M e t 6 А.

п-оФ

MINISTRY OF HEALTH OF UKRAINE

NEUROLOGY

Edited by L. SOKOLOVA, M. D., D. Sc.

Professor of Neurology Department of Neurology Bogomolets National Medical University,
Kyiv

Textbook for students of higher medical institutions of IV level of accreditation

Vinnytsia Nova Knyha 2012

 ABBREVIATION
S

АСА — anterior cerebral arteries

ADEM — acute disseminated encephalomyelitis
AF — atrial fibrillation
AIDS — acquired immunodeficiency syndrome
ALS — amyotrophic lateral sclerosis
aPTT — activated partial thromboplastin time
AVMs — arteriovenous malformations
BA — basilar artery
BBB — blood-brain barrier
CA — cerebral arteries
GAG — cytosine-adenine-guanine
CBC — cerebral blood circulation
CBF — cerebral blood flow
CK — creatinekinase
CMTD — Charcot — Marie — Tooth disease
CMV — cytomegalovirus
CNS — central nervous system
CRP — C-reactive protein
CSF — cerebrospinal fluid
GT — computed tomography
СТА — CT angiography
CVD — cerebrovascular disease
DNA — deoxyribonucleic acid
DOPA — dihydroxyphenylalanine
DVT — deep vein thrombosis
DWI — diffusion weighted imaging
EBV — Epstein — Barr virus
EGG — electrocardiogram
ECHO — echocardiography
EDSS — Expanded Disability Status Scale
EEG — electroencephalography
EMG — electromyography
EP — evoked potentials

Abbreviations | 2
EPS — extrapyramidal system
ESO — European Stroke Organisation
ESR —. erythrocyte sedimentation rate
FA — Friedreich's ataxia
FLAIR - fluid-attenuated inversion recovery
GAA — guanine-adenine-adenine sequence
GABA — gamma-aminobutyric acid
GCS — Glasgow Coma Scale
HAART — highly active antiretroviral therapy
HD — Huntington's disease
HDL — high-density lipoprotein
HHV6 — human herpes virus 6
HIV — human immunodeficiency virus
HLA — human leukocyte antigen
HSV — herpes simplex viruses
ICA — internal carotid artery
ICH — intracerebral hemorrhage
1CP — intracranial pressure
IgG — immunglobulin G
INR — international normalized ratio
IQ — intelligence quotient
IVH — intraventricular hemorrhage
LDL — low-density lipoprotein
L-DOPA — L-dihydroxyphenylalanine
LGMD — limb-girdle muscular dystrophies
MAP — mean arterial pressure
MBP — myelin basic protein
MCA — medial cerebral arteries
MD — muscular dystrophies
MR — magnetic resonance
MRA — magnetic resonance angiography
MRl — magnetic resonance imaging
MS — multiple sclerosis
NAA — N-acetylaspartate
NG — nasogastric
PCA — posterior cerebral arteries
PCR — polymerase chain reaction
PD — Parkinson disease
PET — positron emission tomography
PML — progressive multifocal leukoencephalopathy
PNS — peripheral nervous system

Abbievlalions I 3
PP — primary progressive
PT — prothrombin time
PTT — partial thrombin time
PWI — perfusion weighted imaging
RR — relapsing-remitting
rtPA — recombinant tissue plasminogen activator
SAH — subarachnoid hemorrhage
SCA — spinocerebellar ataxias
SMA — spinal muscular atrophies
SMN — survival of motor neurons
SP — secondary progressive
SPECT — single photon emission computed
tomography
TCD — transcranial doppler
TIA — transient ischemic attacks
TOE — transoesophageal echocardiography
TTE — transthoracic echocardiography

VA — vertebral artery
VZV — varicella zoster virus

4 I Abbreviations
1. SENSITIVE FUNCTION AND ITS ABNORMALITY

The human capacity to feel the impact of various exogenous and endogenous factors on his/her
receptor apparatus is called the sensation.
Classification of sensation. There are the general (simple) and complex sensation. The general
sensation with taking into account the localization of receptors are classified as the superficial (skin
and mucous membranes), proprioceptive or deep (muscles, tendons, joints), and interoceptive
(internal organs) sensation.
The pain, temperature (heat and cold) and touch belong to the superficial sensation. The
proprioceptive sensation includes a sense of passive and active movements (proprioception),
vibration, a sense of pressure and mass, a kinesthetic sense — determination of the skin creases
direction. Interceptive sensation is called a sensation that occurs when internal organs and walls of
blood vessels are being irritated. The impulses accepted from internal organs are not sensed in
practice under normal conditions. When the interoreceptors are irritated, pain of varying intensity and
with a sense of discomfort may occur.
Complex kinds: sense of pinprick localization that identifies the place of induced irritation;
stereognosis is the ability to recognize objects by their palpation (touching); two-dimensional space
sense is specified when a patient with the closed eyes can recognize what figure is written on the skin;
discriminations are the ability to perceive separately two irritations being simultaneously dealt at
close range.
Complex kinds of sensation do not have individual analyzers; they are provided with common
kinds of sensation.
Specific sensation is also distinguished; it includes vision, hearing, smell, taste, touch.
All the nerve elements that provide perception, information holding and processing belong to the
sensory systems (from the Latin word 'sensus' — sense) or to the system of analyzers according to I.
P. Pavlov. They perceive and process irritators of different modalities.
Analyzer is a functional system, which consists of receptors, afferent pathways and the applicable
zone of the cortex of the cerebrum. An analyzer provides perception, passage and processing of the
homotypic nerve impulses.
An analyzer's cortex ends are the primary projection zones of the cerebral cortex for which
somatotopic organization is definitive.

Pathways of sensitivity
When an irritator is active, a certain sense occurs in the cerebrum as soon as a nerve impulse
accepted from a receptor on the relevant navigation comes to sensitive areas of the cerebral cortex.
These paths are not identical to superficial and deep sensation kinds, but they are surely
three-neurorial.
Pain and temperature pathway. The first neuron of pathways is featured by the nerve cells of
sensitive spinal ganglion. Peripheral processes (dendrites) of these cells within a spinal nerve, plexus,
and peripheral nerve are going to relevant parts of the skin (dermatomes), where they end with
receptors perceiving irritations. Central processes (axons) of spinal ganglion's cells are going to the
spinal cord and participate in the formation of the dorsal (sensitive) root which enters the spinal cord.
The fibers end with the cells of posterior horns which are bodies of the second neurons of the
pathways of superficial sensation (Fig. 1.1).
The axons of the second neuron in each segment of the spinal cord make an intersection through the
front white commissure and fall into the lateral spinal1. Sensitive
funiculus (unction from
and its abnormality |5
the opposite side by forming
within it the tractus spinothalam- icus that rises up. An intersection in the front white commissure is
not made in a horizontal plane, but under a certain angle, thus, the fibers enter the lateral funiculus at
1-2 segments upper, that is important to determine the level of spinal affection. The tractus
spinothalamicus fibers conducting thermal and painful irritation of the lower limbs are laterally
placed in the lateral funiculus, and the one of the upper limbs are medially placed, i.e. closer to the
syringo- cele (canalis centralis medullae spinalis) (Auerbach's and Flatau's law). It is of practical
importance for the diagnosis of spinal tumors. In the case of extra medullar localization of tumor, pain
and temperature disorders are generalizing bottom-upwards from the opposite side (the bottom-up
 type of sensory disorders), and in the case of intramedullar tumors, sensitive disorders are effusing on
the opposite side upwards-bottom (the top-down type of sensation disorders development).
The tractus spinothalamicus goes from the spinal cord into the brainstem, where along with the
path of the deep sensation types creates a medial lemniscus.

Fig. 1.1. Pathways of sensitivity:

1— postcentral gyrus;
2— internal capsule;
3— thalamus;
4— medial lemniscus;
5— medulla oblongata;
6 — cervical intumescence, lateral funiculus;
7— lumbar intumescence) lateral funiculus;
8— posterior horns;
9— spinal ganglion — 1st neuron of superficial sensation;
10— spinal ganglion — 1st neuron of deep sensation;
11— Goll's and Burdach's nucleuses.

6 | PART 1: General Neurology

 Having passed through the brainstem the axons of the second neurons end in the lateral ventral
intermediate nuclei of the thalamus. The fibers of the third neuron originate from the cells of these
nuclei, which form the tractus thalamo-corticalis. It passes through the back third of the posterior limb
of internal capsule behind the corticospinal tract, then as a part of the corona radiata it reaches the
cortex of the postcentral gyrus. There is a somatotopic arrangement of agents relating the relevant body
parts: the sensation of the lower limbs is in the upper cortex part of this gyrus, the sensation of the trunk
and upper limb — in the middle one, the sensation of the face — in the bottom. An especially large area
of the postcentral gyrus is reserved for innervation of distal paits of the upper limbs and face.
Fibers conducting a touch sensation, for the most part, go along with agents of a deep sensation.
Pathways of deep and most of the touch sensation fibers. The first neuron of pathways is also
presented by nerve cells of a spinal ganglion. Peripheral processes of these cells form sensitive nerve
fibers and end with receptors in muscles, joints; the receptors in the skin and deep tissues perceive touch
irritations. The axons of spinal ganglion cells within dorsal roots are going into the spinal cord.
However, they do not go to the posterior horn, and form on the same side the dorsal funiculus (Fig. 1.1).
Being a part of the dorsal funiculus and forming thin and wedge-shaped fascicles (Goll's fascicles and
Burdach's columns) the fibers by having uninterrupted passage through the spinal cord to the spinal
bulb. Fibers of a thin fascicle are long, they conduct proprioceptive impulses from the lower limbs and
low part of the trunk, lay more medially, and fibers of a wedge-shaped fascicle are shorter, they conduct
proprioceptive sense generated in the pectoral arch and upper limbs and situated more laterally. Axons
of the first neurons end on the body of the second neurons in thin (the Goll's nucleus — nucleus gracilis)
and wedge-shaped nuclei (Burdach's nucleus — the nucleus cuneatus) located in the medulla oblongata.
Axons of the second neurons make up the tractus bulbothalamicus, which being within the cord moves
to the opposite side by making a full intersection, and then along with the tractus spinothalamicus takes
part in forming the medial lemniscus (lemniscus medialis). The medial lemniscus rises up by passing
through the pons varolii, mesencephalon, and ends in the lateral ventral intermediate nucleus of the
thalamus.
Deep sensation third neuron fibers being along with the superficial sensation a part of the tractus
thalamo-corticalis originate from the ventral intermediate nucleuses of thalamus. They pass through the
back third of the posterior limb of the internal capsule, then as a part of the corona radiata crown reach
cortex of the postcentral gyrus. As described above, there -is a somatotopic distribution of agents'
projection in the postcentral gyrus.

Methods of sensitive function study

Exploring a sensation, a doctor gets subjective information from a patient about his/her feelings that
arise during irritation of the receptor apparatus. Therefore, it is necessary to adhere to certain conditions
during the study. The study should be carried out in a quiet atmosphere, in a warm room, with a patient's
eyes closed. Irritations should be inscribed on symmetric sections, have the same force and duration,
they should be conducted with different intervals, compared the sensation of irritants with "sick" and
"healthy" areas.
During delimitation of sensitive disorders, one should be aware of some guidelines: the clavicle's
level is approximately equal to the C3 segment; the level of the nipples is equal to the T5; the level of
the rib arch — to the T7; the level of the navel — to the T10; the inguinal fold level — to the T12
segment.
Study of superficial sensation. Needle is used in order to test pain sensation. A temperature sense is
studied with using test tubes filled with hot and cold water. A Touch sense is investigated with using a
piece of cotton or paper, with which the skin is abruptly touched.
Study of deep sensitivity. The proprioception and vibration are usually studied in clinical practice.
The proprioception is tested by performing the passive1.movements
Sensitive (unction and its abnormality | 7
of small amplitude on small joints
of the hands, and then on the patient's legs, who is lying with the closed eyes. If any disorders of
sensation are found out in small joints (in the distal phalanxes of fingers), it is required to move to
larger. A vibration sense should be checked with a tuning fork, which foots put on the bony
prominences of the limb and define the time during which the patient feels vibration. Normal duration
of a vibration sensation is 14-16 sec.
Study of complex sensitivity. A sense of localization is checked by applying touch irritants to a
patient who should determine the place of touching. Stereo- gnosis or a three-dimensional space sense
is the ability to recognize the familiar objects by palpating them with closed eyes. The disorder of
stereognosis with full conservation of common sensation types is called astereognosis. A two-
dimensional space sense is studied by drawing different numbers, letters and shapes on the patient's
skin with a blunt object, which he or she must determine.

Kinds of sensitive disorders

Quantitative and qualitative kinds of sensitive disorders are distinguished in clinic. Quantitative
kinds include: anesthesia, hypesthesia and hyperesthesia. Anesthesia is total loss of this or another
sensitive kind. There are anesthesia of pain (analgesia), temperature (termanesthesia), proprioceptive
sense (bathyanesthesia). When all sensation kinds disappear, total anesthesia is determined.
Hypesthesia means reducing of sensation. Hyperesthesia means increasing perception of sensation.
Dissociation or split of sensation is called an isolated losing of one sensation kind while other kinds
of it in the same area are kept. Dissociation occurs when the dorsal horns, tr. spinothalamicus and front
white commissures of the spinal cord are affected.
Quality disorders appear as hyperpathia, dysaesthesia, polyesthesia, and synesthesia. Hyperpathia
is the raising of the excitability threshold. Mild irritations are not perceived, and strong are felt
inadequately, painfully and non-locally. Hyperpathia is strongly marked, when the thalamus is
impaired; median and tibial nerves' trunks are traumatically and partially damaged (in the case of
causalgia).
Dysaesthesia is the abnormality of irritation perception when, for example, thermal irritation is felt
as painful or touching provokes a feeling of pain. Polyesthesia occurs when single irritations are
perceived as multiple. Synesthesia is a sense ot irritation not only in the place where the irritator acts,
but also in any other area.
Sensation disorders may also arise independently, without external irritants. First of all, it is
paraesthesia and pain.
Paresthesia is called a sense of numbness, formication, burning, cold or pricking sensations that
occur without outside influences.
Pain. Perception of pain can be caused by lesions of afferent system different levels. A particularly
intense pain occurs when peripheral nerves, dorsal roots of the spinal nerve, ganglion, sensitive roots of
the cerebral nerve and the thalamus are damaged.
Causalgia (burning pain) occurs, when median and tibial nerves' trunks are traumatically and partially
damaged as a result of irritation of sympathetic fibers of the autonomous nervous system being a part of
these nerves.

Types of sensitive disorders

Peripheral, segmental and conductive types of sensitive disorder (Fig. 1.3) are distinguished.
The peripheral type of sensation disorder occurs when peripheral nerves are damaged and is divided
into neural and polyneuritic.
The neural (mononeuritic) type appears when a certain peripheral nerve is damaged (in the case of
neuropathy). The polyneuritic type occurs, if peripheral nerves are multiply impaired in the distal part of
the limbs (in the case of polyneuropathies). Herewith all kinds of sensitivity are impaired in distal parts
of the upper and lower limbs as "gloves" and "socks".
The segmental type of sensitive disorder occurs, if the dorsal roots, posterior horns, front white
commissure and spinal ganglion are affected.
The dorsal-root type of sensation disorder manifests of pain in corresponding segment and all kinds
of sensation disappear on the side of the lesion. If the roots are involved in the forming of the reflex arc,
the relevant reflex decreases or falls out.
The posterior-horn type manifests of pain and temperature sensations out the dermatomes on the side
8 | PART 1: General Neurology
of the lesion solely, while the joint and muscular, touch sensations and vibration remain on that area.
Therefore, this type of sensation disorder is called the segmental dissociated sensation type. It is usually
identified, if syringomielia occurs.
 If the damage is in the front white commissure of the spinal cord, segmental dissociated disorders also
occur, such as analgesia and thermanaestesia disorders. However, a loss of sensation is often bilateral
and symmetrical, because coming from both sides the axons of pain and temperature neurons are
passing and decussating through the front white commissure.
Lesion of the spinal ganglion has similar symptoms with the dorsal-root type and are accompanied by
falling out of all kinds of sensitivity, with pain and paraesthesia with the appearance of herpetic eruption
of relevant segments (herpes zoster) on the skin.
The conductive type of sensitive disorder appears, if the sensitive pathways on the level of the
cerebral and spinal cord (the lateral and posterior funiculars of the spinal cord, medial lemniscus,
thalamus, internal capsule, corona radiata, postcentral gyrus). If cerebral localization of a process takes
place, conductive sensation disorders appear on the opposite side. If the tractus spinothalamicus is
damaged in the lateral funiculars of the spinal cord, analgesia and thermanestesia loss also occur on the
opposite side by the conductive type from the locus to the bottom. In this case, the upper level of a
sensitive disorder is defined at 1-2 segments below from the spinal cord lesions. Pathology of the spinal
posterior funiculus leads to conductive disorders of proprioceptive, touch sensitivities and vibration
appear on the same side starting from the disorder level.
Syndromes ol sensitive disorders
Depending on the localization of pathological process there are peripheral, spinal and brain sensory
disorders.
Peripheral syndromes are associated with lesions of the peripheral nervous system.
1. Neural syndrome occurs when one peripheral nerve is affected. It is characterized by the peripheral
(mono neural) type of sensation disorder in the autonomous innervation zone of this nerve:
anesthesia or hypesthe- sia of all kinds of sensitivity, paresthesia, pain. As the majority of spinal
nerves are mixed, in the case of their damage, along with sensation disorder there are the motor
(peripheral paresis of relevant muscles), vegetative and trophic disturbances.
2. Polyneural syndrome is characterized by multiple damage of peripheral nerve's in distal parts of
limbs. Disorders of all sensation kinds occur symmetrically by the type of "gloves" and "socks".
There is palpatory nerve-trunks soreness, a loss of reflexes, muscle atrophy and vegetative- trophic
disorders.
3. Plexus lesions (cervical, brachial, lumbar or sacral) is characterized by pain, paraesthesiae, falling
out of all sensation kinds, motor and vegetative disorders in the innervation area of those nerves
emerging from this plexus.
4. Radicular syndrome occurs when the spinal dorsal radices are affected and is accompanied by
disorder of all the sensation kinds by the segmental type. These disorders' area is different than the
peripheral nerves are damaged, because the dorsal radix fibers provide innervation of a certain skin
area — dermatome. The radicular or segmental areas of sensitive innervation are on the trunk skin
as circular bands, and as longitudinal in the extremities. The features of radicular disorder are pain
and paraesthesiae in respective segments.
5. Ganglionaiy syndrome occurs when a spinal ganglion is affected and accompanied by girdle pain
in a relevant segment. All sensation kinds by the segmental type are lost in a corresponding
dermatome, and bubble rashes (herpes zoster) appear on the skin.

Spinal syndromes are associated with lesions of the spinal cord. They are distinguished as segmental
and conductive.
Segmental syndromes 1. Sensitive (unction and its abnormality | 9

1. Posterior-horn syndrome is characterized by the segmental dissociated _ disorder type of pain and
temperature while touch and deep sensations are preserved in this area. Disorders are always
determined on the side of affection, and at the damaged segments' level. Moreover, relevant seg-
mental reflexes reduce or fall out. The posterior-horn syndrome most often occurs when
syringomielia takes place; therefore, it is also called a syringomielia syndrome.
2. Syndrome of front white spinal commissure disorder as the prior one is characterized by the
segmental dissociated disorder type of pain and temperature, but its disorders are bilateral and
symmetrical. Reflexes are preserved when process localization at this level.
II. Conductive syndromes
1. Syndrome of lateral funiculus disorder is characterized by the pain and temperature impairment by
the conductive type on the opposite side. The upper anesthesia limit is determined at 1-2 segments
below from the level of the tractus spinothalamictis affection. It appears more often when extra- and
intra-medullar processes of compression genesis are present. The lateral funiculus damage also
provokes motor disorders on the side of the focus, inasmuch as the lateral cerebrospinal (pyramidal)
pathway is affected.
2. Syndrome of spinal posterior funiculus disorderly accompanied by a total or partial loss of
proprioceptive and vibration. Sensitive ataxia occurs which is determined in Rombetg's position
with closed eyes or while walking at dusk and darkness. Deletion of visual control considerably
strengthens sensitive ataxia. It is objected by bathyanesthesia, missing (off the target) in
coordination testing. Posterior funiculus disorders most often appear when tabes dorsalis and
funicular myelosis occur.

Brain syndromes appear in the case of cerebral structures disorder.

