to misbeliefs.
cause of moderate risk of aggranulocytosis. For instance, the opinion that they are addictive is very
popular - and it is true only for bezodiazepines which
According to the way of administration there are are not major class for psychiatric treatment. It is imper
oral, parenteral and depot- forms of antipsychotics. ative to differentiate between common side effects, usu
Long acting or depo-forms (of fluphenazine, flupenthix- ally tolerable and not dangerous (e.g., dryness in mouth,
ol, zuclopenthixol, risperidone, olanzapine, aripipra- blurred vision, constipation, tremor, hypotonia, sexual
zole, paliperidone) ensure retarded discharge of medi dysfunctions), from the potentially fatal ones which are
cation for a period of between 2 to 4 weeks after muscle very rare but can be efficiently prevented and treated
application (in the case of penfluridol - for a week after (e.g., malignant neuroleptic syndrome, or aggranulo
oral intake), and thus provide alternative to its everyday cytosis). Usually, tolerance to common side effects de
intake. Psycho-education and therapeutic relationship velops in the course of treatment. Millions of people
are of utmost importance for improving patients’ com worldwide use these agents without serious problems,
pliance to antipsychotic treatment. and besides, for years.
Antidepressants improve symptoms of depression Some disabling side effects, such as tardive dyski
and anxiety, and their action is usually related to block nesias, contribute to the perception of mentally ill with
age of the re-uptake of different neurotransmitters like prejudice (known as stigma) based on outward signs.
serotonine, norepinephrine and dopamine, with conse Part of good clinical practice is foreseeing plausible
quent stimulation of their signals. side effects, their explanation, and conforming regime
of dosing to the risk of them.
Tricyclic antidepressants (imipramine, amitrip
tyline, clomipramine) are older class with lots of side O ther biological methods in treatment of mental
effects such as dizziness, dryness in mouth, urine re disorders are electro-convulsive therapy (ECT), sleep
tention, and sexual dysfunction, however along with deprivation, light therapy, vagus stimulation (stimu
marked effectiveness, especially in severe depression. lation of n.vagus), trans-cranial magnetic stimulation
The selective serotonine re-uptake inhibitors (SSRIs) (TCMS). ECT with anaesthesia and myorelaxation is
(fluoxetine, paroxetine, sertraline, citalopram, escitalo- with unsurpassed efficiency in catatonic syndromes and
pram, fluvoxamine), which are currently widely used, severe depression.
have less side effects, commonly headache, nausea, di
minished appetite and libido. They are especially effi
cient in anxiety disorders. PSYCHOTHERAPY
O ther antidepressants are: tetracyclic (maprotiline, Psychodynamic and cognitive-behavioural therapy
trazodone), reversible inhibitors of the monoaminoox- are the main approaches in contemporary psychothera
idase A (R1MA) (moclobemide), selective serotonine py, and the formats of their application are: individual,
and norepinephrine re-uptake inhibitors (SNRIs) (ven- family, and group.
lafaxine, duloxetine, milnacipram), noradrenergic and
specific serotoninergic (NASSAs) (mirtazapine, mi Psychodynamic methods are oriented to interpre
anserin), melatoninergic (agomelatine). tation of unconscious repeating of past experience in
current acts, as well as in attitude to the therapist. The
38. Treatment and rehabilitation of mental disorders/ 203
therapist facilitates the search for analogies between tionalization. Its aim is to found conditions and to train
conflicts with significant figures in the past and repeat skills allowing for individual with mental disorder to
ing problems afterwards. In the course of this teamwork live independent life (autonomy). Stimulation of an
the patient understands own thoughts and feelings that ti-stigma attitudes in the community and establishing
have been hidden to him/her so far, and can probe new services to substitute hospitals, are needed for its suc
type of communication in secure environment, provided cessful implementation.
by therapeutic relationship.
Modern paradigm of this process is the model of
Cognitive-behavioural therapy is based on the un recovery: achieving meaningful existence and satisfac
derstanding of priority of cognitive processes over emo tion, even if disabled and not totally cured. This model
tional ones, and of behavioural habits over motivation. reinforces natural trends to improvement in two ways:
According to its theoretical background basic distur by developing individual skills and environment’s re
bances are in dysfunctional beliefs and in compensato sources.
ry strategies, related to them, which are put into action
automatically in new events. Therapy aims at changing The components and methods of psycho-social reha
beliefs and behaviours by training in opposing them, bilitation include:
and in behavioural re-adjustment. • Maintenance treatment - prerequisite for pre
vention of worsening
• Sheltered homes - transition between institu
PSYCHO-SOCIAL REHABILITATION tion and independent living
• Day centres - with group activities and train
Rehabilitation is approach for facilitating return to ing in skills for survival, self-control, communication,
a previous way of life after severe illness or after de looking for a job, management of money
tachment from the social environment. Psycho-social • Sheltered job places
rehabilitation is based on progress in treatment of the • Social interventions - advocacy, club activities,
mentally ill and on feasibility of care for most of them support, anti-stigma campaigns, legislative initiaves
outside the hospitals - a process denoted as deinstitu • Crisis interventions.