1. Alternate hemianesthesia occurs when the oblong brain is damaged. The spinal nucleus of the
trigeminal nerve and tractus spinothalamicus are involved in the pathological process. The segmental
dissociated anesthesia of pain and temperature on the face arises on the side of the focus, and
conductive hemianesthesia of superficial sensitivity kinds occurs on the opposite side.
2. Syndrome of medial lemniscus disorder within the cerebral pons and peduncle is characterized by a
loss of all kinds of sensitivity on the opposite side by the conductive type, i.e. the syndrome of the
"two hemi" occurs: hemianesthesia and sensitive hemiataxia.
3. Syndrome of thalamus disorder appears on the opposite side as hemianesthesia of all sensitivity kinds
and sensitive ataxia due to proprioceptive sensory disorder, and hemianopsia — falling out of the
opposite iiaives of the vision field — takes place. Thus, the syndrome of the "three hemi" occurs:
hemianesthesia, hemiataxia and hemianopsia. Moreover, when the thalamus is affected, thalamic
pain (hemialgia) in the opposite half of the body is typical: burning, unbearable pain rises as a result
of affecting any irritants (emotions, acute sound, or simple touch).
4. Syndrome of internal capsule disorder due to posterior limb.damaging where sensitive fibers pass
from the thalamus to the cortex. There het- erolaterally is a syndrome of the "three hemi":
hemianesthesia, hemiataxia and hemianopsia. If the pyramid tract is involved in the process, another
syndrome of the "three hemi" appears on the opposite side: hemiplegia, hemianesthesia and
hemianopsia.
5. Cerebral syndrome occurs when the postcentral gyrus is affected. There are its function loss
syndrome and the syndrome of gyrus irritation. Due to the large extent of the cortical sensitivity
centers its disorders are only limited to one part of the body (the upper, lower limb or the face), i.e.
conductive anesthesia or hypesthesia appear by monotype on the opposite side. These are the cortical
symptoms of falling out of sensitive function.
Irritation of a postcentral gyrus by the pathological process provokes paresthesia on the opposite side
in the form of seizures in the relevant localization of the pathological focus on body's areas (the sensor
type of Jackson epilepsy). Paresthesia can be extended to the total half of the body and ends up with
common epileptic seizure.

10 | PART 1: General Neurology

Tests and typical tasks

1. Where із the body of the first sensory neuron of all types of sensitivity localized?
a) spinal ganglion
b) Burdach's and Goll's nuclei
c) spinal cord lateral horn
d) spinal cord posterior horn
e) thalamus

2. Where is the end of the first sensitivity neuron of deep sensitivity localized?
a) postcentral gyrus
b) Burdach's and Goll's nuclei
c) thalamus
d) spinal cord posterior horn
e) spinal cord lateral horn

3. In what part of the brain cortex the projection of a hand sensitive innervation is presented?
a) postcentral gyrus upper part
b) precentral gyrus upper part
c) precentral gyrus middle part
d) postcentral gyrus middle part
e) postcentral gyrus lower part

4. What kinds of sensitivity will be impaired if peripheral nerves are damaged multiply?
a) only pain and temperature sense
b) only touch sense
c) all kinds
d) only kinesthesia
e) only vibration sense
5. What structures damage can cause sensitive disorders by the segmental type?
a) spinal cord posterior horn, spinal ganglion
b) peripheral nerve, anterior root
c) postcentral gyrus, internal capsule
d) thalamus, medial loop
e) spinal cord lateral and posterior funicles

6.Patient feels vibration for 3 seconds In all joints of the lower extremities, muscle- joint sensation is
impaired. There are no palsies or other sensitivity disturbances. What structures are damaged?

7. Sensation of pain and temperature sense are absent on the right upper limb and on the right side of
the patient's trunk, the so called "semi-jacket", but sense of touch is preserved in this region.
Kinesthesia and vibration sense are also present. How is such a type of sensitive disorder called?

8. Apatient complains of the inability to find necessary things in his pocket with the help of his right
upper limb. Superficial and deep sensitivity are present. What is the kind of sensitive disorder in this
case?

9.A patient complains of pain, paresthesia, hypesthesia in the left elbow and in the 4 and 5 fingers of
the hand, which appeared after trauma of the left elbow1. Sensitive
joint. (unction
Whatandtype
its abnormality | 11
of sensitive disorders is there?
What is damaged?

10. A patient has lesion of the spinal cord cross-section. What sensitive disorders will this patient
have?
 2. REFLEX-MOTOR FUNCTION OF THE NERVOUS SYSTEM. SYNDROMES OF
MOVEMENT DISORDERS

Reflex actions are the simplest form of movement. A reflex action is a stereotyped response to a
specific sensory stimulus. The reflex elicited depends. on the site of the stimulus and the strength of
the stimulus determines the amplitude of the response. Reflex responses are used by higher motor
centers to generate more complex movements and behaviors. The neural circuitry responsible for
reflex actions is present at different levels of the motor system and disturbances in these reflexes are
important for localizing lesions in the motor system.
All reflexes are divided into unconditioned and conditioned ones. Unconditioned (instinctive,
inborn) reflexes are an inborn motor reaction, phylogenetic old, under cortex regulative influence and
are the basis of conditioned reflexes. Inborn reflexes are closed in the spinal cord, brainstem and basal
ganglia. Conditioned reflexes are closed in the brain cortex and lay the foundation to higher nervous
functions.
Unconditioned reflexes are classified into superficial and deep ones.
Superficial:

cutaneous from mucosa

- abdominal - corneal
- plantar - conjunctival
- cremasteric - pharyngeal
- anal -palatal

Deep:

tendon periosteal

- flexor ulnar - supraorbital

- extensor ulnar - mandibular (jaw)

- knee - brachioradial

- Achilles (ankle) - scapulohumoral

The monosynaptic stretch reflex is the simplest spinal reflex. As the name implies, the reflex muscle
contraction is elicited by lengthening or stretching the muscle.Ло evoke this reflex, the muscle must be
stretched rapidly, which produces a short phasic contraction. Thus, this reflex is termed a phasic stretch
reflex. In the human subject, sudden stretch is produced by tapping a tendon with a reflex hammer.
Another cutaneous reflex of clinical significance is the superficial abdominal reflex. This reflex is
Tests and typical tasks | 12
elicited by stroking the skin of the.abdomen, which causes a reflex contraction of the abdominal muscles
beneath the stimulus. Thus, stroking the upper abdomen causes contraction of the upper abdominal
muscles, whereas stimulation of the lower abdomen causes contraction of the lower abdominal muscles.
Reflexes arc
Afferent nerve fibers conduct the impulses generated by activated receptors to neurons in the central
nervous system, which fire impulses that are then transmitted through efferent nerve fibers to the cells,
muscles, or organs that carry out the reflex response.
The pathway as a whole is known as the reflex arch Reflex arch functions aie: reflex carrying-out,
muscle trophism supplying, muscle tone supporting.
The scheme of reflex arch are sketched out on Fig. 2.1.
2

Fig. 2.1. The reflex arch of the knee-jerk reflex: 1 — sensory fibers of n. femoralis; 2 — ganglion; 3 —
posterior root; 4 — posterior horn; 5 — intermedium neuron; 6 — anterior horn; 7 — anterior root;
8 — motor fibers of n. femoralis

The spinal cord is divided into segments, each giving rise to dorsal and ventral roots, which are
combined to form the spinal nerve. The axons of all the motor neurons located in one spinal segment
leave the spinal cord through one ventral root and continue into the spinal nerves. These spinal nerves
conform to the embryological myotomes. This myotonia! organization can still be seen in the
rostral-caudal distribution of innervation from the cervical and lumbar cord segments innervating the
upper and lower extremities respectively.

The levels of the spinal reflex arches locking

► Biceps — flexor ulnar (C5-C6 segments),
► Brachioradialis — carporadial (C5-C8 segments),
► Triceps — extensor ulnar (C7-C8 segments),
► Abdominal (T7-T12 segments),
► Cremasteric (L1-L2 segments),
► Quadriceps — knee (-jerk) (L2/3-L4 segments),
► Plantar (L5-S1 segments),
► Achilles (S1-S2 segments),
► Anal wink (S3-S5 segments).

Methods of the inborn reflexes study

To master the methods of the inborn reflexes study fulfill the reflexes study in the following
sequence. The hammer strikes are performed with equal force. Pay attention if the below mentioned
normal reaction is achieved:
► corneal reflex—a carefully touch of the cornea above the iris (not above the pupil) with a soft
paper stripe leads to lids closing
► pharyngeal reflex— a touch of the posterior pharyngeal wall with a spatula leads to swallowing
or coughing movements occur,
2. Reflex-motoi (unction of Hie nervous system. Syndromes of movement disordeis | 13
► palatal reflex— touch the soft palate with a spatula leads to the soft palate elevation,
► mandibular (jaw) reflex— strike your index finger put on the patient's mandible with a hammer
(mouth is half-open) leads to the mandible elevation,
► flexor ulnar (Biceps) reflex — half-bended arms are placed on the patient's abdomen. Press the
arm biceps muscle tendon with your left pollex. Strike your pollex nail with a hammer — as a result
forearm flexion will appear,
 ► extensor ulnar (Triceps) reflex — a patient's arm is bent under an obtuse angle. Strike the arm
triceps muscle tendon (2 cm above the ulnar process) with a hammer — as a result forearm
extension will appear,
► brachioradial (carporadial) reflex— patient's arms are bent in the ulnar joint under an obtuse
angle, they are half-proned and placed on the abdomen. Strike the radius styloid process with a
hammer and arm flexion in the ulnar joint, fingers pronation and flexion will occur,
► abdominal reflexes — make a quick hatched irritation of the abdominal skin with a pointed
object from the peripheral to the middle lower the costal arches (superior), on the umbilical level
(middle), above the fallopian ligament and abdominal wall muscles contraction will occur,
► knee (Quadriceps) reflex — a patient's legs are half-bent in his knee joints. Place your left arm
under the patient's joints. Strike the thigh quadrate muscle tendon under the kneecap with a hammer
— as a result legs extension in the knee joint will appear,
► Achilles reflex— a patient's leg is bent in the hip and knee joints. Strike the Achilles tendon with
a hammer and foot plantar extension will occur,
► plantar reflex — perform a hatched skin irritation of the sole external edge with a blunt object
and toes plantar flexion will occur.

An interruption of the reflex arches at any point weakens or abolishes the reflex. Reflexes changes:
► areflexia — absence of reflex,
► hyporeflexia — decrease of reflex,
► hyperreflexia — increase of reflex,
► anisoreflexia -— different expression of symmetric reflexes.

Patlioloyical reflexes
Some reflexes, especially spinal and brainstem reflexes, are normally observed or elicited only in
the developing nervous system. As the nervous system and higher motor centers get mature, these
reflexes are suppressed, only to reemerge if damage of the higher motor centers modulates the reflex.
Reflexes that can he elicited only in the diseased state are called pathological reflexes. It indicates
dysfunction of the pyramidal (corticospinal) tract.

Pathological reflexes on feet

extensor flexor

- Oppenheim - Zhukovski’s

- Gordon - Rossolimo

- Babinski’s - Bechterew

- Shaffer

- Chaddock

Oral automatism reflexes:

> nasal-lip (nasolabial),
14 | PART 1: General Neurology
> lip,
> palmar-chin (palmomental),
> distant-oral.

Pathologic synkinesises:
► global,
► imitative,
► coordinative.
 Defense (protective) reflexes (withdrawal leg).

Methods of pathological reflexes study

Exploring the extensor group foot pathological reflexes:
► Babinski reflex— make a hatched skin irritation of the plantar external edge, slow hallux (great
toe) extension with a flaccid separation of other toes will occur,
► Oppenheim reflex— same response to a downward stroke of the examiner's thumb on the
patient's shin,
► Gordon reflex— the same response to squeezing of the calf muscles
► Sheffer's reflex— press the Achilles tendon, slow hallux extension with a flaccid separation of
other toes will occur.
Exploring the flexor group foot pathological reflexes:
► Rossolimo reflex— strike easily with your fingers on the plantar surface of the terminal
phalanges of the patient's ll-IV toes, quick plantar flexion of the toes will occur,
• Becliterew reflex— strike with a hammer on the dorsal foot above lll-IV metatarsal bones, quick
plantar flexion of toes will occur,
► Zhukovski reflex — strike with a hammer on the sole under the toes, quick plantar flexion of the
toes will occur.
Exploring the oral automatism:
► nasal-lip reflex— strike easily with a hammer on the nose root, the lips are stretched ahead,
► lip reflex— strike easily with a hammer on the lips, the lips are stretched ahead,
► palmar-chin reflex— make a hatched irritation of the palmar skin over the thenar, chin muscles
at the same side are contracted.
Voluntary motor functions are realized through the pyramidal system —
a complex of cells which connect primarily to the cortical motor areas cortex and segmental apparatus
of the spinal cord and brainstem. The functions of this system are:
► movements ensuring;
► segmental apparatus regulation (activation of a-motoneurones and breaking the activity of reflex
arches);
breaking reflex automatism of subcortical, brainstem and spinal levels.
The cortex motor centers are located anterior to the central sulcus, in the frontal lobe and include
precentral gyrus, paracentral lobule, premotor area and prefrontal area (the frontal pole). The last one
supports the integration of all informational signals and forms a motor act model. The premotor area
establishes connection with the extrapyramidal system and cerebellum. The precentral gyrus
(Brodmann area 4) is the primary motor area. The corticospinal tract is composed of axons of pyramidal
neurons iocated in layer 5 of the cerebral cortex mostly in the precentral gyrus. Layer 5 in the primary
motor cortex contains distinctive giant pyramidal neurons known as Betz cells — the upper motor
neuron (the central neuron). The axons of these cells become the corticonuclear or pyramidal tract and
represent one of several descending influences on the motor neurons of the brainstem and spinal cord.
The motor neurons in the primary motor area have a somatotopic organization. The homunculus was
mapped by Penfield and colleagues (Fig.2.2).

2. Reflex-motoi (unction of Hie nervous system. Syndromes of movement disordeis | 15

 /
Fig. 2.2. A somatotopic organization of the motor cortex

The axons of the corticospinal tract gather together, forming the corona radiata, and descend
through the posterior limb of the internal capsule into the cerebral peduncles of the midbrain. In the
brainstem corticonuclear tract comes to the end. In the pons the corticospinal fibers form bundles
interspersed with a variety of other descending and crossing white matter tracts. When the fibers
enter the medulla, they form a very discrete, easily recognizable bundle on the ventral surface of the
medulla known as the pyramids, therefore the corticospinal tract is often referred to as the pyramidal
tract.
At the bottom of the medulla, most of the corticospinal fibers cross the midline in the decussation of
pyramids and continue in the lateral funiculus of • the spinal cord as the lateral corticospinal tract. Most
rior corticospinal tract. Some of them cross the midline in the anterior spinal commissure only once they
reach the level of their target motor neurons. The lateral and ventral corticospinal axons terminate in
motor neurons of anterior horn, where the lower motor neuron (peripheral neuron) is presented. The
lateral corticospinal tract is involved in the movement of the contralateral extremities, whereas the
anterior corticospinal one innervates the paravertebral muscles involved in the posture: the neck, trunk
and perineum on two (both) sides.
Corticonuclear fibers destined for the motor nuclei of the cranial nerves leave the corticospinal tract
in the brainstem. Muscles of the head, except for the lower facial muscles (VII cranial nerve) and tongue
(XII cranial nerve), receive both crossed and uncrossed corticonuclear fibers. Therefore, as a rule, in a
patient with a lesion of the corticonuclear tract on one side, one seldom sees significant weakness of the
jaw, pharynx, or larynx. The motor nuclei of cranial nerves VII (lower portion) and XII receive
contralateral cortical innervation only.
Methods of motor function study
A doctor carries out an examination in such a sequence. At first it is necessary to exam the volume of
active movements in the limbs, including Barre's test (upper and lower), the "Budda" posture, the
examination should be started with big joints to small ones. The muscle strength of the limbs is
estimated by five point score: 5 — normal, 0 — absence of the muscle strength.
When examination of passive movements in limbs is performed the doctor estimates the muscular
tonus of the limbs, defines the presence of atrophy and fibrillar twitches.
Methods of the physiological and pathological reflexes exploration were described above.
16 | PART 1: General Neurology
Remember that pathological reflexes of the extension group are physiological for children under one.
After examination the character of paralyses on the basis of the revealed pathological symptoms the
topical diagnosis established.
Impairment of motor function
The interruption of the pyramidal tract causes paralysis of the corresponding muscles. Paralysis
(palsy, plegia) means the absence of movement. The partial interruption of the pyramidal tract causes
paresis — limitation of movement, incomplete paralysis. Paralysis of one limb is called monoplegia,
two limbs' paralysis on one side — hemiplegia, two symmetric limbs' paralysis — upper or lower
paraplegia, four limbs' paralysis — tetraplegia.
Depending on damaged motoneurons there are classified central or peripheral paralysis. Central
palsy occurs when the upper motor neuron (central neuron) is affected. Peripheral — if the lower motor
neuron (peripheral neuron) is damaged. The upper motor neuron syndrome is marked by weakness,
an increased muscle tone — spasticity, hyperactivity of the tendon reflexes — hyperreflexia, and the
presence of the pathological reflex (Babinski sign). This pattern of motor disturbance occurs when
there is an interruption of descending projections from the motor neurons in the cerebral cortex and
brainstem that modulate excitation of the alpha and gamma motor neurons. "The higher nejvous system
arrangements inhibits (or controls) the lower, and thus, when the higher are suddenly rendered
functionless, the lower rise in activity" J. H. Jackson,
With hyperreflexia of the periosteal and tendon reflexes there will be enlargement of reflex zones,
the knee and foot clonuses appear, abdominal and plantar reflexes are diminished or absent. The
difference in tone in the arm and the leg extensors can also give rise to spastic seizure, or the clasped
knife phenomenon, the process that has been compared to opening the blade of a clasped knife.
The upper motor neuron syndrome may lead to gait changes: paraspastic gait, leg circumduction,
spastic-ataxic gait, Wernicke — Mann gait.
Diseases of the lower motor neuron may affect the cell body in the anterior spinal horn itself or its
axon in the spinal root and peripheral nerve. Signs of disease of the lower motor neuron — the
peripheral palsy features — include muscular weakness, diminition of muscle tone (flaccidity), atrophy,
fascicula- tions, and loss of tendon reflexes.
Fasciculalions or fibrillar muscular twitching are a feature of disorders of the anterior horn cell or
root compression; therefore fasciculations are often viewed as an ominous sign. Forcible contraction or
percussion of the muscle may increase the frequency of fasciculations. In thin elderly men with sus-
pected amyotrophic lateral sclerosis, the shoulder girdle and pectoral muscles are often a good place to
look for fasciculations or fibrillation. In patients with more subcutaneous adipose tissue, the first dorsal
interosseous muscle of the hand is better.
The lower motor neuron syndrome may lead to gait changes such as steppage gait (foot-drop gait).

Syndromes of motor tract damage in different levels

Motor trad cerebral lesions
Precentral gyrus lesion:
> function loss syndrome — central monoparesis on the opposite lesion's side,
> irritation syndrome — motor jacksonian epilepsy.
Internal capsule lesion gives central hemiparesis with central mimic muscles and tongue paresis,
hemianesthesia of all sensitivity kinds, hemianopsia on the opposite lesion's side. Very often Wernicke
— Mann's position will occur: the arm is flexed at the elbow and wrist and adducted against the chest
while the leg is stiffly extended and the foot is inverted and flexed in a plantar direction. Spasticity in
the adductors of the leg leads to the tendency of the hemiplegic leg to "scissor" over the healthy leg.
Brainstem lesion leads to crossed paralysis (alternated): ipsilateral symptoms of cranial nerve
nucleus lesion and central hemiparesis of contralateral extremities.

Spinal cord lesions

Over cervical intumescence (C1-C4) lesion leads to central tetraparesis, conductive impairment of all
2. Reflex-motoi (unction of Hie nervous system. Syndromes of movement disordeis | 17
sensitivity kinds and pelvic disorders.
Cervical intumescence (C5-Th1) lesion gives peripheral paraparalysis in arms, central paraparalysis
in legs with conductive impairments of all sensitivity kinds and pelvic disorders.
Thoracic part of the spinal cord (Th3-Th12) lesion leads to inferior central paraparalysis with
conductive impairments of all sensitivity kinds and pelvic disorders.
Lumbar intumescence (L1-S2) lesion leads to inferior peripheral paraparesis with conductive
impairments of all sensitivity kinds and pelvic disorders.
 Half of the spinal cord diameter lesion gives Brown-Sequard syndrome: movement impairments and
deep sensitivity disorders on the lesion side, conductive impairments of superficial sensitivity on the
opposite side.
Spinal cord anterior corn lesion leads to segmental peripheral paresises with muscles fibrillation
without sensitive disorders.
Medullary cone (S3-S5) lesion does not give paresises, sensitivity is impaired in the perineum, true
enuresis will occur.

Peripheral nervous system lesions

Anterior radix lesion leads to segmental peripheral paresises without sensitive impairments,
fascicular twitches can be observed.
Peripheral nerve lesion leads to peripheral paresises with sensitive disorders in the nerve
innervation area.

Tests and typical tasks

1. Where are unconditioned reflexes locked?
a) in the limbic system
b) in the cerebellum
c) in the segmental part of the spinal cord
d) in the cerebral cortex
e) in the thalamus

2. Indicate the deep periosteal reflex:

a) knee
b) corneal
c) plantar
d) biceps
e) supraorbital

3. Indicate the pathological foot reflexes of the extensor group:

a) Zhukovski's reflex
b) palmar-chin reflex
c) Oppenheim reflex
d) Bechterew reflex
e) Rossolimo's reflex

4. Point to the signs of the central palsies:

a) areflexia, muscle atonia
b) muscle hypertonus, pathological reflexes
c) muscle atrophy, deep reflexes hyperreflexia
d) deep reflexes areflexia, muscle atrophy
e) hyperesthesia, hyperpathia

5. Point to signs of the peripheral palsies:

a) pathological reflexes
b) areflexia, muscle atonia

18 | PART 1: General Neurology

 c) deep reflexes hyperi eflexia
d) muscle hypertonus, oral automatism
e) hypoesthesia, anesthesia
6. A patient after his arm trauma doesn't have right extensor ulnar reflex. What nerve is damaged? -
7.A patient with hyporeflexion has weakness of the right hand and low muscular tonus. What
pathology has the patient got?
8 A patient who has had stroke doesn't have active movements in the left limbs The muscular tonus
and
t ' ' reflexes are
'. V'increased.
, What is this dysfunction?
9. A patient has peripheral paresis of the arms and central paresis of the legs. Indicate the location of
the process.
10. A patient suffers from attacks of clonic convulsions in the right foot, which last during a minute.
How is the attack mentioned called? What is affected?

3. THE EXTRAPYRAMIDAL SYSTEM AND SYNDROMES OF ITS LESION

The movements are formed as a result of consequent functioning of separate neurons of the
pyramidal tract and of a great complex of nervous structures outside the pyramidal system that are
united into the extrapyramidal system. This functioning coordinates the strength and duration of
movements.
When an action is performed voluntarily, a person doesn't keep in his conscious memory the work
scheme of the consequence of motor acts. A change of some muscular contractions with others is
automatic. These motor automations are provided by the activity of the extrapyramidal system (EPS).
The main functions of the extrapyramidal system are: regulation of the muscular tonus, preparing
muscles for action, creation preliminary conditions for performing motor acts, maintaining the
posture, providing motor expressions of emotions, creation an individual expression of movements,
responsibility of automatic, stereotypic and reflector protecting movements.
The main structural anatomical levels are:
1. Cortical — the premotoric gyrus of the cortex (field 5.6 on Brodman);
2. Subcortical
► the nucleus caudatus,
► the nucleus lenticular is ( putamen, globus pallidus)
3. Brainstem
► the substatia nigra, red nucleus, Darcshevich's nuclei, inferior olivas, vestibular nuclei,
formatia reticularis, Lues' body (subthalamic nucleus), lower olives.
4. The spinal level descending tracts and structures of the spinal cord:
► the tractus reticulo-spinalis, tractus vestibulo-spinalis, tractus rubrospinal, tractus
tecto-spinalis, tractus olivo-spinalis. The spinal cord: gamma-motor neurons and a-small motor
neurons, which are located in the anterior horns of the spinal cord.
There are two parts of the extrapyramidal system:
► the pallidal system — the globus pallidus, substantia nigra, red nucleus, Darkschevich's nuclei,
inferior olivas, vestibular nuclei, formatia reticularis, Lues' body (subthalamic nucleus), lower
olives;
► the striatic system — the cerebral cortex, caudate nucleus, putamen.
The striatic and pallidal systems coordinate functioning and they are united into the striatic-pallidal
system. The pallidal structures are responsible for the increase of movements (activating), the striatic
structures — for their decrease inhibition3. Hie(Fig. 2.1)system and syndromes of its lesion | 19
extrapyramidal
The biochemistry of neurotransmission is basic to a pathophysiological understanding of
extrapyramidal disease. Dopamine is the neurotransmitter used by nigrostriatal neurons, which exerts
an inhibitory influence on the cholinergic interneurons of the striatum. Gamma-aminobutyric acid
(GABA) is the neurotransmitter of the inhibitory striatopallidal pathway. The pars compacta of the
substatia nigra contains about 80 % of the total dopamine content of the brain in healthy individuals, but
is severely depleted of dopamine in patients with Parkinson's disease.
There are two main syndromes of the extrapyramidal system's lesion:
 1. Hypertonic-hypokinetic syndrome or syndrome of parkinsonism;
2. Hypotonic-hyperkinetic syndrome or syndrome of involuntary movements (hyperkynesis).
In 1817 the English doctor James Parkinson was the first who described the major manifestation of
this syndrome and this disease was called Parkinson's disease. In 1920 Tretiakov noticed that the greater
is a cell loss in the substantia nigra, the lower concentration of dopamine is in the striatum.
Now there are two forms of parkinsonism: primary and secondary. Primary parkinsonism (94-96 %)
is named Parkinson's disease (idiopatic parkinsonism).

Fig. 2.1. The structures of the extrapyramidal

system:
1 — putamen;
2 — globus pallidus dorsalis;
3 — globus pallidus ventraiis;
4 — nucleus caudatus;
5 — substatia nigra;
6 — Lues' body;
7 — upper hill;
8 — vestibular nucleus;
9 — thalamus

Secondary parkinsonism: postencephalitic, vascular, toxic, post-traumatic, drug-induced, oncologic

are seldom.

Pathology
A loss of melanin-containing neurons in the substatia nigra with glial proliferation is the pathological
cause of Parkinson's disease.
Lesion of the substantia nigra and degeneration of the nigrostriatal pathway cause the reduction of
dopamine's synthesis and its amount. As the result the influence of dopamine on the nucleus caudatus
is decreased that leads to the increasing of cholinergic activity, counterinhibition of the nucleus
caudatus and increase of its inhibitory influence on the motor activity. Clinically it is manifested as
hypertonic-hypokinetic syndrome (Parkinson's desease).

Hypertonic-hypokinetic (parkinsonism syndrome)

It arises due to lesion of the substantia nigra and nigrostriatal tract. The typical triad of
symptoms of Parkinson's disease:
► muscular rigidity
► hypokinesia (akinesia)
► tremor
The main pathogenetic mechanisms of parkinsonism are:
1. The degeneration of dopamine neurons is in the substantia nigra, 70- 80 % cell loss in it, a low
concentration of dopamine in the striatum, the influence of the striatum on pallidum. As a result
akinesia occurs.
2. The rigidity is a result of increasing of the tonic reflex on muscles tension.
3. In the ventrolateral nucleus of the thalamus were detected neurons with frequency of firing that
corresponds 20to| PART
the 1:rhythm of tremor.
General Neurology

Muscular rigidity
Hypertonia of the extrapyramidal type manifests itself mainly as parkinsonian rigidity, as
exaggerated, viscous, waxy resistance to passive movement that can be felt by the examiner during the
entire movement. Testing of the muscle tone often reveals the so-called cogwheel phenomenon: the
examiner, applying passive movement across a joint, feels a varying resistance, as if the two opposing
joint surfaces were composed of cogwheels repeatedly engaging and disengaging with each other.
Hypokinesia (achinesia)
Impairment of primary automatic movement is the most impressive manifestation and sooner or later
becomes prominent in all parkinsonian patients. There is a generalized reduction of spontaneous
movement called hypokinesia or akinesia.
Hypomimia is the characteristic mask-like face as a result of akinesia. Sometimes a patient has
unwinking eyelids (look of a python). Blinking is less frequent than normal. There is a "flexor muscles"
posture (mannequin's posture) (Fig. 3.2).

Tremor — is the most impressive, though by no means an obligatory involuntary movement in

Parkinson's disease. Parkinsonian tremor is practically always most prominent at rest and is regular,
rhythmic, mainly distal tremor, with the frequency of 4-8 Hz and variable intensity, that decreases or
disappears on voluntary movement. Characteristic "swallowing of pills", "counting of coins"
movements of the fingers may be seen. It decreases or disappears while moving or sleeping,
increases when a person is agitated.
In a clinical picture of a Parkinson's patient the additional sings may be bra- dilalia (monotonous
slow speech), acheirokinesis (the absence of coordinated movements while-walking), micrographia
(paucity of movement causes reduction in the size of the patient's handwriting), impairment of the
movement starting process, pro- and retropulsion are the inability of the patient to regain his or her
balance when briskly pushed forward by the examiner.
These basic signs of Parkinson's disease sometimes may be combined with psychic disorders:
achaii ia (while speaking such patients are boring), bradypsychia (slow thinking). In 50-60 % cases
in Parkinson's patients we can define depression
Besides, in a clinical picture of a Parkinson's patient there may be vegetative reactions:
hypersalivation, hyperhidrosis, constipation and greasy face seldom.

Hypotonic-hyperkinetic syndrom
Arises due to lesion of the striatic system. Combination of muscular hypotonia with involuntary
movements (hyperkinesias). Hyperkinesias always disappear while sleeping
The main kinds of hyperkinesias:
► chorea
► athetosis
► hemiballism
► myoclonus
3. Hie extrapyramidal system and syndromes of its lesion | 21
► torsion dystonia
► tics
► hemispasm
 Chorea is characterized by fast polymorphic movements, non-stereotypic chaotic involuntary
movements in different muscular groups against the background of the low muscular tonus. It is
usually more pronounced in distal segments of the extremities. When severe, however, they may be
of a very high amplitude, randomly directed and extremely disturbing. Grimacing and lip-smacking
may be prominent.
Athetosis consists of slow, irregular, exaggerated, uncomfortable- and cramped-appearing
involuntary movements that are more pronounced in distal portions of the extremities. It is snakelike
movement of any combination of flexion, extension, adduction and abduction in varying degrees.

Hemibalism — lateral swinging movements of proximal parts of the extremities. These disorders
are characterized by lighthing-like, high-amplitude, flinging ("ballistic") movements simultaneously
involving multiple segments of a limb. It is similar to "wingbeat".

Tics are stereotypic hyperkinesias of the face and upper shoulder girdle muscles, which remind
voluntary movements (winking, neck, shoulder, head twitching), but never prevent voluntary
movements.

Torsion dystonia (lat. torsjo — twisting) — corkscrew-like movements of the body, neck and
pelvic girdle muscles.

Spastic curvature of the neck — a local form of torsion dystonia.

Hemispasm of the face — rhythmic twitching of half face muscles.
Paraspasm of the face — bilateral twitching of face muscles.
Writing spasm — reminds "an obstetrician's hand".

Tests and typical tasks

1. Describe physiological functions of the extrapyramidal system.
a) realization of automatic movements, muscle tone support
b) realization of conditioned reflexes, coordination
c) realization of voluntary movements, constriction of smooth muscles
d) constriction of striated muscles, realization of voluntary movements
e) constriction of striated muscles, function of equilibrium

2. Choose the symptoms of pallidar system impairment

a) low muscular tone, ataxia
b) peripheral paresis of the extremities, a low muscular tone
c) bradykinesia, micrographia, a high muscular tone
d) a low muscular tone, hyperkinesis
e) central paresis, hyperkinesis

3. Choose the symptoms of striatic system impairment

a) high muscular tone, propulsions
b) amimia, monotonous speech
c) propulsions, amimia
d) a low muscular tone, hyperkinesia
e) monotonous speech, bradykinesia

4. Choose the symptoms of Parkinson disease

a) central paresis of the extremities, hyperkinesis
b) propulsions, monotonous speech
c) the upper muscular tone, peripheral paresis of the extremities
d) central paresis of the extremities, hyperkinesis
e) peripheral paresis of the extremities, hyperkinesis

5. Choose extrapyramidal hyperkinesis

 a) intention tremor, motor jackson epilepsy
b) epileptiform convulsions, functional tremor
c) epileptiform convulsions, propulsions
d) motor jackson epilepsy, functional tremor
e) athetosus, chorea

6. The patient has hypomimia, slow movements, an increased muscular tonus of the plastic type,
tremor. Name the pathological syndrome. Which structures are impaired?

7.The child has quick, swinging unvoluntary, non-stereotypical movements in the muscles of the face
and extremities. How is this syndrome called? Which structures are impaired?

8.3 years ago the patient had encephalitis with pathologic sleepiness. In neurological status the doctor
finds muscle rigidity, tremor on the right side. Name the pathological syndrome. Which structures are
impaired?

9.The patient has used a narcotic drug for a long time. In neurological status the doctor finds muscule
rigidity, tremor, postural imbalance, hypomimia. Name the pathological syndrome. Which structures
are impaired?

10. The patient has worm-like slow movements in distal parts of the extremities, the muscle
hypotonus, disorder of hepar function. How is this syndrome called? Which structures are impaired?

4. THE CEREBELLUM AND IT'S PATHOLOGY

The main cerebellar functions are:

► Function of equilibrium and stabilization of the body weight centre
► Regulation of the muscular tonus
► Coordination

The cerebellum is connected with all other parts of the central nervous system by means of its
peduncles. Cerebellar proprioreception gets to the cerebellum by two spino-cerebellar tracts — anterior
and posterior. As a part of the first neuron irritation from proprioreceptors of muscles, joints, tendons,
perosteum gets to the basis of the posterior horn of the spinal cord by peripheral nerves through the
posterior roots. Here Clark cells (the second neuron) are located, axons of which, not making the
decussation, go up to the posterior surface of the lateral funiculus of the spinal cord, creating the
posterior spino-cerebellar tract or Flexig's tract (the tractus spino-cerebelaris dorsalis). Reaching the
medulla oblongata, this tract as a part of the lower peduncle of the cerebellum enters the cerebellum,
finishing mainly in its worm (Fig. 4.1).
The spino-cerebellar anterior tract or Hoover's tract (the tractus spino- cerebelaris ventralis) takes its
origin from the cells of the posterior horns of the spinal cord, axons of which pass to the anterior part of
the lateral funiculus of the opposite side, and going up to the spinal cord and brain post, in the level of
the upper of cerebellum make the second decussation and through the upper cerebellar peduncles reach
the worm. The cortex of the cerebellum contains third neurons, the axons of which switch on the cortex
cells of the cerebellar hemisphere. The shoots of these cells go the dentate nucleus (tractus
3. Hie extrapyramidal system and syndromes of its lesion | 23
cerebellodentatus), and from it as a part of the dento-rubral tract through the upper peduncle of the
cerebellum go to the opposite red nucleus, making the decussation of the upper cerebellar peduncle.
Axons of the cells of red nucleus create the Forel's decussation just after leaving and as a part of
rubrospinal tracts reach alpha- and gamma mononeurons of spinal cord.
The cerebellum gets afferent proprioceptive impulses from vestibular tracts (the tractus
vestibule-cerebelaris), olives (tractus olivo-cerebelaris) and nuclei of the posterior funiculi — the thin
and the wedge-like ones.
As far as Flexig's pathway does not make decussation, and anterior spinocerebellar Hoover's tract
makes it twice, all irritations from the left part of the body get to the left part of the cerebellum, and
from the right part — to the light one. Thus brain is connected with the body homolaterally (Fig. 4.1.).

Fig. 4.1. Cerebellum and it's communications:

1 — tr. cortico-pdntinus;
2 — nucleus ruber;
3 — tr. rubro-spinalis,
4 — nucleus of pons;
5 — Flexig's tract (tr. spino-
cerebellaris dorsalis);
6 — posterior horn of spinal cord;
7 — anterior horn of spinal cord;
8 — Hoover's tract {tr.
spinocerebellars ventralis);
9 — spinal ganglion; 1 0 — nucleus dentatus;
11 — tr. dento-rubralis;
12 — tr. cebello-thalamicus

The lower peduncles provide connection with the brainstem

and spinal cord: tr. spinocerebellaris dorsalis (Flexig's). tr.
vestibulocohlearis, tr. olivoce- bellaris, fibre arcuate externe.
The cerebellum is included in a system of voluntary
movements coordination due to its links with the brain cortex.
The afferent cortico-cerebellopontine tracts go to the
cerebellum, carrying impulses about the planned action by
the brain cortex. These are two-neural tracts. The first neuron is a corticopontine tract. It takes
beginning from frontal, occipital, temporal lobes. First one passes through the semioval centre, anterior
limb of the internal capsule and ends in the nuclei of the pons on its side. Occipitotemporopontine tract
starts from the occipital lobe and posterior parts of the temporal gyri, goes through the posterior limb ot
the internal capsule and ends in the nuclei of the same side of the pons.
The middle peduncles provide connection with pons. They are presented by fibers of tr.
pontocerebellaris. They connect the nuclei of the pons with the opposite hemisphere of the cerebellum.
The upper peduncles of the cerebellum connect cerebellum with the middle brain. They include two
systems: the afferent one — from the spinal cord to the cerebellum — tr. spinocerebellars ventralis
(Hover's); efferent one — from the cerebellum to the structures of the extrapyramidal nervous system
— tr. cerebellotegmentalis and tr. dentorubralis.
Equilibrium and regulation of the muscle tone are the functions of the floc- culo-nodular lobe
(vermix).The main function of the cerebellum hemisphere is coordination of movement and synergy.
Impairment of the cerebellum produces cerebellar ataxia. There are two types of ataxia: the static
one (it develops at lesion of the vermix) and the dynamic one (it develops at lesion of hemispheres).
Static ataxia means standing and walking disorders. It is checked
4.1he cerebellum in Romberg
and It's pathology | 24 test.
Dynamic ataxia can be observed while moving. The main signs are the following: nystagmus,
scanning speech, intention tremor, missing while coordinator tests checking, dysmetria,'muscular
hypotonia, adiadochokinesia, macrographia, asynergia.
Nystagmus. Discoordination in the work of muscles that ensure the eyeballs movements lead to
involuntary rhythmic quickly repeated jerking of eyeballs when looking aside or looking up.
Scanning speech. Chopped, explosive speech with separate, effortful pronunciation of each syllable.
 Intention tremor. Oscillating deviation from the optimal path of movement that increases in the
amplitude as the target is approached, generally due to lesions of the dentate nucleus or its efferent tract.
It is easily detected with finger-nose and heel-knee tests.
Dysmetria. Incorrect amplitude or velocity of a planned movement.
Muscle hypotonia. A diminished muscle tone on passive movement.
Adiadochokinesia. Impaired performance of rapid alternating movements, due to
inadequately rapid and fluid alternation of agonist and antagonist contraction. One may
test this with rapid alternating pronation and supination of the forearm.
Macrographia. Writing with big letters.
Asynergia. Backward bending of the trunk without concomitant knee flexion, resulting in
a loss of balance.
The main kinds of ataxias:
 Cerebral
 Sensitive
 Vestibular
 Cortical

Cerebral ataxia characteristics:

 “drunken” gait
 nistagmus
 unsteadiness in Romberg’s
 bends or falls toward side of lesion
 scanning speech
 intention tremor and missing the mark in coordinational tests
 muscular hypotonia
 macrographia
 adiadochkinesia
 small influence of visual control on a degree of symptoms’ intensity
Sensitive ataxia signs:
 disorder of proprioceptive sensation
 “stamping gait”
 patients don’s feel their movements
 aggravation of symptoms by the absence of visual control
Vestibular ataxia signs:
 appearance of vertigo
 horizontal nistagmus
 nausea, vomiting
 parasympathic reactions
 aggravation of symptoms by head movements
 unnatural head position
 increased excitability of the vestibular apparatu
 healing disorder
Cortical ataxia characteristics:
 unsteady gait, especially on turns with bending toward the side, opposite to lesion
 in Romberg's position bends or falls to the side, opposite to lesion
 ataxia is combined with other symptoms of brain lobes lesion
4. The cerebellum and it's pathology | 25
I
Tests and typical tasks
1. What are the functions of the cerebellum?
a) keep organism ready for movements, realization of voluntary movements
1
b) realization of conditioned reflexes, coordination of movements
c) coordination of movements, regulation of the musci ilar tone, regulation of equilibrium
d) keep organism ready for movements, realization of voluntary movements
e) performing of mimic expression, miostatic regulation

2. What are the symptoms of cerebellum impairment?

a) scanning speech, intention tremor
b) myoclonus, hearing disorder
c) high reflexes, muscular rigidity
d) high reflexes,' presence of pathological reflexes
e) muscular rigidity, micrographia

3. What kind of speech disorder appears in the case of cerebellum impairment?

a) silent speech
b) dysarthria
c) aphasia
d) scanning speech
1
e) anarthria

4. Indicatethe coordination tests

b) a) reflexes exam finger-nasal test, diadochokinesia test
c) Rhinne's and Weber's tests
d) upper and lower Barre's tests
e) examination of the muscular tone
5. Indicate signs of cerebellar ataxia
a) hearing disorder, micrographia
b) dysarthria, high reflexes
c) muscular rigidity, micrographia
d) high reflexes, the presence of pathological reflexes
e) dysmetria, missing the mark with coordination tests

6. A tumor has destroyed the vermis in a patient. How will this pathology be manifested?
7. Will coordinational disorders be observed in the frontal lobe lesion?
8. A patient has problems with speech. It is chopped, explosive speech with a separate, effortful
pronunciation of each syllable. The neurological examination shows missing the mark with
finger-nasal and heel-to knee, tests. Which syndrome has this patient?
9.In a patient were observed acute ischemic stroke in the cerebellum. Now he has scanning speech,
impairments in coordinational tests. There is imbalance in Romberg's test. Which syndrome has this
patient? How will muscle tonus be changed?
4. the cerebellum and it's pathology | 26
10. A tumor has destroyed the right hemisphere of the cerebellum. A patient has nystagmus, missing
the mark with finger-nasal and heel-to knee tests in the right side, adiadochokinesia in the right side.
Which syndrome has this patient?