204 / Psychiatry
abulia - lack of volitional activity, and thence lack uneasy positions after moving them, and after removing
of goals and acts outer support by hands. Waxy flexibility is its manifes
agitation - motor restlessness caused by extreme tation.
anxiety. May escalate to fussy nonproductive excite catatonia - syndrome of disturbed motor and voli
ment with feeling of threat. tional behaviour manifested with: stupor, excitement,
agoraphobia - anxiety provoked by situations in with shifts between them, waxy flexibility, odd posing,
which escape in case of a possible threat may be diffi ambivalence, ambitendency, stereotypies, echolalia,
cult, like going out without a companion, travelling, or echopraxia, imitation, chaotic activity
getting into crowds or full halls. Frequently accompa catatonic excitement - threatening condition with
nies and complicates panic attacks. disorganized motor activity, autonomous disturbances
affect - outwardly manifested emotional response; and destructiveness
intense emotion; in a wider sense, equivalent to emotion catatonic stupor - threatening condition with im
in general mobility, with clear or clouded consciousness, auton
affective flattening - blunted or absent emotions. omous disturbances, waxy flexibility, odd and uneasy
Negative symptom of schizophrenia. poses, negativism, ambitendency, refusal to eat, mutism
akathisia - motor disturbance as a side effect of cognition - system of learning processes related to
some neuroleptics manifesting with uncontrollable brain functions such as intellect, thinking, memory
trampling on in place compliance - intake of drugs as prescribed, or other
akinesia - extremely reduced gesture, expression, kind of cooperation with the treatment, e.g. coming to
and motor activity visits in time
alogia - deficit of logic argumentation and logic compulsion - act that is repeated despite realizing
connections; sign of negative symptomatology in chron its irrationality, with a sense of inner coercion. Usually
ic psychosis accompanies obsessions (e.g., compulsive washing with
ambivalence - contradictory attitudes towards an obsession for contamination), may have ritual mode,
object and is frequently followed by subjective relief.
ambitendency - contradictory behavioural trends, confabulation - memory impairment in which gaps
e.g. holding out and withdrawing one’s hand in the memories are filled in with imaginary events.
amentia - psycho-organic syndrome with qualita Found in Korsakoff’s syndrome.
tive disturbance in consciousness, total disorientation, congruency - degree of correspondence between
incoherent speech and impossible contact. Sign of se experience and affect, e.g. self-confidence in hyperthy-
vere bodily state. mia and pessimism in low mood. It is characteristic of
anhedonia - inability to experience pleasure affective syndromes, while its lack may be found some
antidepressants - medicines for treatment of de times in schizophrenia (incongruent affect).
pression; do not cause addiction consciousness - degree of alertness (vigility) and
antipsychotics - medicines for treatment of psy adequacy of the sensorium. May be disturbed quantita
chotic symptoms. The older generation, called also tively (e.g., somnolence) or qualitatively (e.g., delirium)
neuroleptics or conventional antipsychotics, frequently content - one of the clinical interview dimensions
have extra-pyramidal side effects, while the new gen that is related to description of what is going on (in
eration, called also atypical antipsychotics, often have terms of topics and behaviours) —as compared to the
metabolic side effects. Do not cause addiction. process of the interview
anxiety - universal human reaction to threat; as a contra-transfer - emotional attitude to the patient
disease syndrome, it has psychological (mainly anxious with origin in the clinician’s past personal experience
anticipation), muscular (inability to relax), and autono conversion - psychological defence mechanism in
mous (autonomous excitement) manifestations, all out which commonly joint ideas and emotions are separated
of conscious control resulting in unacceptable drives, feelings or ideas re
autism - detachment from the surroundings (to the maining in the area of the unconsciousness, however be
extent of their total exclusion), as well as from one’s ing “replaced” by pseudo-neurological manifestations
own emotional experience (such as pareses, hypoesthesia, aphonia, etc.)