ли
 5. CRANIAL NERVES PATHOLOGY

Cranial nerves that start from the brain (number 12, Fig. 5.1) innervate the skin, muscles, organs of
the head and neck and some other organs of the thorax and abdominal cavity nerves. Ill, IV, VI, XI, XII
are motor nerves, V, VII, IX, X are both motor and sensory nerves, I, II, VIII are sensory nerves, that
support specific innervation of olfactory, optic and acoustic organs. Pairs I and II are brain derivatives,
and they don't have nuclei in the brain stem. Other cranial nerves exit from the cranial stem or come into
it, where their motor, sensory and vegetative nuclei are located. The nuclei of the pairs III and IV are
disposed in the cerebral pedunculi, pairs V, VI, VII, VIII — in the pons tentorium, pairs IX, X, XI, XII
— in the medulla oblongata.

Olfactory nerve (п. olphactorius)

Olfactory nerves (pair I) starts from olfactory cells that are located in the mucosa of the upper part of
the nasal cavity. Their dendritis perceive aromatic substances. Axons of the olfactory nerves form
olfactory nerves, go into the cranial cavity and reach the olfactory bulb. The second neurons of the
olfactory analyser are located here. Their fibres form the right and left olfactory tracts on the base of the
frontal lobe. Olfactory tracts fibres run to the olfactory centres: the olfactory triangle, anterior
perforating substance and septum pellucidum, where they commute into the third neurons. Their fibres
make a partial cross and put olfactory irritation from the olfactory subcortical centres to both
hemispheres. The cortical olfactory centres are located in the inner surface of the temporal lobe in the
uncus

Examination of olfactory function

A patient is asked to smell low aromatized substances with a known but not strong smell with closing
of each nostril separately. 5. Cranial nerves pathology | 27

Pathology of swelling includes: swelling decreasing (hyposmia), absence (anosmia), increasing

(hyperosmia), distortion (parosmia) and olfactory hallucinations (when patients smell without
irritation).
The most common causes of bilateral olfactory disorders are inflammatory pathological processes in
the nasal cavity. One-sided hypo- or anosmia appears when there is a damage to the olfactory bulb, tract
and triangle before fibers crossing that go to the cortical olfactory projection zone. This pathology
occurs when the patient suffers from a tumour or an abscess located in the anterior cranial fossa, that
injure the olfactory bulb or tract. In this case, olfactory disorders appear on the injured side. One-sided
damage of the olfactory tract upper olfactory subcortical centres doesn't cause anosmia, because each of
the olfactory centres and nose parts are connected with both cortex olfactory departments. In this cases
olfactory agnosy may occur. Cortex olfactory area irritation in the temporal lobe causes olfactory
hallucinations (it can be an epileptic aura).

Optic nerve (n. opticus)

Optic nerve (pair II) is the initial part of the visual analyser. Its receptors are located in the retina.
Their impulses run to the retinal bipolar and ganglier cells. Its axons form the optic nerve that contains
fibres from its own eye. Optic nerve passes through the optic canal and gets the cranial cavity. Medial
fibers of both optic nerves pass to the opposite side arid form the crossing — optic chiasma (chiasma
opticum). Lateral fibres do not cross each other and stay on their own side.
Then fibres of the optic nerve form the right and left optic tracts (tracti op- tici). Each tract contains
fibres from the same retinal side of the both eyes — undecussated fibers from its side eye and
decussated from the other one. Each optic tract finishes in the subcortical sight centres: the lateral
geniculate body, pulvinar thalami and superior colliculus of the midbrain Then optic fibres run through
the posterior leg of the internal capsule and form visual radiation (Graciole fasciculus), that runs to the
cortex of the inner side of the occipital lobe, where the cortical visual analyser is located. It contains
calcarine sulcus and gyruses that are located on both sides: the cuneus above and the lingual gyrus
bellow. Optic fibres that go to the superior colliculus of the midbrain take part in the ciiiar reflect arc
forming (pupils constricting after eyes illumination): optic nerve and tract -> superior colliculus —►
inserted neuron -> parasympathetic nucleus of oculomotor nerve (Yakubovych nucleus) on the own
and opposite side -> oculomotor nerve sphincter pupillae muscle. Thus optic fibres are connected with
the parasympathetic nucleus from their own side and the opposite one, that is why lighting of one eye
causes both pupillary constriction. Constriction of the stimulated eye is called a direct pupillary light
reaction. Constriction of the unstimulated eye at that time is called a consensual pupillary light reaction.
Visual analyser examination includes visual acuity, visual fields and ocular fundus definition.
Visual acuity is determined for each eye separately with the help of special tables.
The perimeter is used for the examination of limits of the visual field of each eye. The visual field can
be estimated approximately by the division of the towel in half or comparing with the vision of the
doctor. The condition of the optic nerve disk is defined by the methods of ophtalmoscopy. In normal it
is pale pink, with clear boundaries. If the optic nerve or optic chiasma or optic tract is damaged, the
optic nerve disk atrophy occurs and it becomes pale. During an increase of intracranial pressure, the
stagnant disks develop. Their borders become not clear. After congestion, the secondary atrophy of the
disk can develop.
Affection of the oplic analyser. Complete optic nerve damage causes a monocular visual loss
(amaurosis) (Fig. 5.2,1), direct pupillary light reaction loss and the preservation of the consensual
pupillary light reaction of the blind eye during lighting of the healthy eye. A visual decrease caused by
optic nerve affection is called amblyopia. When there is optic nerve pathology, we can see primary disk
atrophy on the ocular fundus.
The main syndrome of the damage of all upstream formations of the visual analyzer is hemianopia,
that is a fallout half of the visual field (Fig. 5.2, 2-3).
The visual field is a part of space that can stationary be seen. Things from the right part of the visual
field are accepted to be a left half of retina and contrary, because a reversed image is designed on the
retina.
The damage of the optic chiasm also causes the defection of the vision function of both eyes.
28 | PART 1: General
Although, the character of theseNeurology
changes can vary according to the damaged part of the chiasm. If the
central part of it is injured, the internal part of both retinas becomes "blind". It happens due to the
hypophysis tumor. That is why patient doesn't see from external (temporal) halves of both visual fields.
In this case, the visual field of the right eye. loses its right half, the visual field of the left eye misses its
left half. Such hemianopsia is called heteronomous or bitemporal (Fig. 5.2, 2). Sometimes bilateral
injury of non-crossed fibres of the optic chiasm can happen. In this case external parts of the retinas
become "blind" and internal halves of the visual fields are dropped out. It causes binasal heteronomous
hemianopsia.
 If the left or right half of visual fields is lost, that is why such hemianopsia is called homonymous left-
or right-sided. Therefore, the right optic tract damage leads to the left-sided hemianopsia and vice versa
(Fig. 5.2, 3). The damage to visual radiation or the cortical part of the visual analyser can be completed
rarely due to Wide fibres localisation, of these regions. A partial injury to these regions leads to
quadrant homonymous hemianopsia (dropout of the quarter of the visual field of both eyes) (Fig. 5.2,
4-6). Here is the upper quadrant of the same name retina in the region of the uncus and the lower one —
in the region of the lingual gyrus. That is why, when the right uncus is damaged, the right upper retinas
quadrants are "blind" and the left lower quadrants of the visual fields are lost (Fig. 5.2, 5). If the right
lingual gyrus is injured, the left upper quadrants of the visual fields are lost (Fig. 5.2, 6).

Limited defects of vision inside the visual field are called scotomas, which can be observed
during an incomplete damage to the visual fibres. Pathological processes in the region of the
occipital lobe, that irritate the visual centre cause the appearance of photopsias (flickering sparks,
strips and highlights) and visual or light hallucinations, that can be the aura of an generalised epi-
leptic attack. Sometimes damage to the external surface of the occipital lobe can be accompanied
by visual agnosia, when a patient can't recognize and distinguish things according to their
appearance.

Oculomotor group nerves (oculomotor, trochlear, ahriucens nerves)

Nerves that innervate muscles turning an eyeball belong to the oculomotor group. They are:
oculomotor (pair III), trochlear (pair IV) and abducens (pair VI) nerves.
Movements of the eye are supported by its outer striated muscles: the superior, inferior, lateral,
medial rectus, superior and inferior oblique muscles. The superior oblique muscle that turns the eye
down and out is innervated by the trochlear nerve. The lateral rectus muscle that turns the eye out is
innervated by the abducens nerve. Other muscles and the levator palpebrae muscle are innervated
by the oculomotor nerve, that supplies such movements of the eye: up, inside and down. The
scheme of eye movements is on the Fig. 5.3.
Fig. 5.3. The scheme of eye movements and striated
muscles:

1 — m. superior rectus;
2 — m. lateral rectus;
3 — m. inferior oblique;
5. Cranial nerves pathology | 29
4 — m. inferior rectus;
5 — m. medial rectus;
6 — m. superior oblique
 Oculomotor nerve (n. oculomotorius)
The oculdhiotor nerve contains somatic motor fibers that innervate extraocular striated muscles
and vegetative parasympathetic fibers that innervate intraocular smooth muscles. The oculomotor
nucleus originates in the cerebral peduncles in the level of the superior colliculus, There are located
five nuclei, each of them is connected with the innervation of a definite striated muscle. The
parasympathetic Edinger — Westphal nucleus innervates the smooth sphincter pupillae muscle that
supports pupil constriction, and Perlia nucleus, that innervates the ciliary muscle (providing
accommodation). Accommodation is the ability of the eye to see clearly things that are situated
closer.
The oculomotor nerve goes out from the brain from medial side of the peduncles and from the
skull via the superior orbital fissure.

Symptoms of oculomotor nerve affection

► dropping of the upper eyelid (ptosis)
► divergent strabismus

► patient suffers from diplopia {during looking on the side of paralysed muscles and nearby)
» absence of the eyes movements up, inside and limited down, infringement of convergence
(simultaneous inward movement of both eyes toward each other).
► dilatation of the pupil (mydriasis)
► paralysis of accommodation
► exophtalmos (bulging of the eye anteriorly out of the orbit)

Methods ot oculomotour nerve function examination

A patient is asked if he suffers from diplopia. He is inspected for ptosis, exophthalmos,
enophthalmos, anisocoria (irregularity of the pupils), constriction of the pupils, convergent strabismus,
divergent strabismus, eye movements to the sides, convergence and accommodation.
A pupillary light reaction, convergence and accommodation are inspected. A direct and consensual
pupillary light reaction is examinated. At first one eye is light, then the second eye. A pupillary reaction
to convergence is inspected by the approximation of the reflex hammer to the nose bridge (in normal the
pupils should be constricted). It is inspected of^rgyll Robertson Syndrome presence: the absence of the
direct and consensual pupillary light reflex but presence of the pupillary reaction to convergence and
accommodation (in the case of the neurosyphilis) or inverse to it (in the case of an epidemic
encephalitis).
Affection of the oculomotor nucleus or its fibers in the cerebrum peduncles is accompanied by the
damage to the nearest structure. It causes alternating syndromes. When the pathological process is
located in the base of the cerebrum peduncles, fibers that start from the oculomotor nucleus and
piramidal tract are affected. This is accompanied by alternating Weber syndrorhe arising (Table 5.1).
This syndrome is30 characterised by muscles paralysis, that are innervated by the oculomotor nerve, and
| PART 1: General Neurology
contralateral central hemiparesis or hemiplegia (Fig. 5.4).
When the pathological process is located inside midbrain tentorium we can see an affection of the
oculomotor nucleus, red nucleus and dento-rubralis tract. In this case Ben&dikt syndrome arises (Table
5.1). It is characterised by the presence of oculomotor nerve palsy and contralateral ataxia including
tremor.
 Fig. 5.4. Alternating Weber syndrome:
1— precentral gyrus;
2— internal capsule;
3— peripheral paresis of muscles that
are innervated by the oculomotor nerve;
4 — cervical intumescence;
5 — anterior horns;
6 — lumbar intumescence;
7 — motoneurons of anterior horns;
8 — lesion in the cerebrum peduncles;
9 — central hemiparesis;
10 — central tongue hemiparesis;
11 — central hemiparesis of mimic muscles

Trochlear nerve (n. trochlears)

The trochlear nerve (pair IV) is motor. Its nucleus is disposeded in the cerebrum peduncles on the level
of the inferior colliculus. This nerve exits from base of the brain and circumflexes the lateral surface of
the cerebrum peduncles. The nerve exits from the skull throught the superior orbital fissura. It supplies
superior oblique muscle. Nerve affection on one side causes diplopia during looking down and
convergent strabismus.

Abducens nerve (n. abtlucens)

Abducens nerve (pair VI) is motor. Its nucleus is disposed in the lower part of the pons, in the
projection zone of the facial colliculus (the internal geniculus of the facial nerve fibres). The abducens
nerve exits from the brain between the pyramids of the medulla oblongata and pons and from the skull
—г through the superior orbital fissura. This nerve supplies the lateral rectus muscle. Affection of this
nerve causes external eyeball movement limitation and convergent strabismus. Strabismus causes
5. Cranial nerves pathology | 31
diplopia that increases during looking to the side of the affected muscle or farther. An abducens nucleus
damage causes Foville syndrome (Table 5.1). It is characterised by abducens nerve palsy and facial
nerve palsy {diplopia, convergent strabismus, peripheral paresis of mimic muscles) on the affected side
and contralateral central hemiparesis, sometimes — a contralateral hemisensory loss.
Damage to all oculomotor group nerves causes total ophthalmoplegia: eye movements are absent,
the pupil is dilated, a loss of its light reaction. Damage to external eye muscles is called external
ophthalmoplegia, of internal — internal ophthalmoplegia.
 Gaze innervation. Innervation of conscious consensual movements of the eye — gaze — is
realized by the cortex. The cortex centre of the head and eyes turning to the opposite side is located in
the dorsal part of the medium frontal lobe. From this centre fibres run through the anterior leg of the
internal capsule, form decussation in the brainstem, finish near the nuclear of the abducens nerve on
the opposite side, where the brainstem centre of the gaze is located. Turning of the eyes to the right are
supported by the left cortex and right brainstem centre of the gaze. Consensuality of the eyeballs
movements is provided by the medial longitudinal fasciculus system that go through the brainstem and
connect nucleus of cranial nerves III, IV, VI, VIII, XI.
Lesion of the medial longitudinal fasciculus, brainstem or cortical gaze centre results in consensual
eye movement inability — the inability of the gaze to an appropriate direction (gaze paresis or palsy).
Cortical gaze centre damage results in gaze paralysis on the side, opposite to the lesion. In these
cases the head and eyes of the patient are turned to the side of damage ("eyes are looking at the lesion")
and turn away from the paralyzed limbs. When there is a damage to the brainstem gaze centre, we can
see consensual eyes movements defection (gaze paresis) to the side of a lesion. The head and eyes are
turned to the direction opposite to the lesion ("eyes are looking at the paralysed limbs").
Trigeminal nerve (n. trigeminus) r
Trigeminal nerve (pair V) is both a motor and sensory nerve. It consists of three divisions. Two first
of them are sensory, the third is mixed, consists of motor and sensory fibres. Sensory fibres supply
sensitivity of the face, cornea, sclera, conjunctiva, mucous membrane of the nose and nasal sinuses, oral
cavity, tongue, teeth, dura mater. Motor fibers innervate the chewing muscles.
The 1s' neuron of the sensory tracts is located in the trigeminal ganglion (Gasser's). Their dendrites
form three divisions: first — n. ophtalmicus, second — n. maxillaris, third — n. mandibulars. The last
division also contains motor fibres fiom the motor nuclear of the trigeminal nerve in the pons teg-
mentum. Radicle of the n. trigeminus enters the brain from the lateral surface of the pons. In the pons
deep and tactile sensitivity fibres ascend to the upper part of the pons and finish on the n. terrninalis
(principalis), where the II neuron is located. Fibres of the superficial sensitivity finish in the nucleus
longitudinalis, descending radicle (II neuron), that stretch from the pons to the spinal cord and supply
superficial sensitivity of the segmental Zelder's zones. Axons of the second neuron make decussation,
join to lemniscus medial and finish in the thalamus, where the third neurons of the sensory tract are
located. From the thalamus the third neuron goes to the postcentral gyrus (Fig. 1.1).
Trigeminal nerve examination consists of sensitivity examination of the parts that are innervated by
it and chewing muscles function. Complaints of the face pain, pain during palpation of the exit points of
the nerve can be identified. Painful, tactile and temperature sensitivities in the zones of nerve in-
nervation and segmental Zelder's zones, corneal, supraorbital, conjunctival, mandibulary reflexes are
examined. Chewing muscles atrophy, their tension during palpation and deviation of the mandible to
one side when opening the mouth are identified.
Pathology. Trigeminal nerve lesion is accompanied by intensive pain (neuralgia), anesthesia,
hypesthesia of all sensitivity kinds by the peripheral type in the innervation zone of the damaged tissue
(Fig. 5.5). N. ophtalmicus damage is accompanied by a corneal, supraorbital, conjunctival reflexes loss.
The motor nucleus or its fibres damage causes peripheral paresis of the chewing muscles on the
damaged side with their atrophy, inability to compress the jaws on this side and mandible deviation to
the damaged side during opening of the mouth. The mandibulary reflex is lost.

32 | PART 1: General Neurology

 I

Fig. 5.5, The trigeminal nerve system: 1 — n.ophtalmicus; 2 — n.maxillaris; 3 — n.mandi- bularis; 4 —
trigeminal ganglion; 5 — motor nucleus of the trigeminal nerve; 6 — n. terminalis of the trigeminal
nerve; 7 — the nucleus longitudinalis, descending radicle; 8 — the bulbothaiamicus tract; 9 — the
thalamus; 10 — the thalamocortical tract; 11 - the precentral gyrus; 12 — the postcentral gyrus; 13 —
the corticonuclear tract

і
Trigeminal ganglion lesion causes disturbance of all kinds of sensitivity on the side of the lesion
and herpes mostly in the innervation zone of one — two branches.
Lesion of different parts of the spinal tract of the trigeminal nerve (the nucleus longitudinalis,
descending radicle) causes dissociated violation of pain, temperature sensitivity on the face by the
segmental type in appropriate circular Zelder's zones with tactile and deep sensitivity preservation
(Fig. 5.5).

Facial nerve (n. facialis) |

The facial nerve (pair VII) gives innervation to mimic muscles. The intermediate nerve has
gustatory, parasympathetic salivatory and lacrimatory fibers j in the facial nerve topographic
composition. Gustatory fibers give innervation і to the anterior two thirds of the tongue. Secretory
salivatory fibers innervate the submandibular and sublingual (Rivinus') glands. Secretory lacrimatory
fibers give innervation to the lacrimal gland.
The facial nerve motor nucleus is situated in the lower part of pons Varolii. The nucleus upper part
receives corticonuclear tract from the precentral gyrus of both hemispheres and innervates upper facial
muscles. The nucleus lower part receives the corticonuclear tract from the contralateral precentral gyrus
and innervates the lower facial muscles.
The facial nerve leaves the brain at its base as well as intermediate nerve fibers close to the
vestibulocochlear nerve in the pontocerebellar angle. The intermediate nerve fibers are mostly situated
in the medulla oblongata and are common with pair IX (the gustatory nucleus of the solitary tract, the
upper salivatory nucleus).
Out of the brain the facial nerve together with the intermediate nerve fibers and auditory nerve go to
the internal acustic meatus in the petrosal part of the temporal bone. Here the facial and intermediate
nerves enter the facial nerve canal where they give the first branch — the greater petrosal nerve (n.
petro- sus major) that consists of parasympathetic fibers and innervates the lacrimal gland. Involvement
of this nerve33leads
I PART 1:to xerophthalmus
General Neurology (dry eye). j
Lower goes the nerve to the stapedius muscle (n. stapedius) that gives motor fibers in the tympanic
cavity. Disorders of this muscle innervations are manifested with hyperacusia — uncomfortable
increased sound perception.
Still lower goes Ihe cord of the tympanum (chorda tympani) that has gustatory fibers for the anterior
two thirds of the tongue and salivatory — for the submandibular and sublingual glands.
After the cord of the tympanum origin motor fibers of the facial nerve leave the skull through the
stylomastoideus hole and is spread to the face as a 'greater goose claw'. The facial nerve innervates all
mimic muscles (at the exception of muscle that lifts the upper eyelid).
While examining facial nerve function one defines face asymmetry, flatness of the forehead skin
folds and nasolabial fold. The patient is asked to lift up the eyebrows, to frown, to close, the eyes, to
wrinkle his nose, to show the teeth, to puff up his cheeks, to whistle, to blow. One considers the
presence of watering eye or dryness of an eye conjunctiva, hyperacusia, examines taste on the anterior
two thirds of the tongue.
Pathology. Lesion of the facial nerve or its nucleus manifests itself in peripheral palsy of half face
mimic muscles. There are no skin folds while frowning. The eyebrow does not lift up, the eye doesn't
close (lagophthalmos). While closing one's eyes tightly the eyeballs lift up and there is white sclera
stripe on the lesion side (Bell's symptom). The mouth's angle is dropped down, the nasolabial fold is
smoothed. A patient cannot show the teeth on palsy's side because the mouth angle is fixed. The
supraorbital, corneal and conjunctival reflexes are decreased or absent (the efferent links of the reflex
arches are impaired).
Depending on the level of facial nerve impairment mimic muscles peripheral palsy combines with
other symptoms. If the cerebellopontine angle (auditory nerve neurinoma) as well as peripheral palsy
mimic muscles are impaired, symptoms of auditory and intermediate nerves' disturbance are manifested
— hearing impairment, xerophthalmus, taste disorders on one's tongue (hypo-/ ageusia) and a dry
mouth. If located in the facial nerve canal peripheral palsy of mimic muscles combines with
xerophthalmus, hyperacusia and a loss of taste. If the facial nerve is involved after it leaves the bone
canal only peripheral palsy of mimic muscles and lacrimation are shown.
The facial nerve lesion in pons combines with the impairment of the pyramidal tract that is shown as
Millard — Gubler alternating syndrome (Table 5.1). There are peripheral palsy of mimic muscles on the
involved side and central palsy of the opposite extremities (Fig. 5.7). In different case together with
facial nerve fibers in the pons and pyramidal tract the nucleus of the abducent nerve is impaired —
Foville syndrome is evident, as mentioned above.

34 | PART 1: General Neurology

 Fig. 5.7. Millard — Gubler alternating syndrome:
1 — precentral gyrus;
1 2 — capsula interna;
3 — peripheral paralysis of mimic
TJnr muscles;
4 — cervical intumescence;
5 — anterior horns;
6 — lumbar intumescence;
7 — motoneurons of anterior horns;
8 — lesion in the pons;
9 — central hemiparesis;
10 — central tongue hemiparesis

Central paresis of mimic muscles appears because of pathology in the lower part of the precentral
gyrus or in the corticonuclear tract on the side opposite to the lesion. Central paresis of mimic muscles
often combines with extremities paresis also on the side opposite to the lesion that usually appears in
stroke. Central paresis of mimic muscles unlike peripheral one arises just in the lower part of the face
(lower the palpebral fissure) muscles which receive one-side innervation from the cerebral cortex. The
supraorbital, corneal and conjunctival reflexes are kept since their reflex arches are not interrupted.

Vestibulocochlear nerve (n. vestibulocochlear)

The vestibulocochlear nerve (pair VIII) is sensory, consists of two independent nerves — vestibular
and cochlear that possess different functions.

Cochlear nerve (n. cochlearis) is auditory, conducts auditory stimuli from the cochlea spiral organ
auditory receptors of the internal ear. The first neuron is situated in the base of the cochlea in spiral
ganglion. Axons of ganglion cells form the cochlear nerve that as well as vestibular and facial nerves
enters the skull cavity through the internal acustic meatus and gets into the brain in the pontocerebellar
angulus. The cochlear nerve ends in the brainstem ventral and dorsal auditory nuclei of the lateral pons
part and medulla oblongata where the second neurons of the auditory analyzer are situated. Auditory
fibers of these nuclei added to fibers of grey matter supplemental formations (the superior olive,
trapezoid body nucleus) partly go to the opposite side and up in the brainstem forming the lateral
lemniscus and end up in the subcortical auditory centers — the interior geniculate body and inferior
tubercle of the lamina tectum of the mesencephalon. The third neuron starts from the interior geniculate
body, passes through the internal capsule and radiate crown to the cortical part of the auditory analyzer
that is situated in transverse upper temporal (Heschl's) gyrus. The semidecussation of auditory fibers
provides bilateral connection of the hearing organ to the subcortical and cortical auditory centers.
Methods of cochlear nerve function examining include examining of hearing acuity, bone and air
conduction. Hearing acuity is examined separately for each ear with the help of whisper and loud
speech at the distance of 6-7 meters. Normally a healthy ear hears whisper at the distance of 6-7 meters,
loud speech — at 20 meters. Hearing acuity is examined precisely with the help of audiography.
 Patient's hearing could be decreased at the disorder of the sound-perceiving or sound-conducting
apparatus in the middle ear. Tuning tests are held to define which of the system (sound-perceiving or
sound-conducting) is Impaired. Bone and air conduction are usually examined neurologically with the
help of tuning fork with frequency of vibration 128 per second.
Rynne test. The vibrating tuning fork is placed on the mastoid. After a patient stops to feel the
vibration the tuning fork branches are moved to external acustic meatus at the distance of 1-2 cm. The
healthy person hears a sound through the air almost twice longer than through the bone. In this case the
test result is registered as positive. If the patient after the bone does not hear a sound through the air this
is evident for sound-conducting apparatus impairment (otitis, otosclerosis etc.) and is registered as
negative Rynne test.
Veber test. The vibrating tuning fork is placed in the middle of the patient's vertex. Normally a sound
is felt with both ears or in the middle. In case of unilateral disorder of the sound-conducting apparatus
bone conduction would be better than the air one that's why the patient feels a sound with the sick ear
better. In the case of unilateral disorder of the sound-perceiving apparatus (the spiral organ, cochlear
nerve) the tuning fork sound is felt better by the healthy ear.
Pathology of the auditory analyzer. There are such disorders as complete loss of hearing, deafness
(anacusis), hearing impairment (hypacusis), increased perception (hyperacusis). A one-sided decrease
or a hearing loss is possible only during pathology of the inner ear, cochlear nerve or its nuclei (in
neurological clinic often because of cochlear nerve neuropathy or its neurinoma in the pontocerebellar
angulus). Unilateral impairment of the lateral lemniscus, subcortical auditory centers or cortical part of
the auditory analyzer doesn't produce perceptible hearing disturbances. If the pathological process
irritates the cortical part of the auditory analyzer auditory hallucinations occur which could be the aura
of the generalized convulsive seizure.
Vestibular nerve (n. vestibularis) is a part of the vestibular analyzer that provides information
perception and analysis of the head and body situation in the space. The vestibular nerve conducts
impulses from the semicircular ducts and statoconic apparatus of the inner ear. The peripheral neuron of
the vestibular analyzer is situated in the vestibular ganglion that is located in the inner ear. Its axons as
a part of the vestibular nerve together with the cochlear nerve make their way through the internal
acustic meatus toward the vestibular nuclei of the brainstem. These nuclei carry bodies of the second
neurons of the vestibular analyzer whose axons go in different directions, providing connection of the
vestibular apparatus with cerebellum, the oculomotor group of the nerves nuclei via the system of the
medial longitudinal fascicle, with the anterior spinal cord horns, brainstem reticular formation, vagus
nerve nucleus and other structures. Numerous connections of the vestibular analyzer explain the
presence of different symptoms once it is impaired. The cortical part of the vestibular analyzer is
situated in the cortex of the temporal lobe beside the auditory projection area.
Examining the vestibular analyzer's function includes checking the presence of spontaneous
nystagmus, balance disturbances, performing coordination tests, defining excitability of the vestibular
analyzer with the help of caloric and rotative tests, electronystagmography.
Vestibular analyzer pathology. Vestibular disorders appear as a result of vestibular analyzer injury
in any level: because of inner ear diseases, once the vestibular nerve is impaired especially in the
pontocerebellar angulus, in the braistem pathology, brain cortex lesions. The leading symptoms of
vestibular function disturbances are systemic vertigo and nystagmus. Vertigo is a feeling of rotation of
enclosing subjects in one direction.

Glossopharyngeal nerve (n. glossopharyngeus)

The glossopharyngeal nerve (pair IX) is mixed, contains somatic motor fibers, fibers of general and
taste sensitivity, parasympathetic secretory fibers. The glossopharyngeal nerve has four nuclei — the
motor double nucleus and the nucleus of general sensitivity common with the vagus nerve, also the
taste nucleus common with the intermediate nerve and the inferior salivatory nucleus. The nerve leaves
the brain in the area of the posterior lateral sulcus of the medulla oblongata behind the olive and the
skull — via the jugular foramen.
Motor nerve fibers innervate just one pharyngeal muscle — stylopharyngeal. The nerve provides
sensitive innervation
36 I PART 1:of theNeurology
General posterior third of the tongue, the soft palate, throat, pharynx, anterior
surface of epiglottis, also of the auditory tube and tympanic cavity. Taste nerve fibers perceive mostly
bitter and salt taste irritations from the posterior third of the tongue. Parasympathetic secretory nerve
fibers from the inferior salivatory nucleus innervate the parotid salivatory gland.
 Pathology. Once the glossopharyngeal nerve is impaired, the sensitivity of mostly bitter taste (hypo-
or ageusia) on the posterior third of the tongue of the involved side is disturbed; there are partial
swallowing disorder and anesthesia of pain, tactile and temperature sensitivity in the innervation area.
Irrittation of sensitive nerve fibers evokes neuralgia with seizures of one-sided pain at the root of the
tongue, palatine tonsil, soft palate, throat that appears while swallowing, intense chewing, talking.
Isolated impairment of the glossopharyngeal nerve leads to decreasing of pharyngeal and palatal
reflexes as a result of a partial breakage of its reflex arches.

Vagus nerve
The vagus nerve (pair X) is mixed, the longest of all cranial nerves. It contains parasympathetic,
somatic motor and sensitive fibers. The nerve has three nuclei, two of which: is the motor and sensitive
— are common with the glos- sopharyngeal nerve; also there's posterior parasympathetic nucleus. The
vagus nerve goes out of the brain in the posterior lateral sulcus of the medulla oblongata and leaves the
skull via the jugular foramen. It is situated between the carotid artery and jugular vein; the vagus nerve
penetrates in the chest and enters the abdominal cavity via the esophageal foramen of the diaphragm
giving numerous branches to innervate the internal organs. Sensitive fibers of the vagus nerve innervate
the dura mater of the brain, the depth of the external acustic meatus, pharyngeal mucous membrane,
mucous mei'nbrane of larynx, trachea, bronchi, lungs, digestive tract and other organs.
Motor somatic fibers of the vagus nerve innervate transversal striated muscles of the pharynx, soft
palate, larynx and epiglottis. Motor parasympathetic fibers innervate nonstriated muscles of the trachea,
bronchi, esophagus, stomach, small intestine and upper part of the large intestine; secretory fibers go to
the stomach, pancreas, inhibitory fibers — to the heart, vasomotor ones — to vessels.
Examination of glossopharyngeal and vagus nerves function in neurological clinic is carried out
usually together. One asks how patient swallows, if there is choking, if the liquid food hits the nose. One
should pay attention to voice disturbances (hoarse, snuffling), soft palate drop; follow the mobility of
the soft palate while articulating "a", whether the uvula deviates to the side. Examine the taste on the
posterior third of the tongue, pharyngeal and palatal reflexes, pulse and bieathing rates, blood pressure.
Pathology. As a result of unilateral lesion of the vagus nerve motor fibers one can observe a drop of
the soft palate on the impaired side, its immobility while articulating "a", uvula deviation to the healthy
side. The voice is soundless, hoarse (dysphoria) because of vocal cords paralysis on the lesion side. If
the bilateral nerve damage is present swallowing disturbance appears — dysphagia. Soft palate
paralysis leads to hitting of a fluid diet upon the nose and shuffling voice, epiglottis paralysis produces
choking while eating. Pharyngeal and palatal reflexes are decreased or absent. The vagus nerve
irritation manifests itself by hiccup.

Accessory nerve (n. accessorius)

Accessory nerve (pair XI) is motor; innervates sternocleidomastoid and trapezoid muscles. Long
motor nucleus of accessory nerve consists of two parts. The upper (cerebral) part is situated in the lower
areas of the medulla oblongata, the lower (spinal) part of the nucleus is placed at the base ot the anterior
horns of spinal cord upper cervical segments. The axons of the nucleus spinal part cells leave with thin
radices on the lateral surface of the spinal cord and going up unite to the trunk that enters the posterior
cranial fossa via the great occipital foramen. Here it joins fibers from the cerebral part of the accessory
nerve nucleus that leave the brain in the posterior lateral sulcus of the medulla oblongata. The general
nerve trunk leaves the brain via the jugular foramen and innervates muscles pointed.

Examination of accessory nerve function. While examining muscle function that are innervated
with the accessory nerve a patient is asked to turn the head to the sides, to tilt it to the front, to lift up the
5. Cranial nerves pathology | 37
shoulders (to shrug the shoulders), to move the scapulas toward the spine, to lift up the arm higher than
the horizontal level.
Pathology. The damage to XI cranial nerves' pair manifests itself with paralysis or paresis of these
muscles. As a result of trapezoid muscle paresis the shoulder on the involved side is dropped, the
inferior angle of the scapula deviates from the spine to the side and up ("iwinged scapula'), lifting up the
upper extremity higher than the horizontal level is limited. The weakness of the sternocleidomastoid
muscle is shown as disturbed turn of the head to the healthy side, the muscle is poorly outlined.
Bilateral damage of accessory nerve manifests itself as hanging down of the head {the dropping head
symptom).

Hypoglossal nerve (n. hypoglossus)

The hypoglossal nerve (pair XII) is clearly motor; innervates tongue muscles. The nucleus of this
nerve is situated in the lower area of the medulla oblongata. The nerve goes out of the brain in the
anterior sulcus of the medulla oblongata between the pyramid and inferior olive; and leaves the skull
via hypoglossal canal of the occipital bone.,
Examination of hypoglossal nerve function. While the patient puts out the tongue one examines
whether it is deviated to either side from the medial line. Define the presence of atrophy and fibrillar
muscular twitching. Examine the speech articulation.
Pathology. As a result of hypoglossal nerve damage appears peripheral paralysis or paresis of the
tongue muscles with atrophy. While putting out the tongue from the mouth it deviates to the side with
paralysed muscles. Also the speech articulation is disturbed, it becomes unintelligible [dysarthria), at a
total tongue immobility (glossoplegia) — even impossible (anarthria).

38 I PART 1: General Neurology

Fig. 5.8. Jackson alternating syndrome:
1 — the precentral gyrus;
2 — the internal capsule;
3 — peripheral tongue paresis;
4 — the cervical intumescence;
5 — the anterior horns;
6 — the lumbar intumescence;
7 — the motoneurons of the anterior horns;
8 — lesion of one half of the medulla oblongata;
9 — central hemiparesis

The hypoglossal nerve nucleus and pyramid tract damage on one half
of the medulla oblongata manifests itself as Jackson alternating
syndrome (Table 5.1). There are peripheral paresis of half of the
tongue with atrophy and fibrillar muscular twitching on the lesion side
and central hemiparesis on the opposite side (Fig. 5.8).
There is central paresis of the opposite half of the tongue at the
unilateral damage to corticonuclear fibers because the nucleus of pair
XII connects just with the opposite hemisphere by the corticonuclear
tract. In central paresis there is no tongue muscle atrophy.

5. Cranial nerves pathology | 39

Bulbar and pseudobulbar syndrome

A combined injury of bulbar group nerves and its nuclei is observed in clinic more often than
isolated. The symptom group of motor disorders that appears with the impairment of IX, X, XII cranial
nerves' pairs' nuclei or their radices on the brain basis is called bulbar syndrome.
Bulbar syndrome could be uni- or bilateral with the development of peripheral muscles' paresis or
paralysis that are innervated by IX, X, XII pairs. Disturbances of swallowing (dysphagia), voice
(dysphonia), speech articulation (dysarthria) are observed. A patient chokes while eating, swallowing
is difficult or even impossible (aphagia). The food hit to the trachea and bronchi could produce
aspiration pneumonia. The tongue is atrophied. At the pathology of XII pair' nucleus fibrillar muscular
twitching are observed. Pharyngeal and palatal reflexes are decreased or absent.
Vegetative disturbances are possible in the bulbar syndrome (breathing disturbances, heart function
disorder) which in some cases provide unfavorable prognosis.
Central paresis of muscles that are innervated by bulbar nerves is called pseudobulbar syndrome. It
appears just if there is bilateral damage of the corticonuclear tract that goes from the motor cortical
centers to bulbar group nerves' nuclei. The impairment of the corticonuclear tract in one hemisphere
does not lead to this combined pathology because muscles innervated by bulbar nerves (except for the
tongue) receive double-sided cortical innervation. Since pseudobulbar syndrome is central paralysis of
swallowing, phonation ifand speech articulation it also manifests itself with dysphagia, dysphonia,
dysarthria. Unlike bulbar in pseudobulbar syndrome atrophy of tongue muscles and fibrillar muscular
twitching are not observed, pharyngeal and palatal re- flexes^are kept, the mandibular reflex is
increased. In pseudobulbar syndrome patients show reflexes of oral automatism (lip, nasolabial,
palmomental etc.), that is explained by disinhibition of the brainstem and subcortical formations at
bilateral damage of the corticonuclear tract; these are the levels where reflexes named are enclosed.
This is the reason why sometimes forced crying or laugh is observed. In pseudobulbar syndrome motor
disorders may be accompanied by memory, attention and intelligence deterioration. Although the
damage is symmetrical and manifests itself severely pseudobulbar syndrome is less dangerous than
bulbar since It is not accompanied by vital functions disturbances.
Alternating syndromes
The brainstem which contains the mesencephalon, pons cerebrum and medulla oblongata has long
ascending sensitive and descending motor conducting tracfe; and also the cranial nerves' nuclei.
In view of the corticospinal (pyramidal) tract that gives deccusation lower the brainstem while the
sensitive spinothalamic tract decussates in the spinal cord, unilateral damage to these tracts on the
brainstem level manifests itself with central muscle paresis and a sensitivity loss on the opposite to the
lesion side. Lesions in the brainstem are characterized by cranial nerves' nuclei' (or nucleus') damage
with developing of their impairment symptoms on the lesion side. That is why damage in the brainstem
manifests itself by the development of alternating syndromes: symptoms of cranial nerve nucleus'
impairment on the lesion side and central hemiparesis or hemiplegia and also conductive sensitivity
disturbances on the opposite side. These syndromes are called alternating. A combination of nucleus
damage symptoms with conductive tract disorder symptoms is very significant for topical diagnosis
since it points to damage to cranial nerve nucleus either fibers inside the brainstem or the cranial nerve
itself.
Depending on the pathological process localization the alternating syndromes sort on peduncle
(mesencephalon damage), pontine (pons damage) and medullary (medulla oblongata damage) (Table
5.1). At the pathology in the cerebral peduncle develop Weber and Benedikt syndromes, at pons dam-
age — Millard — Gubler and Foville syndromes. At the medulla oblongata damage more often Jackson
and Wallenberg — Zakcharchenko alternating syndromes are observed.
In the case of ischemic stroke in the basin of the posterior inferior cerebellar ar- teiy appears
Wallenberg — Zakcharchenko alternating syndrome. There are soft palate and vocal cord palsy (the
nuclei of pairs' IX, X) on the lesion side, segmental dissociated disturbance of pain and temperature
sensitivity on the face (the nucleus of pair V), Horner syndrome (sympathetic fibers), cerebellar
disorders (the spinocerebellar tract). Conductive pain and temperature sensitivity disturbance as a result
of spinothalamic tract damage is observed on the opposite side.
Alternating syndromes develop in the cases of cerebral blood circulation disturbances, inflammatory
and tumor brainstem processes; and they do have a great significance for topical diagnosis.
 Alternating syndromes