bradipsychia - retarded thought flow and retarded defence mechanisms - universal psychological pro
mental activity in general cesses which protect individual from becoming aware
catalepsy - motor disturbance in catatonia where of particularly painful experiences by keeping them in
parts of the body remain (or, “freeze”) in unnatural and the area of unconsciousness or by processing them to
_______ 39. Glossary of basic terms in psychiatry / 205
acceptable modes. Most common defence mechanisms without explanation and specific topic. Usually goes
are repression, denial, projection, rationalization, soma before crystallization of delusions at the onset of acute
tization, dissociation. psychosis.
delirium - acute psycho-organic syndrome with delusional perception - unusual interpretation and
qualitative disturbance of consciousness, confusion, finding a special meaning in otherwise perceptually un
visual illusions and hallucinations, fear changed picture of the world; may have the hint of a
delusion (delusional idea) - firm wrong belief which “right guess”
is not shared by others in patient’s subculture, does not dementia - chronic psycho-organic syndrome with
allow considering alternatives, and is not influenced by decline of cognition (predominantly of memory and in
evidence and logical arguments. Although there may be tellect) in clear consciousness
obviously absurd delusions, seemingly plausible ones depersonalization - experience of mental or bodily
do also exist - their morbid nature is based not on their change, e.g. loss of common feelings, or perception of
content but on their incorrigibility and predominance some parts of the body as remote
over what others perceive as reality. Symptom of psy depression - affective syndrome manifesting main
chosis. Kinds of delusions: ly with low mood, anhedonia, retardation, disturbance
secondary - developed as elaborations of oth in appetite, sleep and libido, weight loss, inferiority
er psychopathological phenomena, e.g. delusional feelings
conviction for surveillance with special technique derealization - experience of change in outer world,
based on auditory hallucinations commenting one’s e.g. objects may look alien, strange, with blunted col
behavior ours
depressive - for guilt, sinfulness, poverty, dis dissociation - psychological defence mechanism in
eases (hypochondriac), and similar topics that are which commonly joint ideas and emotions are separat
connected with, and stem from, depressive mood. In ed, resulting in removal of unacceptable drives, memo
nihilistic delusions, there is conviction that parts of ries, or other contents
the organism do not work. drive - biologically determined urge like hunger or
for passivity - conviction that one’s body or ex desire for sex, with increase of its power when not sat
periences are controlled by outer instance, e.g. that isfied. Motivates behaviour, though unconsciously or in
thoughts and behaviour are ruled by outside; they are socially transformed manner.
frequently related to the so-called first-rank symp dysmorphophobia - experience of changed bodi
toms for schizophrenia ly scheme and appearance; may lead to the conviction
megalomaniac - for unusual abilities, talents, of existing deformity and to spending hours in front of
mission or descent. May be overestimation of normal mirror
characteristics and therefore, may look understandable dysphoria - affect of anger; in a wider sense, equiv
(most frequently in mania), or may be totally impossi alent to unpleasant affect in general
ble (e.g. heir of the Pharaohs, or being God’s messen dysthymia - low mood. Symptom of depression.
ger) - in schizophrenia, or in organic psychoses The term is also used to denote mild but chronic de
paranoid - for damage, spying on, surveillance, pressive condition that does not meet criteria for major
poisoning, discreditation, framing up. Common line depressive episode.
in the variety of topics is evil-mindedness against echolalia - imitative repetition of words or phrases.
individual. Specific paranoid variation is reference Catatonic symptom.
delusion: conviction that seemingly neutral state echopraxia - imitative repetition of acts. Catatonic
ments or events refer to the patient, e.g. on the news symptom.
they are hinting about him/her, or the laughter of emotions — known also as feelings; encompass a
strange persons is intended for him/her. domain of mental life that is closely related to drives
prim ary - originating as “finding out” a mean and to the experience of pleasure or displeasure. They
ing at first hand, as inflexible fact which does not express attitude and influence all other psychological
need evidence to be proved; usually anteceded by processes adding certain tint to them. Dominating emo
delusional mood tional background is called mood, while the momentary
systematized - system of delusional convic outwardly expressed emotional response —affect. 1.mo
tions that are interconnected and joint in common tional disturbances may be quantitative (dysthymia, hy
plot with a trend for expansion of topics’ area, e.g. per thymid) or qualitative (parathymia).
strange glance of a stranger may indicate that he has empathy - ability to understand and experience oth
been recruited by conspiracy network following the er’s inner experience
subject, etc. Sophisticated systematization with fan euphoria - inflated affect which is not perceived as
tasy scope is sometimes denoted as paraphrenia - contagious (like hyperthymid) but rather as silly gaiety,
a sign of stability of the delusional system and of with flat joking and cynicism. It is characteristic of or
chronification. ganic states.