Table 5.1.
Syndrome Affected Ipsilateral symptoms Contralateral
name neurological symptoms
structures
Peduncle
Weber - nucleus n. diplopia central
oculomotor» ptosis of the superior eyelid hemiparesis
- the cortico-spinal divergent strabismus
tract exophthalmus mydriasis
disturbances of accommodation
and convergence the absence of
pupillary light reflex
Benedikt - nucleus n. diplopia choreoathetosis
oculomotorii ptosis of the superior eyelid hemiataxia
- red nucleus divergent strabismus
- the dentato-rubral exophthalmus mydriasis
tract disturbances of accommodation
and convergence the absence of
pupillary light reflex
Pontine
Foville - nucleus n. diplopia central
abducentis convergent strabismus peripheral hemiparesis
- nucleus n. facialis paresis of mimic muscles
- the cortico-spinal
tract
Millard - - nucleus n. facialis, peripheral paralysis of mimic central
Gubler muscles hemiparesis
- the cortico-spinal
tract
Medullary
Avellis - nucleus n. palate paresis vocal central hemiparesis
glossopha- ryngei cord paresis
- nucleus n. vagi
- the cortico-spinal
tract
Schmidt - nucleus n. palate paresis vocal central hemiparesis
glossopha- ryngel cord paresis

5. Cranial nerves pathology | 41

 Syndrome name Affected Ipsilateral symptoms Contralateral
neurological symptoms
structures
- nucleus n. vagi trapezius and
- nucleus n. stemocleldo-
accessorli mastoideus muscles
- nucleus n. peripheral paresis
hypoglossl tongue peripheral
- the co'rtico-splnal paresis
tract
Jackson - nucleus n. tongue peripheral central hemiparesis
hypoglossi paresis
- the cortlco-spinal
tract
Wallenberg - Zakchar- - nucleus n. palate paresis vocal hemihypalgesia
chenko glossopha- ryngei cord paresis Horner hemlthermhyp- aestliesia
- nucleus n. vagi syndrome segmental
- descending loss of pain, and
sympathetic fibers temperature sensation
- the nucleus of on the face hemiataxia
descending tract of
cranial nerve V
- the
spino-cerebellar
tract
- the
spino-thalamic tract

Tests and typical tasks

1. Damage to what part of the visual analyzer is the cause of amaurosis?
a) thalamus
b) internal capsule
c) optic tract
d) optic nerve
e) occipital lobe

2. Indicate the localization of the pathological center in the case of alternating Weber syndrome
a) oculomotor nerve
b) abducens nerve
c) cerebri peduncle
d) pons
e) medulla

3. Indicate
the abducens nerve damage symptoms
a) convergent strabismus, double vision

42 I PART 1: General Neurology

b) ptosis of upper eyelid, mydriasis
c) divergent strabismus, double vision
d) absence of pupil reaction, enophthalmia
e) peripheral paresis of mimic muscules, sensitive disorder on the face
4.Impairment of what nerve structures leads to sensitive disorders on the face of the segmental
dissociative type?
a) trigeminal nerve branches
b) trigeminal nerve ganglion
c) postcentral gyrus lower part
d) internal capsule
e) nucleus of the trigeminal nerve spinal tract

5. Where is the source of damage localized in the case of central paresis of mimic muscles?
a) facial nerve nucleus
b) corticonuclear tract
c) facial nerve
d) abducens nerve
e) trigeminal nerve
6.A patient has left mimic muscles peripheral paresis and hearing disturbances on the left ear. Where
is the lesion localized?
7. Apatient with a left hemisphere stroke has tongue deviation to the right side and dysarthria. What
paresis of tongue muscles is present?
8.A patient has progressing pituitary adenoma that supresses the centre of the optic chiasm. What
vision changes will appear?
9. After
flu the patient has paroxysmal pains with impairment of all sensitive modalities in the right
forehead area. Which nerve is impaired? What reflexes may dissapear?
10. A patient has dysarthria, dysphagia, dysphonia, tongue muscle atrophy and fasciculations,
respiratory and cardio-vascular dysfunction; the pharyngeal reflex is absent. Define the type of
muscular paresis. Name the syndrome.
6. ГНЕ AUTONOMIC NERVOUS SYSTEM AND SYNDROMES OF ITS LESION

The autonomic (vegetative) nervous system is responsible for the process of nutrition of an organism,
metabolism, growth, reproduction, circulation of liquids, so it controls the activity of the internal
organs.
According to the international anatomic nomenclature, the term "autonomic nervous system" is
commonly used. However, the previous name "vegetative nervous system" is traditionally used in
Ukrainian literature.
The іуаіп functions of the autonomic nervous system
a) trophotropic—regulation of the activity of the internal organs, maintenance of the stability of the
internal environment of the organism — homeostasis;
b) ergotropic — provision of adaptive processes — all forms of the psychic and physical activity of
an organism.

Mentioned physiological functions are regulated independently (autonomic- ly), without a conscious
control of them.
The autonomic nervous system is divided into the following parts: supra- segmental and segmental
levels. The last one according to the structure and functional peculiarities is divided into the
sympathetic and parasympathetic nervous system (Fig. 6.1). The sympathetic nervous system
6. I lie autonomic nervous system and syndromes of its lesion | 43
innervates all the organs and tissues of an organism, unlike the parasympathetic one. The central
nervous system, most vessels, uterus, modular layer of adrenal glands, sudoriferous glands don't have
parasympathetic innervations.
The suprasegmenlal level is presented by the hypothalamo-limbico-retic- uiaris complex.
 Hypothalamus
The hypothalamic zone plays an important role among subcortical structures.
The hypothalamus is connected with many structures of the central nervous system, it provides
integration of the somatic and vegetative activity of an organism.
The main functions of the hypothalamus are regulation of heart-vascular activity, regulation of
endocrine glands' function, regulation of lipid, water, mineral metabolism, thermoregulation, the
emotional behavior, the homeostasis of the internal environment of an organism.
Pathogenesis of hypothalamic syndrome is conditioned by the peculiarities of its vascularization: the
intensity of capillar blood supply and high penetrability of its vessels lead to an increase of the
penetrability of vessels of this part for great-molecular compounds (toxins, viruses, hormones and other
humoral substances). It leads to high susceptibility of the hypothalamic zone in the case of arising of
different pathological processes.
The Limbic System
The complex'of limbic system structures is an organizer of the unity of many functions of an
organism. It consists of: the bulbus, tractus and trigonum ol- phactori, substantia perforate anterior,
septum pellucidum, gyrus cinguli, gyrus hypocampalis, corpus amygdaloidem, mediobasal surface of
the frontal lobe.
Here primary synthesis of all sensivity, the analysis of the state of the internal environment are
carried out and elementary needs, motivations, emotions are formed. The limbic system provides
interaction of vegetative, visceral, sensor- motor and emotional systems. Its state influences on the level
of consciousness, attention, memory, ability to orientate in space, motor and psychic activity, ability to
perform automatic movements, disorders of sleep and awakeness.

Formatio reticularis
Thet reticular formation of the brainstem plays a significant role in the suprasegmental part also. It
has its independent role, but is also one of the integrative apparatuses of the brain. The nuclei of the
reticular formation (there are about 100 ones) form suprasegmental centres of vital functions:
respjrative, vascularmotor, cardial activity, swallowing, vomiting and so on. The reticular formation
also controls the state of sleep and awakeness, the physical and tonic state of muscles, decodes
informative signals from the environment.
Interaction of the reticular formation with the limbic system provides organization of conscious
activity according to changeable conditions of the environment.

Pathology of the suprasegmental level

The lesions of the hypothalamus cause hypothalamic syndromes. There are main forms of
hypothalamic syndromes.
1. Neuroendocrine form manifests as Itsenko — Cushing syndrome, adiposogenital dystrophy,
central/hypothalamic obesity, pituitary cachexia (Simmonds' disease), diabetes insipidus,
idiopathic edemas, persisting lactorrhea-amenorrhea.
2. Vegetative vascular dystonia form. It is associated with the crisis of the paroxysmal character.
There may be 3 variants of crisis: sympathy-adrenal, vagoinsular and mixed attacks. The signs of a
sympathy-adrenal attack are: a pale and dry skin, shortness of breath, dizziness or faintness, high
blood pressure, tachycardia, feeling of internal tremor, fear of dying. The opposite signs of a
vagoinsular attack: hyperemia of the face, sweating, bradycardia, decreasing blood pressure.
Mixed attacks begin as the sympathy-adrenal and finish as the vagoinsular crisis or vice versa.
3. Thermoregulative disturbances. The permanent body temperature rise is up to 37.1-37.5. There is
asymmetry under the arms and in the rectum.
4. Neurotrophic form is associated with trophic disturbances (skin dryness, neu- rodermitis, ulcers,
bed sores, acute perforates of stomach and esophagus).
5. The neuromuscular form is characterized by myastheno-like, myotono-like syndroms.
Sometimes may be cases of paroxysmal myoplegia.
44 I PART 1: General Neurology
6. Disorders of sleep and awakeness are associated with insomnia, lethargy and sleeping inversion.

The symptoms of Limbic System lesion are emotional disturbances, changes of eating behavior
(anorexia or bulimia), sleeping disorders, sexual disturbances, memory disorders.
The segmental part of the autonomic nervous system
The parasympathetic part is divided into the craniobulbar and sacral parts.
The craniobulbar part
There are the fibers from parasympathetic Yakubovich's and Perlea's nuclei included in the
oculomotor nerve that provide innervation of smooth eye muscles: the muscle sphincter pupillae and
muscle ciliaris, the latter ensures accomodation of crystalline lens.
There are the fibers from secretory lacrimal nucleus included in the facial nerve that provide
innervation of the tear-exciting gland.
There is the same anatomic structure of the superior and inferior salivatori- us nuclei included in the
facial (VII pair of cranial nerve) and glossopharyngeal nerves (IX pair) that innervates parotid,
sublingual, submandibular glands.
Dorsal (visceral) nucleus of the nervus vagus (X pair of the cranial nerve) innervates heart,
gastrointestinal tract, gastric glands and other internal organs (except for the small pelvis).
The sacral pan of the vegetative nervous system
There are the fibers from segments S2-S4 included in the pelvic nerves (nn. pelvici) that provide
innervation of the urinary bladder, rectum, genitals.
The sympathetic nervous system consists of cells of the lateral horns of the spinal cord (C8-L2).
The axons of these cells form preganglionary fibers (white connecting branches). Some of them end in
the sympathetic column, which consists of 20-23 nodi: cervical-3, thoracic — 10-12, lumbar — 3-4,
pelvic — 4. Then postganglionary fibres (grey connecting branches) are formed for all organs and
tissues of anorganism.
Some fibres are not disconnected in the sympathetic column, but go directly to prevertebtal
ganglions, making plexuses, for example, the plexus celiacus.
Sympathetic innervation of the eye — the ciliospinal centre (cells of the lateral horn C8-T2). The
axons of these cells are interrupted in the upper cervical ganglion. Then postganglionary fibres form
sympathetic plexus around a.carotus internal and rise upwards into the skull for the innervation of such
muscles as m.dilatator puppilae, m.orbitalis, m.tarsalis superficialis.
Sympathetic fibers from the ganglion stellate go around vertebral arteries, innervate vessels in the
vertebra-basilar basin and give branches to the heart and larynx. The thoracic pad of the sympathetic column
gives branches to the heart, lungs, pleura and organs of abdominal cavity. Sympathetic fibers from the
sacral part go to organs and vessels of the small pelvis.

Clinical symptoms ot segmental parts lesion

They include the symptoms of III, VII, IX and X cranial nerves lesion if craniobulbar part is
damaged.
Lesion of the ciliospinal centre or cervical sympathetic nerve leads to Bernard — Horner syndrome:
partial ptosis, miosis and enophtalmia. Irritation of sympathetic fibers results in syndrome Pourfour du
Petit: widening of palpebral fissure, mydriasis, exophthalmia.
Afterwards, there may be the following symptoms:
1. Sympathalgias (heart-like pain in the innervation area, senestopathies, paresthesias).
2. Vasovegetative syndrome — a change in skin color, tissue swelling, numbness, cold hands and
feet.
3. Trophic vegetative syndrome — dryness, skin desquamation, a loss of
hair, formation of ulcers, hyperkeratosis, nail fragility, arthropathy.

Vegetative innervation of the urinary bladder

Regulation of urinary function is performed by reflex, involuntary, and conscious mechanisms.
The urinary bladder has double vegetative (sympathetic and parasympathetic) innervation of smooth
muscles: the detrusor and internal sphincter. Thenervous
6. I lie autonomic spinal parasympathetic
system and syndromes of its lesion centre
| 45 is located in the lateral
horns S2-S4 of the spinal cord. Parasympathetic fibers from this centre are included in pelvic nerves
and innervate detrusor urine. Sympathetic innervation begins from the spinal sympathetic centre — the
lateral horns T1-L2 of the spinal cord, and innervates the internal sphincter,
 Regulation is performed with the help of cortical control and the voluntary reflex of regulation of
urination. The afferent part of this reflex begins from receptors of the internal sphincter, includes spinal
ganglions, posterior radixes, posterior funiculars, medulla, pons and finish in sensory zones of the
cortex (girus fornicatus). The efferent part from the cortical motor centre of urination (the paracentral
lobule) passes through the bilateral cortico-spinal tracts (the lateral and anterior funicles of the spinal
cord) to the spinal centers of urination, which fibers are included in anterior radixes, genital plexus,
n.pudendus and innervate the external sphincter of the urinary bladder.
The neurogenic bladder is a syndrome that unites disorders of urination, which appear in the case
of lesion to nervous tracts or centres which innervate the urinary bladder and provide conscious
function of urination. There are central and peripheral types of disorders of urinary bladder function.
Central types of disorders of urination appear in the case of bilateral lesion of the cortex and its
connections with spinal (sacral) centres of urination.
There are:
a) urinary retention (retentia urinae);
b) periodic urinary incontinence (incontinentia intermittens);
c) an imperative urinary urge.

Peripheral types of urination disorders appear in the case of the localization of the pathologic
process in sacral segments of the spinal cord, radixes of the
cauda equina and peripheral nerves, so parasympathetic innervation of the urinary bladder is impaired.
There are:
a) paradoxal urinary retention (the ishuria paradoxa);
b) real urinary-incontinence (the incontinentia vera).

Tests and typical tasks

1. What structures of the brain belong to the suprasegmental level of the autonomic nervous system?
a) hypothalamus, limbic-reticular complex
b) substantia nigra, sympathetic trunk
c) spinal cord lateral horns, medulla
d) sympathetic trunk, postganglionary sympathetic fibers
e) parasympathetic nuclei of cranial nerves, medulla

2. What is the formation of the segmentary level of the sympathetic autonomic nervous system?
a) hypothalamus
b) lateral horns cells of C8-L2 segments
c) limbic system
d) substantia nigra
e) medulla

3. What is the formation of the segmentary level of the parasympathetic autonomic nervous system?
a) reticular formation
b) lateral horns cells of C8-L2 segments
c) limbic system
d) X pair dorsal nucleus
e) hypothalamus

4. What is the craniobulbar formation of the parasympathetic nervous system?

a) Gaulle's and Burdah's nuclei
b) red nucleus
c) caudatus nucleus

46 I PART 1: General Neurology

 d) limbic system
e) salivatory nuclei
5 What is the function of the suprasegmental level of the autonomic nervous system? -
visual function
memory substrate
muscle tone support
sensitive innervation
coordination of movements
6. A patient has a heart rhythm disturbance, heat-like pain in his face, neck and arm. What is the
preliminary diagnosis?
7. A
patient has bradycardia (heart rhythm lowering), blood pressure lowering, asthmatic breath,
myosis. Name the attack that withholds these symptoms.
8. A patient has a tumor at S3-S5 level. What kind of urinary disturbance does the patient show?
9.A patient has a pale, dry skin, tachycardia, high blood pressure, tremor, fear of dying. Name the
attack that withholds these symptoms.
10. Young woman has amenorrhea, diabetes insipidus, trophic disturbances, insomnia, What is the
preliminary syndrome?
7. THE BRAIN CORTEX. LOCALIZATION OF CORTICAL FUNCTIONS. DISTURBANCES
OF HIGHER CEREBRAL FUNCTIONS

The grey «natter which covers all the surface of hemispheres is called the cerebral cortex
(neocortex). Different areas of the cerebral cortex may be distinguished from one another by their
histological features and neuroanatomical connections. Cytoarchitecture is the science of the cortex
cell structure. Most of the cerebral cortex consists of the isocortex which has six distinct cytoar-
chitectural layers:
► lamina molecularis,
► lamina granulans externa,
► lamina piramidalis externa,
► lamina granulans interna,
► lamina ganglionaris,
► lamina multiformis.

In 1909 K. Brodmann published his maps of cortical areas in humans. Brodmann's classification of
cortical areas is based on distinguishing histological features of adjacent areas of the isocortex. K.
Brodmann distinguished 11 areas and 52 fields (Fig. 7.1.).
Myeloarchitecture is the science of fibers structure. There are distinguished association,
commissural and projection fibers. Association fibers connect different regions of one hemisphere,
commissural — both hemispheres, and projection ones connect the brain and spinal cord.

Localization of cortical functions Projection areas

By following the course of axons entering and leaving a given cortical area, projection ones may
determine the other structures
7.1 lie to which
brain cortex. Localization it is
of cortical connected
functions. Disturbancesby afferent
of higher and efferent
cerebral functions | 47 pathways.
The primary projection areas are those that receive most of their sensory impulses directly from the
thalamic relay nuclei:
> primary somatosensory cortex, Brodman areas 1, 2, 3,
> the visual, area 17,
> the auditory, areas 41, 22,
> the olfactory, areas 27, 28.

The primary motor cortex (area 4) sends motor impulses directly down the pyramidal pathway to
somatic motor neurons within the brainstem and spinal cord.
 Fig. 7.1. Brodmann's map of the brain cortex'

The primary projection areas are somatotopically organized and serve the contralateral half of the
body.
Proceeding putward along the cortical surface from the primary projection areas, one encounters the
secondary projection areas — motor, sensory, visual, auditoiy — which subserve higher functions of
coordination and information processing, and tertiary projection areas, which are responsible for
complex functions such as voluntary movement, spatial organization of sensory input, cognition,
memory, language, and emotion,
Secondary projection areas:
► motor, areas 6, 8,
► sensory, areas 5, 7a,
► visual, area 18, 19,
► auditory, area 42.
Main tertiary projection areas: 7b, 9,10, 37,39, 40, 45.

Functional asymmetry of the brain

Many tasks are performed primarily by one of the two hemispheres. This was called the cerebral
dominance. Now we speak about supplementary specialization of hemispheres: the left hemisphere is
responsible for logical and abstract thinking, the right hemisphere — for creative and concrete
thinking. The specialization of hemispheres is concerned with the predominant hand.

Higher cerebral functions

► speech and language
► reading and writing
► praxis and gnosis
► memory48 I PART 1: General Neurology
► mentality
► consciousness
 Language refers to symbolic communication. It is the ability to converse, comprehend, repeat, read,
and write. The language ability depends on central processing for either comprehension or formulation
for expression the sounds and symbols of prepositional communication.
Language processes are lateralized in the left hemisphere. This has been determined through
anatomoclinical correlation in patients with language disturbances and associated brain pathology.
Approximately 90 percent of the general population are right-handed. They prefer using their right
hand for most motor activities, especially skillful tasks. Approximately 99 percent of right-handed
individuals have language functions in the left hemisphere.
Speech is the ability to vocalize by coordinating the muscles controlling the vocal apparatus. It is the
mechanical aspect of oral communication. Normal speech involves a highly coordinated sequence of
contractions of the respiratory musculature, larynx, pharynx, tongue, and lips. These muscles are inner-
vated by the facial, vagal, hypoglossal and phrenic nerves.
Coordination of the movements of the articulatory structures requires simultaneous and coordinated
activation of these cranial nerves, not only by direct cortical motor activation but also by indirect
involvement of the basal ganglia and cerebellum. The basal ganglia and cerebellum are also important
participants in producing prosodic aspects of speech, including pitch or frequency, stress or differential
emphasis placed on syllables, and rhythm or timing.
Speech comprehension and production. Words first reach the peripheral auditory apparatus and
finally reach the primary auditory cortex (Heschl's gyrus) in the superior temporal gyrus. The auditory
language content undergoes preliminary decoding in the auditory association cortex, Wernicke's area
(Brodmann's area 22) located in the posterior third of the left superior temporal gyrus. The auditory
information travels from Wernicke's area to the motor association cortex, Broca's area (Brodmann's
area 44, 45) located in the posterior part of the inferior left frontal convolution, through the arcuate
fasciculus, a band of white matter deep to the supramarginal gyrus connecting both language areas.
Broca's area initiates a motor plan that is transmitted to the primaiy motor cortex (Brodmann's area 4)
to pronounce words. The motor cortex, in coordination with the supplementary motor area, basal
ganglia, and cerebellum, sends corticobulbar fibers to implement speech sounds.
Reading depends on visual stimuli, like written words, reaching the primary visual cortex
(Brodmann's area 17). The visual stimuli are then transmitted from the primary visual cortex, to the
visual association cortex. The word stimuli are further processed in the heteromodal association cortex,
the angular gyrus, for semantic meaning and integration with other sensory modalities and past
experiences.
Writing requires a transfer of language information to the motor association cortex superior to
Broca's area and then to motor neurons in the primary motor cortex (Brodmann's area 4), projecting to
the arm and hand. Writing to dictation involves a transfer of auditory information from Wernicke's area
to the anterior motor areas.
Praxis is the ability to perform voluntary skilled movements (for example, using a knife to cut a slice
of bread). For each specific skilled movement, there is a set of spatial loci that must be converted in a
specific temporal pattern. It is proposed that movement formulas represented in the inferior parietal
lobe are stored in a three-dimensional supramodal code. For the corticospinal neurons to properly
activate the motor nerves, the stored spatial-temporal knowledge has to be transformed into a motor
program.
The medial premotor cortex including the supplementary motor area appears to play an important
role in mediating skilled movements. The convexity premotor cortex also receives projections from the
parietal lobes as well as from the medial premotor cortex and projects to the primary motor area.
Gnosis is the ability to recognize a known object. There are distinguished visual, auditory, gustatory
and olfactory gnosis. Gnosis is provided for secondary and tertiary projection areas.
Methods of higher cerebral
7.1 lie brain functions
cortex. Localization examining
of cortical functions. include
Disturbances defining
of higher cerebral functionsa| 49
disturbances of consciousness
— confusion, stupor, coma. The degree of consciousness impairment is estimated according to
Glasgow Coma Scale (GGS). A doctor has to determine patients' perception of themselves and their
environment, behavior and responses to external stimuli. In the normal state of consciousness, a patient
is fully conscious, oriented and awake.
Examining a comprehension (receptive) and spontaneous (expressive) speech includes determination
of motor (Broca's), sensory (Wernicke's) and amnestic aphasia.
While examining higher cerebral functions one defines counting, reading, writing, gnosis and praxis.
 Disturbances of higher cerebral functions Speech and language disorders

Language disorders are termed aphasias, and involve language disturbances in comprehension,
production, or both.
Speech disorders are termed:
► dysarthria, a disturbance in articulation,
► dysphoriia, a disturbance in vocalization or phonation.
Patients with dysarthria or dysphonia retain their language ability despite their speech disturbance.
Description of the symptom is important in characterizing and differentiating speech and language
disorders;
► difficulty in articulation or vocalization implies a speech disorder,
► whereas the inability to find words, comprehend, read, or write is indicative of a language
disorder.
Differentiation of speech and language disorders has an important localizing value for underlying
pathology within the nervous system and helps distinguish among different etiological processes.

Kinds of aphasias
Broca's aphasia (also called anterior, motor, or expressive aphasia) is characterized by the absence or
severe impairment of a spontaneous speech, while comprehension is only mildly impaired.
Wernicke's aphasia (also called posterior, sensory, or receptive aphasia) is characterized by severe
impairment of comprehension. A spontaneous speech remains fluent and normally paced, but
paragrammatism, paraphasia, and neologisms make the patient's speech partially or totally
incomprehensible (word salad, jargon aphasia).
Amnestic (anomic) aphasia. This type of aphasia is characterized by impaired naming and
word-finding. A spontaneous speech is fluent but permeated with word-finding difficulty and
paraphrasing. The ability to repeat, comprehend, and write words is essentially normal.
Global aphasia involves all aspects of language and severely impairs spoken communication. A patient
cannot speak spontaneously or can only do so with a great effort, producing not more than fragments
of words. Speech comprehension is usually absent; at best, patients may recognize a few words,
including their own name.

Other disturbances of higher cerebral functions

Apraxia. While some patients complain of a loss of ability to perform skilled movements, most do not
recognize their disability or complains about it. The activities to be inquired about include the use of
common bathroom tools such as toothbrushes, razors, combs, and brushes.
Agnosia — the inability to recognize a known object. May be visual, auditory, gustatory and olfactory
agnosia. Alexia — the inability to read and no other language disturbance. Agraphia may be defined as
a loss or impairment of the ability to produce written language, caused by brain dysfunction. Acalculia
— the inability to count.
Autotopagnosia — the inability to identify one's own extremities and other parts of the body.
Anosognosia — the inability to identify one's own defect, for example, he- myparalysis.

Syndromes of lobes damage

Frontal lobe:
► central palsies on the opposite side,
► one-side anosmia,
► mental disturbances ('frontal' psychics),
► frontal ataxia,
► motor aphasia, loss of writing (agraphia),
► sight palsy,
► prehensile movements,
►motor Jackson epilepsy (when the lobe is irritated). Occipital lobe:
50 I PART 1: General Neurology
► visual agnosia, agnosia to colors,
► agnostic alexia,
► homonymous hemianopsia,
 ► photopsies and visual hallucinations (when irritated).
Parietal lobe:
► tactile anesthesia,
► alexia,.agraphia, acalculia, ^ apraxia,
► autotopagnosia, anosognosia,
► sensory Jackson epilepsy (when the lobe is irritated). Temporal lobe:
► sensory and amnestic aphasia,
► ataxia, homonymous hemianopsia,
► auditory, gustatory, olfactory agnosia,
► auditory, taste, olfactory hallucinations (when irritated),
► vestibular vertigo (dizziness),
► deja vu, jamais vu syndromes — more than once or not once seen.

Syndromes of hemispheres damage

Left hemisphere:
► central palsies on the opposite side,
► aphasia,
► alexia,
► agraphia,
► acalculia,
► apraxia.
Right hemisphere:
► central palsies on the opposite side,
► autotopagnosia,
► anosognosia,
► mental disturbances,
► parakinesia — continual movement in the right extremities.

Tests and typical tasks

l . What pathology you will see when precentral gyrus Is damaged?
a) hemianesthesia
b) tactile amnesia
c) monoplegia
d) hemianopsia
e) olfactory agnosia
2. Name type of aphasia:
a) motor
b) dysarthria
c) dysphonia
d) alexia
e) mutism

3. Where is the cortical part of the olfactory analyzer localized?

a) frontal lobe
b) occipital lobe
7.1 lie brain cortex. Localization of cortical functions. Disturbances of higher cerebral functions | 51
c) anterior central gyrus
d) temporal lobe
e) postcentral gyrus

4. Where is the pathological lesion in the case of visual hallucinations localized?

a) optic nerve
b) postcentral gyrus
c) temporal lobe
d) subcortical optic centers
e) occipital lobe
5. The sensory aphasia is characterized by:
a) loss of ability to name familiar subjects
b) disturbance of speech comprehension
c) disturbance of expressive speech
d) speech dysarthria
e) disturbance of complex logically-grammatic structures comprehension
6 Patient understands when talking to him but he is not able to talk back. No anar- thria is observed.
What is the name of syndrome? Where is the lesion site?
7. Examination of the patient revealed central monoparesis of the right hand,,motor aphasia. Give
topical diagnosis.

8.A patient is not able to name objects but he explains how to use them Name what is damaged and
how is disturbance called?
9. A
patient has the left arm astereognosis, superficial kinds of sensitivity are saved.
Where is 0 lesion localized?
10. A patient suffering from stroke is unable to perform skilled movements — to comb one's hair,
brush teeth, button up. What is damaged and how is disturbance called?

52 I PART 1: General Neurology

 8. CEREBROSPINAL FLUID. MENINGEAL AND HYPERTENSIVE SYNDROMES

The brain and spinal cord are invested by three meningeal membranes called the dura mater,
arachnoid, and pia mater. The subarachanold space lies between the arachnoid and pia mater. It is filled
with cerebrospinal fluid. Ventricles are called the spaces which are filled with cerebrospinal fluid and
protect the brain by cushioning it and supporting its weight. The two lateral ventricles extend across a
large area of the brain. The third ventricle lies between two thalamic bodies. The fourth ventricle is
located between the cerebellum and the pons.
Cerebrospinal fluid (CSF) is a clear, colourless liquid that surrounds the brain and spinal cord and
fills the spaces in them (Fig. 8.1). CSF arises from the blood and returns to it at a rate of about 500 ml a
day. In the adult the total volume of CSF is about 150-200 ml. The circulation of CSF is completely
replaced about every 4 hours. CSF is produced by the choroid plexus located in the lateral, third and
fourth ventricles. From the lateral ventricles CSF drains through the two foramina of Monro, which are
also known as the interventricular foramina, into the third ventricle, and then through the aqueduct of
Sylvius into the fourth ventricle.

Fig. 8.1. Ventricles and the direction of

cerebrospinal fluids flow:
1 — superior sagittal sinus;
2 — arachnoid villi;
3 — subarachnoid space;
4 — lateral ventricles;
5 — choroid plexus;
6 — third ventricle;
7 — aqueduct of Sylvius;
8 — fourth ventricle;
9 — the central canal of the
spinal cord

From the fourth ventricle, the CSF flows into the subarachnoid space through the foramina of
Magendie and Luschka to the 'basal cisterns' under the brain. After that the flow of CSF is mainly up
and over the whole brain surface, partly flows down around the spinal cord. Completing the circuit
back to the bloodstream, the fluid drains via the valve-like arachnoid villi (arachnoid granulations) into
the sagittal sinus. Some of CSF is also taken up into veins around spinal nerve roots and into the
lymphatic system.
Physicians frequently perform lumbar puncture. Indications to lumbar punctures are suspected
CNS infection, subarachnoid hemorrhage, central nervous system tumors. Contraindications to
lumbar punctures ж local skin infections over a given puncture site, raised intracranial pressure.
Lumbar puncture is performed by inserting a needle between the L3-L4 or L4-L5 below the lower
end of the spinal cord (the spinal cord ends near L1-L2) (Fig. 8.2).
Routine examination of CSF includes visual observation of color and clarity . and commonly
performed tests: a quantitative assessment of protein and glucose levels, cell counts and differential,
microscopic examination and culture.8. Additional
Cerebrospinal fluid. tests such
Meningeal as measuring
and hypertensive pressure and a polymerase
syndromes | 53
chain reaction also may be performed. Knowing which tests to order and how to interpret them allows
physicians to use CSF as a key diagnostic tool in a variety of diseases.
 Fig. 8.2. Lumbar
puncture: 1 — the
spinal column

Normal CSF appearance is clear and colourless. Pressure ranges from 70 to 180 mm H 20, pH —
7.31. White Cells: 0-5 x 106 per liter (all lymphocytes with no neutrophils). Red Cells — absent.
Protein: 0.2-0.4 grams per liter (or less than 1 % of the serum protein concentration). Glucose: 2.5-4.0
mmol per liter, approximately one half of the fasting plasma glucose.

Pathology
Xanthochromia is most often caused by the presence of blood, but several other conditions should
be considered. The presence of blood can be a reliable predictor of subarachnoid hemorrhage. An
increase of white blood cells in CSF may occur in case of viral, bacterial, fungal, and parasitic
meningitis.
• The white blood cells differential helps to distinguish these causes. For example, viral infection is
usually associated with an increase in lymphocytes, while bacterial and fungal infections are
associated with an increase in neutrophils. A low glucose level occurs in bacterial meningitis.
Tuberculoses meningitis is characterized by an increase in lymphocytes, a decrease of glucose and
chlorides in CSF. High total proteins levels in CSF are seen usually in tumors.
There are two kinds of quantitative indices dissociation in liquor: cells- protein and protein-cells
dissociation. Cells-protein dissociation means that the cells count increases more than the protein
level or proteins level is normal. Cells-protein dissociation occurs in meningitis. Protein-cells
dissociation means that the protein level increases more than cells or the cells level is normal.
Protein-cells dissociation is caused by tumor. An increase of lymphocytes in CSF is called
lymphocyte pleocytosis, occurs in the case of serous meningitis. Raise of neutrophils — neutrophil
pleocytosis, occurs in the case of purulent meningitis.
The cultural method is the gold standard for determining the causative organism in meningitis.
However, a polymerase chain reaction is much faster and more sensitive in some cases.
CSF hypertension syndrome is caused by an increased intracranial pressure.
Causes of hypertension syndrome:
► a cerebral or extracerebral mass such as brain tumor, massive infarctions with edema, extensive
traumatic contusion, parenchymal, subdural, or extradural hematoma or abscess;
► obstruction to the flow and absorption of CSF;
► any process that expands the volume of CSF (meningitis, subarachnoid hemorrhage);
► an increase in venous pressure due to cerebral venous sinus thrombosis;
► generalized brain swelling (ischemic-anoxic states).

The clinical manifestations of increased intracranial pressure in children and adults are a diffuse
headache (very often at the night and in the morning), vomiting, nausea without diarrhea, dizziness,
sleepiness, irritability, impaired consciousness and seizures..
Additional research data. A CSF hypertension can often be recognized by investigation of
eye-ground. Papilloedema is usually present. If the pressure ' of the CSF is raised, on X-ray of the skull
dilatation of the entrance into Turkish saddle, osteoporosis of its back may be seen. There is an
enlargement of 54the ventricular
I PART system on MRl and CT.
1: General Neurology
Meningeal symptoms
They are a sign of irritation of the meningeal membranes, such as seen in meningitis, subarachnoid
hemorrhages. IMeck stiffness, Kernig's signs and three Brudzinski's are termed meningeal signs.
Neck stiffness — inability to flex the neck forward passively due to an increased neck muscle tone
and stiffness.
Kernig's sign is assessed when the patient is lying supine, with the hip and knee flexed to 90 degrees.
In a patient with a positive Kernig's sign, there is the resistance to passive extension of the knee while the
hip is flexed.
A positive Brudzinski's upper sign occurs when flexion of the neck causes involuntary flexion of the
knee. Positive Brudzinski's middle sign — pressure on the pubic symphysis leads to the reflexive knee
flexion. Positive Brudzinski's low sign — investigation of the Kernig's sign leads to involuntary knee
flexion in the opposite leg.
Meningeal pose —a hyperextended posture when patient throws back the head and flex the legs.
General hypersthesia and hyperesthesia of organs of senses include photophobia (intolerance to a
bright light) and phonophobia (intolerance to loud noises).
Reactive painful phenomena: painfulness when pressing eyeballs, points of outlet of branches of
trigeminal, occipital nerves, cheek-bone Behterev's symptom.

Hydrocephalus
The term hydrocephalus is derived from the Greek words "hydro" meaning water and "cephalus"
meaning head. When for any reason the volume of CSF increases within the head, the size of the
ventricles also increases and it is known as hydrocephalus. The reasons for CSF excess may include:
too much produce, too little reabsorption back into the venous system, blockage in flow.
Hydrocephalus may be congenital or acquired. Hydrocephalus may also be communicating or
non-communicating. Communicating hydrocephalus occurs when the flow of CSF is blocked after it
exits the ventricles, the CSF can flow between the ventricles. Non-communicating hydrocephalus —
also called "obstructive" hydrocephalus — occurs when the flow of CSF is blocked along one or more
of the narrow passages connecting the ventricles.

Tests and typical tasks

1. Indicate,
which types of meningitis are characterized by lymphocyte pleocytosis,
a decrease of glucose and chlorides in cerebrospinal fluid:
a) herpetic
b) syphilitic
c) tuberculous
d) enterovirus
e) parotitic

2. Cerebrospinal fluid returns back directly to the venous system by means of the:
a) cerebral veins
b) arachnoid villi
c) choroid plexus
d) cerebral aqueduct
e) apertures in the third ventricle

3. A 25-year old woman suffered from headfluid.

8. Cerebrospinal injuries
Meningeal after a vehicle
and hypertensive accident.
syndromes | 55 Her MRl reveals an
epidural hematoma. Between which two structures has blood been accumulated?
a) between the arachnoid and the pia mater
b) between the dura and the pia mater
c) between the dura and the arachnoid layer
d) between the skull and the dura mater
e) between the pia and the arachnoid layer

4 If the foramen of Monro is obstructed, what pathology would occur in this case?
a) non-communicating hydrocephalus
 b) communicating hydrocephalus
c) subdural hematoma
d) epidural hematoma
e) subarachnoid hemorrhage

5. What changes in the eye fundus can we see in the case of CSF hypertensive syndrome?
a) optic neuritis
b) optic nerve atrophy
c) Salus symptom
d) chorioretinitis
e) optic disks edema

6.Patient's CSF is turbid, cytosis is 1600 cells per 1 mm3 (90 % of neutrophils), protein is 0.4 g/l; sugar
- 1.2 millimole per litre. What is the name of such a ratio of cells and protein?

7.During examination at clinic, it was noted that the size of an infant's head was larger than normal
expected for her age. Ultrasonography examination indicated obstruction of the cerebral aqueduct,
enlargement of the lateral and third ventricles. What is the name of such pathology?

8 Results of the examination of CSF In a patient who had meningeal signs are following: 120 cells per
mm3 (mostly lymphocytes) and protein 0.99 g/l. What diagnosis can be suspected? How do we name
similar cell-changes in CSF?

9. A patient suffers from pain in the back. Examination of CSF showed: protein 5.2 g/l, cells 5 per mm1.
How can we name changes in CSF? What disease can be suspected? '
10. A boy of 7 years old has acutely fallen ill. His temperature has raised up to 38.2 С he has vomited
repeatedly and restlessly. His head is tossed back, the meningeal symptoms are present. No extremity
paresis is observed. Describe meningeal symptoms. What does their appearance indicate?