delusional mood - intense feeling of perplexity, formal thought disorder - lack of logical connec
bewilderment, hidden meaning, and undefined threat, tion in preserved grammatical structure of speech. In
206 / Psychiatry
its characteristic form, it is relatively rare and is found severe social dysfunction.
in schizophrenia. It has to be differentiated from some negativism - refusal to execute (passive negativism)
difficult to comprehend statements in delusions and in or execution of the opposite (active negativism) of the
abnormal personalities, from accelerated shifts of topics instruction, incidental to catatonia
in mania, and from speech interruptions by hallucina neologism - word that does not exist in the common
tions or anxiety. language
fugue - wandering without any apparent aim, with neurosis - term that historically has encompassed
constricted or affected consciousness, although the in a wide group of non-psychotic mental disorders which
dividual looks ostensibly with reason and in good order in contemporary psychiatric nomenclature are among
incoherence - severe thought incongruity with dis stress related, anxiety, somatoform, and dissociative
integration of both logical links and grammatical struc disorders. Although not currently used in official classi
ture; it is characteristic of severe psycho-organic syn fications, it is frequently used to characterize the under
drome (amentia) and of schizophasia (a rare condition lying mechanism for these states (unresolved intrapsy
in the course of schizophrenia) as a “word salad” chic conflict) or their defence mechanisms.
illusion - distorted perception of real object. Illu obsession - doubt, thought, idea or urge (as com
sions are subdivided according to perception modalities. pared to compulsion which refers to acts), that are per
intellect - ability for constructive use of learnt ceived as distressing, with feeling of inner coercion, and
knowledge in solving new problems may not be controlled consciously
insight - awareness of the morbid character of one’s overvalued idea - an idea dominating one’s thought
own psychopathological manifestations and the pres content and usually defended with attitude, without
ence of illness however being unaccessable to other arguments (like
Korsakoff’s syndrome - psycho-organic syndrome delusion)
with main impairment in fixation memory function, panic attack - short, sudden and intense outburst
with relatively intact intellect, and confabulations of anxiety, with experience of horror, or fear of heart
manierism - act with aim and social meaning (like attack, death or loss of one’s reason, along with autono
salute making), however displayed in a grotesque man mous and somatic signs like shortness of breath, palpi
ner and in inappropriate context tation, sweating, dizziness
mania - affective syndrome manifesting mainly paralogia - odd, improbable, and alien to the ac
with elation, increased energy, initiativeness, libido, ac cepted logic, thought relations. As compared to alogia,
celerated psychic tempo, self-overestimation logic relation is distorted rather than missing. Milder
hallucination - abnormal perception in the absence form ofform al thought disorder.
of real object. Hallucinations are subdivided according paramimia - qualitatively inadequate mimic ex
to perception modalities. Frequent are visual hallucina pression
tions in delirium and auditory hallucinations in schizo parathymia - qualitatively inadequate emotional
phrenia. response. Along with paramimia, it makes expression
hebephrenia - one of the clinical forms of schizo bizarre and unintelligible. Relatively rare symptom in
phrenia with main feature disorganization in behaviour, some forms of schizophrenia.
thought, and emotions, and relatively less conspicuous personality disorder - enduring model of behaviour
positive symptoms, such as delusions and hallucinations which is repeated in different situations and leads to
hyperthymia - elevated (joyful or angry) mood that dysfunction in different areas (e.g., intimacy, work, so
is frequently contagious. Symptom of mania, cial relations). It manifests with combinations between
hypobulia - diminished volition activity character and temperamental anomalies that are qualita
memory - function for storing and, later, retrieving tive deviations from the relative population norms.
information. According to the distance in time from the phobia - unreasonable fear of specific object or sit
event, memory is: immediate (for events before seconds uation; although its irrationality is realized, it may not
to minutes), short-term (for up to few days), and long be overcome willingly when in contact with the object
term (in remote past). or with the situation leading thus to their systematic
mood stabilizers - medicines for stabilizing emo avoidance
tional lability and for prevention of affective episodes. positive symptoms - psychotic symptoms constitut
Do not cause addiction. ing qualitatively new phenomena, products of psychosis
mutism - lack of speech in preserved ability for (as compared to loss of normal functions in negative
talking. Symptom of stupor, dissociative disorder, or or symptoms): delusions, hallucinations, first-rank symp
ganic brain syndrome. In elective mutism lack of speech toms
is selective and related to the circumstances. process —one of the clinical interview dimensions
negative symptoms - inherent of chronic schiz that is related to interchange of emotions, experiences
ophrenia when normal human functions are reduced: and attitudes between participants — as compared to
lowered drives, social withdrawal {autism), blunted content of the interview
affect, poor initiative {hypobulia), poor speech and pseudo-hallucination — perceptual disturbance
thought {alogia), neglected hygiene. Primary cause for without the physical signs and object localization of real
39. Glossary of basic terms in psychiatry / 207
hallucinations, e.g. voices sounding in the head thought blocking - experience of sudden interrup
psycho-motorics - area of motor and mimic activity tion of thought process and “emptying of the head”.
charged with expression and meaning (as compared to Belongs to the so-called first-rank symptoms and
neurological motor activity that is without psychologi frequently causes delusional interpretations, e.g. that
cal content) one’s thoughts are stolen.
psychosis - pathological state in which perception thought broadcasting - experience of detachment
and interpretation of reality or oneself are qualitatively of the thoughts from one’s inner subjective space and
changed as compared to those that are habitual for the their transmission in the distance. May resemble a
individual and his cultural tnvironment. Characteristic physical feeling, or may be compared to radio signal
psychotic symptoms are delusions, hallucinations, and transmission.
grossly disorganized behavior. thought echo experience of repetition of certain
remission - a period in the course of a disease with thoughts shortly (few seconds) after their rise, some
out symptoms times with the characteristics of being spoken “aloud”
rehabilitation - approach for facilitating return to or echoing back
one’s previous way of living after severe illness or de thought insertion - experience of one’s own
tachment from social environment. Aim of the approach thoughts as alien and inserted from outside
is to train skills that may help the individual to function thought process - part of the cognitive human ap
independently. paratus; complex instrument for applying one’s intel
relapse - recurrence of disease symptomatology af lectual abilities in solving problems, revealing links
ter a period of remission and assessing reality. Thought disturbances are: ac
resistance - unconscious attitude of rejection and cording to tempo ((accelerated, retarded)', according to
“sabotage” (of help, interview or treatment), that is con structure (or form) {paralogical, formal thought disor
nected with certain comfort ensured by the status-quo der, incoherent)', and according to content {obsessions,
social phobia - anxiety that is provoked by social overvalued ideas, delusions).
circumstances, where individual may be subject to eval thoughts spoken aloud - experience of one’s own
uation by others, such as public events or meetings thoughts sounding in the head; when severe - with the
stereotypy - simple, repetitive movements without conviction that they may be heard outside of the head
apparent meaning, e.g. rhythmical rocking forwards and transfer - emotional attitude to the clinician with
backwards origin in patient’s past personal experience
stigma - attribution of group labels to the mentally volition - the energy needed for purposeful activ
ill, resulting in prejudices and discrimination against ity: from choice of aim, through contest between pros
them and cons motives, to decision making, and its execu
stupor - total immobility with areactivity and mut tion
ism in relatively clear consciousness. It is found in de waxy flexibility (flexbilitas cerea) - changed
pression, catatonia or dissociative disorder. muscle tone with passive movement of bodily parts
tardive dyskinesias - unintentional movements in causing moderate and even resistance (similar to the
different muscle groups, most frequently in oro-facial feeling when slicing hard yellow cheese) and resting in
ones, as late complication of neuroleptics intake unnatural positions. Catatonic symptom.
208 / Psychiatry
RECOMMENDED READINGS
American Psychatric Association (APA). Diagnostic and statistical manual o f mental disorders, 5lh ed. Arling
ton, VA: American Psychiatric Publishing, 2013
Gelder M, Harrison P, Cowen P. Shorter Oxford textbook o f psychiatry. Oxford: Oxford University Press, 2006
Greenberg M, Shergill SS, Szmukler G, Tantam D. Narratives in psychiatry. London: Jessica Kingsley Publish
ers, 2003
Onchev G, Alexiev S, Yalamova I, Akabalieva K, Ganev K. Tests in psychiatry and psychopathology. Sofia:
ARSO, 2016
Sadock BJ, Sadock VA, Ruiz P. Kaplan and Sadock’s synopsis in psychiatry: behavioural sciences/ clinical
psychiatry, l l ,h ed. Baltimore: Walters Kluer, 2014
WHO. 1CD - 1 0 Casebook. The many faces o f mental disorders: adult case histories according to 1CD-10 (eds.
T.B.Ustun, A.Bertelsen, H.Dilling, et al.). Washington, DC: WHO, American Psychiatric Press, 1996
WHO. Diagnostic and management guidelines fo r mental disorders in primary care: ICD-10 Chapter Vprimary
care version. Seattle, Toronto, Bern, Gottingen: Hogrefe & Huber Publishers, 1996