56 I PART 1: General Neurology

 9. ADDITIONAL METHODS IN NEUROLOGY

Electroencephalography
Electroencephalography (EEG) is a dynamic test of electrical activity of the brain, the most important
diagnostic study in epileptology, provides information about the function, rather than the structure of
the brain. EEG is performed by placing 20 electrodes on the scalp,(the standard EEG is recorded
through mounted electrodes on the scalp according to the 10-20 system), amplifying the activity and
displaying it on a monitor or paper. EEG is obtained either as a bipolar recording (in which potential
differences are measured between the scalp electrodes) or as a monopolar recording (in which the
difference is measured between each scalp electrode and a reference electrode). Brain waves are
summations of excitatory and inhibitory potentials that are projected through the reticular nucleus of the
thalamus to the cerebral cortex.
The normal EEG changes in the early years of life but becomes standardized during adolescence.
Normal rhythm in adults in awake with closed eyes, resting state is alpha (8-13 Hz), disappears by
opening eyes or in a drowsy state. Beta activity (14- 30 Hz) may be seen in the frontal areas. Theta
rhythm (4-7 Hz) is normal during drowsiness or sleep, abnormal in alert. A small amount of theta
activity may be present in the awake. Delta rhythm (less than 4 Hz) is normal during drowsiness or
sleep, abnormal in alert. Delta activity is always abnormal in awake patient. Slow waves (delta, theta)
may emanate from the white matter and represent a disruption of neural pathways. Spike, sharp waves—
pathological EEG patterns, represent polarization shifts of neuron groups in the cortex.
Hyperventilation, photostimulation and sleep deprivation are all provocative methods by which focal
disturbances of brain function are more visible.
EEG is useful in the nonspecific diagnostic of diseases with focal structural lesions: encephalitis,
tumors, strokes, brain abscesses. These diseases show focal slow activity. Focal temporal lobe
discharges occur in herpes simplex encephalitis. EEG helps to recognize coma, dementia and brain
death. Electrocerebral silence is seen in brain death. Generalized slowing (mostly delta and theta
activities) is seen in metabolic encephalopathy, postseizure states, dementias, encephalitis or anoxia.
The method should be evaluated in cases of epilepsy or any altered state of consciousness. EEG is
usually recorded between seizures (interictal) but sometimes — during seizures (Ictal). The diagnosis of
epilepsy is clinical and the normal interictal EEG does not exclude a diagnosis of epilepsy. Spike and
sharp waves may be focal (partial epilepsy) or generalized (primary generalized epilepsy). Sleep
deprivation moves up patients into the superficial stage of sleep, in which the likelihood of a
paroxysmal electrical disturbance is the greatest. •

Evoked potentials
Evoked potentials (EP) are potentials, recorded from the projection cortex zones following
stimulation of appropriate sensory receptors.
Visual EP. Electrodes, which are placed over the occipital lobes, will record visual stimuli (usually
alternating checkerboard flash patterns are used in each eye as the stimulus). Latency and amplitude
changes may occur in optic neuritis or multiple sclerosis.
Brainsiem-auditoiy EP. Electrodes, which are placed over the vertex, will record cerebral potentials
following auditory signals (usually a click). It can help to distinguish lesions of the auditory nerve (e.g.,
acoustic neuroma) from brainstem lesions (e.g., stroke) by the shape and distribution of the evoked
potentials.
Somatosensory EP. Electrodes, which are placed over the parietal lobes, will record stimulation of the
peripheral nerves. Commonly they are used during spinal 9. Additionalsurgery to determine
melliods in neurology | 57 if there has been an
interruption in nerve or spinal transmission.

Electromyography
Electromyography (EMG) is an electrical recording of muscle activity that helps in the diagnosis of
neuromuscular diseases. Muscles are stimulated by signals from lower motor neurons. This stimulation
causes electrical activity in the muscle, which in turn causes contraction. This electrical activity is
detected by a needle electrode inserted into the muscle and connected to a recording device. The
interpretation of EMG results requires analysis of the onset, duration, amplitude, and other
characteristics of the EMG patterns. Muscle tissue normally shows no EM6 activity at rest or when
moved passively by the examiner. EMG activity is decreased in long-standing muscle disorders where
muscle tissue is replaced by fibrous tissue or fat.
EMG is performed more often to help to diagnose different diseases causing muscle weakness:
muscular dystrophy, congenital, mitochondrial, metabolic myopathies, myotonias, peripheral
neuropathies, radiculopathies, nerve lesions, amyotrophic lateral sclerosis, polyneuropathies, spinal
muscular atrophy, Guillain — Barre syndrome, ataxias, myasthenia. .

Angiography
Angiography is still considered the "gold standard" for identifying blood vessel stenosis and
aneurysms. Cerebral angiography was invented by the Portuguese neurologist and neurosurgeon Egas
Moniz, who performed the first carotid angiogram in 1927. Cerebral angiography is mainly used for the
diagnosis or exclusion of the following: stenosis and occlusion of intra- and extracranial blood vessels,
cerebral venous and venous sinus thrombosis, ruptured and unruptured cerebral aneurysms, specific
arterial abnormalities including dissection and fibromuscular dysplasia, or irregular caliber and mycotic
aneurysms in inflammatory and infectious conditions, arteriovenous malformations and fistulae, brain
tumors (characterization of blood supply). In addition, angiography is part of the interventional
treatment (by the neuroradiologists) of aneurysms, arteriovenous malformations and fistulae, arterial
stenosis and vasospasm. It is also a part of the technique of intraarterial thrombolysis, and of
mechanical extraction of intravascular thrombi.

Ultrasonography
Ultrasonography — neurologically relevant diagnostic techniques using ultrasound include
Transcranial Doppler (TCD) and duplex-ultrasonography of the intra- and extracranial arteries.
This technique is based on the effect first described by Christian Doppler in 1843. Doppler
ultrasound, a special application of ultrasound, measures the direction and speed of blood cells when
they move through vessels. The movement of blood cells causes a change in pitch of the reflected sound
waves (called the Doppler effect). A computer collects and processes the sounds and creates graphs or
color pictures that represent the flow of blood through the blood vessels.
In recent years, the capabilities of ultrasound flow imaging have been increased enormously. Color
flow imaging is now commonplace and facilities such as 'power' or 'energy' Doppler provide new ways
of imaging flow in duplex-ultrasonography. Color flow imaging can be used to identify vessels,
requiring examination, to identify the presence and direction of flow, to highlight circulation anomalies,
throughout the entire color flow image, and to provide beam/vessel angle correction for velocity
measurements. Pulsed wave Doppler is used to provide analysis of the flow at specific sites in the vessel
by investigation. When using color flow imaging with pulsed wave Doppler, the color flow/B-mode
image is frozen while the pulsed wave Doppler is activated. Recently, some manufacturers have
produced concurrent color flow imaging and pulsed wave Doppler, sometimes referred to
triplex-ultrasonograpliy.
When these modes are used simultaneously, the performance of each is decreased. Because
transducer elements are employed in three modes (B-mode, color flow and pulsed wave Doppler), the
frame rate is decreased, the color flow box is reduced in size and the available pulse repetition
frequency is reduced, leading to increased susceptibility to aliasing. Power Doppler is also referred to
energy Doppler, amplitude Doppler and Doppler angiography. The magnitude of the color flow output
is displayed rather than the Doppler frequency signal.
A Doppler ultrasound may help to diagnose many conditions, including arterial occlusion or stenosis
by blood clots, decreased blood circulation, aneurysms and venous insufficiency.

Computed tomography

58 I PART 1: General Neurology

Computed tomography (CT) is a digital technique that produces cross-sectional anatomical images.
Its physical basis is the differential absorption of roentgen rays by tissues of different density. Tissue
density is expressed on the Hounsfield scale, named in honor of the inventor of CT. The scale runs from
-1000 to +1000 Hounsfield units. The density of water is, by definition, 0 Hounsfield units. When CT
images are acquired one section at a time, the roentgen ray tube has to be rotated through 360° for image
acquisition, and then back to the starting position, and the patient table must then be moved forward, in
preparation for the next image. An improved technique for image acquisition is known as helical
(spiral) CT. In this technique, the roentgen ray tube continually, rotates in one direction, while the
patient table continually moves forward at a constant speed. As a result, the examination time is
reduced, and the images are sharper; normal and pathological anatomy are well demonstrated, and
functional and dynamic CT studies can also be performed. The use of intravenous contrast material
enhances the visibility on CT of blood, and of tissue in which there is a disruption of the blood-brain
barrier. Contrast raises the sensitivity and specificity of CT for a wide range of pathological processes.
CT can be used in trauma,, intracerebral hemorrhage, and shift of midline structures. Contrast
enhancement is necessary for differentiation between stroke and neoplasms.
Advantages of CT. CT provides better visualization of bone and acute hemorrhage. A CT is generally
sufficient in emergency situations. In the case of the clinical suspicion of an acute subarachnoid
hemorrhage, the imaging study of choice is CT. For the elderly patients with focal neurological signs or
confusion, a CT usually suffices to detect all potentially treatable conditions, such as hydrocephalus and
chronic subdural hematoma. CT may be a necessary test in patients who cannot undergo magnetic
resonance imaging scanning (e.g., claustrophobia, pacemaker, metallic stent, aneurysmal clips).
Disadvantages ofCT: the exposure of the patient to ionizing radiation, fails to show infarcts for up to
24 h, lack of anatomic detail, fails to identify multiple sclerosis plaques,
CT angiography (СТА) — the basis for selective imaging of blood vessels: if one digitally
suppresses other structures with high roentgen-ray absorption, such as bone, and then reformats the
sectional images of the vasculature in three dimensions, a projectional image results, resembling the im-
ages of conventional angiography. СТА is a suitable technique for aneurysm screening, and for the
demonstration of stenosis of the carotid artery or other vessels.

Magnetic Resonance Imaging

Magnetic resonance imaging (MRI) Is based on the theory of nuclear magnetic resonance,
discovered by Bloch and Purcell in 1946. The visible physical entities In MRI are the protons of
hydrogen nuclei, which are richly abundant іп all tissues containing water, proteins, lipids, and other
biological macromol- ecules. Protons rotate around their own axis, and therefore have a small magnetic
field. Protons' rotation in the transverse plane of the external magnetic field emits electromagnetic
radiation, which can be detected with a radio antenna or coil. This radiation constitutes the magnetic
resonance (MR) signal. MRl provides much better tissue contrast than conventional roentgenography or
CT. MRl gives excellent anatomic details and shows virtually all structural lesions. The MR image is a
planar (tomographic) map of the MR signal as it varies in intensity across different types of tissue.
Tumors and other tissues with .high free-water content appear dark on T1-weighted and bright on
T2-weighted or proton density images. Cerebrospinal fluid consists largely of free water, it is thus very
dark on T1- weighted images and very bright on T2-weighted and proton density images. T1-weighted
images are more suitable than T2-weighted images for the demonstration of necrosis and cystic change
within a tumor, or for the demonstration of subacute hemorrhage. Diffusion weighted imaging (DWI) is
extremely valuable to identify early stroke signs. Perfusion weighted imaging (PWI) is helpful in
demonstrating area at risk in stroke (ischemic penumbra). Gradient echo is helpful for hemorrhage, both
old and new. T2 weighted image shows edema and white-matter lesions well. FLAIR sequences are
useful for evaluation of multiple sclerosis. Contrast enhancement with gadolinium will better show
neoplasms and multiple sclerosis plaques. MRl is a good noninvasive method for spinal cord lesions or
herniated disks in cervical or lumbar regions.
9. Additional melliods
Advantages of MRl. MRl provides better tissue visualization thanin neurology
CT and | 59thus allows the diagnosis of
many conditions affecting the brain parenchyma that are not usually visible on CT, including
microangiopathy, axqnal injury in head trauma, the plaques of multiple sclerosis, encephalitises, and
other conditions.
MRl is absolutely conlraindicated in the presence of cardiac pacemakers, neurostimulators, cochlear
implants, ferromagnetic aneurysm clips, and other ferromagnetic foreign bodies in tissues that are
susceptible to injury.
 Magnetic resonance angiography (MRA) is the initial imaging of choice; 2-D time of flight shows
extracranial circulation (vertebral and carotid bifurcations); 3-D time of flight demonstrates intracranial
circulation; contrast-enhanced studies will show the aortic arch and aortic branches.
Radioisotope Studies
Single photon emission computed tomography (SPECT) and Positron emission tomography (PET)
both involve measurement of the photons emitted by intravenously administered radioactive isotope
tracers.
SPECT enables the measurement of regional cerebral perfusion, oxygen and glucose consumption,
and blood volume and can thus be used as a test of brain functions. The radioactive tracers are
radioactive technetium or iodine compounds. PET requires a cyclotron for the production of the
short-lived radionuclides that emit positrons. It is used to generate tomographic images of local cerebral
blood flow, cerebral blood volume, oxygen consumption, glucose consumption, and intracellular pH. It
enables the performance of biochemical studies in vivo. Coupling of these radioactive tracers with
specific biologically active chemicals, such as DOPA, enables the investigation of specific metabolic
processes in the brain. PET and SPECT have vastly increased our understanding of the pathophysiology
of numerous cerebral diseases. Their primary use in epileptology at present is as part of the preoperative
evaluation of candidates for epilepsy surgety.

Tests and typical tasks

1. Name normal rhythm in adults in the awake with closed eyes.
a) spikes
b) alpha rhythm
c) sharp waves
d) delta rhythm
e) theta rhythm

2. Electromyography is the additional method for diagnostic of:

a) epilepsy
b) brain tumors
c) myopathies
d) meningitis
e) stroke

3. Indicate the pathology where the encephalography complexes "spike - wave" appear.
a) epilepsy
b) meningitis
c) syringobulbia
d) multiple sclerosis
e) poliomyelitis

4. What additional method provides information about the function of the brain?
a) Computed tomography
b) magnetic resonance imaging
c) electromyography
d) encephalography
e) duplex ultrasonography

60 I PART 1: General Neurology

 5. What additional method is used for the detection of stenosis or occlusion of the cerebral vessels?
a) encephalography
b) magnetic resonance imaging of the brain
c) Computed tomography of the brain
d) craniography
e) duplex ultrasonography

6.A 30-years-old woman is presented with a I week history of double vision, weakness in legs,
dizziness. Four years ago she had an episode of the right eye's vision lost for a few weeks. On
examination she has nystagmus, a pale right optic disc, increased reflexes in her legs, her gait is ataxic.
Which disease can be suspected? What additional diagnostic method can prove the diagnosis?

7. A52-years-old man is presented with a history of progressive difficulty of walking over the last 4
months. He has noticed weakness, stiffness in his right leg. During the last 2 weeks there were three
occasions, when his right leg has been jerking repeatedly for about 5 min. On examination - increased
tone and reflexes in the right leg with an extensor plantar response. Sensation is normal. Which
disease can be suspected? What additional diagnostic method is the most important in this case?

8.A 9-years-old patient has short sudden periods of consciousness loss without seizures lasting only
few seconds with a blank stare and an interruption of ongoing activity. These attacks occur many
times during the day. The patient doesn't remember attacks. Neurologic and cognitive examination
results are normal. His diagnosis is absence form of epilepsy. What additional diagnostic method is
the most important in this case? What can be seen in it?

9. A 18-years-old patient complains of weakness in distal parts of the extremities. On examination he

has a decrease of the power and reflexes in his legs and arms and sensory disorders in distal parts of
the extremities. His father has the same disease. What additional diagnostic method can prove the
diagnosis?

10. A 14-years-old patient has been suffering from partial epilepsy for 3 years. What pathological EEG
patterns may be seen in this case?

LIST OF ANSWERS

PART I. General Neurology

Chapter 1. Sensitive function and its abnormality

1. a. 2. b. 3. d. 4. c. 5. a. 6. Posterior funicles. 7. Segmental dissociative type of sensitive disorder. 8.
Loss of stereognosis. 9. Mononeural type, ulnar nerve. 10. Conductive hypesthesia or anesthesia of all
sensitivity kinds.

Chapter 2. Reflex-motor function of the nervous system. Syndromes of movement disorders

1. c. 2. e. 3. c. 4. b. 5. b. 6. Right radial nerve. 7. Peripheral monoparesis of the right hand. 8, Central left
side hemiplegia. 9. Anterior horns and lateral funiculi of the spinal cord on the level of C 5-Th,
segments. 10. Motor jackso- nian epilepsy. The upper part of the left precentral gyrus is irritated.
9. Additional melliods in neurology | 61
Chapter 3. Extrapyramidal system and syndromes of its lesion
1. a. 2. c. 3. d. 4. b. 5. e. 6. Hypertonic-hypokinetic, parkinsonism syndrome. Pallidal system is
impaired (substantia nigra). 7. Chorea, the striatic system is impaired (caudate nucleus). 8.
Hypertonic-hypokinetic, parkinsonism syndrome. 9. The pallidal system is impaired (substantia nigra).
10. Hypotonic-hyperkinetic syndrom (athetosis), the striatic system is impaired (caudate nucleus).

Chapter 4.Cerebellum and it's pathology

I. c. Z. a. 3. d. 4. b. 5. e. 6. Static ataxia. 7. Yes, there will be cortical ataxia. 8. Dynamic ataxia. 9.
Cerebellar ataxia, muscular hypotonia. 10. Cerebellar ataxia in right side.

Chapter 5. Cranial nerves pathology

1. d. 2. c. 3. a. 4. e. 5. b. 6. Cerebellopontine angle. 7. Central paresis of tongue muscles on the right
side. 8. Heteronymous bitemporal hemianopsia, reduced vision acuity, atrophy of optic nerves disks. 9.
The trigeminal nerve. Corneal, conjunctival, superciliary reflexes. 10. Peripheral paresis. Bulbar syn-
drome.

Chapter 6. Pathology of the autonomic neivous system

1. a. 2. b. 3. d. 4. e. 5. b. 6. Vegetative vessel dystonia. 7. Vago-insular attack. 8. Real urinary
incontinence. 9. A sympathy-adrenal attack 10. Hypothalamic syndrome.

Chapter 7. The brain cortex. Localization of cortical functions. Disturbances of higher cerebral
functions
1. c. 2. a. 3. d. 4. e. 5. b. 6. Motor aphasia, back areas of left inferior frontal gyrus (Broca's region). 7.
The middle part of the precentral gyrus, Broca's region in the left frontal lobe. 8. Amnestic aphasia. The
back part of the left superior temporal gyrus is damaged. 9. In the right superior parietal lobule. 10. The
left inferior parietal lobe. Apraxia.

Chapter 8. Cerebrospinal fluid.

Meningeal and hypertensive syndromes
1. c. 2. b. 3. d. 4. a. 5. e. 6. Cells — protein dissociation. 7. Non-communicating (obstructive)
hydrocephalus. 8. Serous meningitis. Lymphocyte pleocytosis. 9. Protein — cells dissociation. Tumor
of the spinal cord. 10. Neck stiffness, Kernig's and Brudzinski's signs. Irritation of the meningeal
membranes.

Chapter 9. Additional researches in neurology

1. b. 2. c. 3. a. 4. d. 5. e. 6. Multiple sclerosis. MRl of the brain. 7. Cerebral tumour. MRl or CT of the
brain. 8. EEG. During the seizure generalized three per second spike-and-wave discharges will register.
9. Electromyography. 10. Focal spike or spike-wave discharges.

62 I PART 1: General Neurology

SPECIAL NEUROLOGY

і 0. Infectious diseases of the central nervous system | 63

 10. INFECTIOUS DISEASES OF THE CENTRAL NERVOUS SYSTEM

Meningitis. Encephalitis. Myelitis. Acute poliomyelitis. Neurosyphilis

Meningitis
Meningitis is an acute infectious disease primarily affecting soft membranes of the brain and spinal
cord. Meningitis is usually primarily diagnosed by a general practitioner.
Classification. According to the etiologic classification, there are the following types of meningitis:
bacterial (meningococcal, pneumococcal, staphylococcal, tuberculous etc.); viral (parotitic, enteroviral,
etc.); caused by tungi (candidal) and protozoal.
It is practically important to divide meningitis into purulent and serous meningitis depending on the
nature of inflammation in the membranes and contents of cerebrospinal fluid. In case of purulent
meningitis it is neutrophilic pleocytosis that is predominantly found in cerebrospinal fluid, in case of se-
rous — lymphocytic pleotsytosis. This classification is widely used in clinical practice.
Depending on the pathogenesis, meningitis is classified into primary and secondary ones. Primary
meningitis develops without previous general infection or infectious lesion of any organ.
Meningococcal and enteroviral meningitis belongs to primary meningitis. Secondary meningitis occurs
as a complication of general or local infectious disease. In this case, the pathogen crosses the
blood-brain barrier and causes meningitis. Tuberculous, staphylococcal, pneumococcal meningitis and
other types of meningitis occur in such a way.
According to the clinical classification, as for the course of the disease there are such types of
meningitis: fulminant, acute, subacute, chronic, and as for the gravity — very severe, severe, moderate
and light.
There are three ways of meninges infecting: contact (perineural and lymphogenous) spread of the
pathogen onto the meninges in case of purulent processes in the areas of paranasal sinuses, the middle
ear, osteomyelitis of the skull, direct infection of cerebrospinal fluid due to open brain or spinal injuries,
hematogenous spread of the pathogen that causes secondary purulent meningitis.
Clinical presentation of various forms of acute meningitis has much in common. Meningitis can be
suspected of basing on the combination of such manifestations:
► syndrome of infectious disease;
► meningeal syndrome;
► syndrome of inflammatory changes in cerebrospinal fluid.

General infectious symptoms of meningitis are various. This can be fever, general fatigue, aching
pain in muscles, inflammatory changes in peripheral blood: leukocytosis with a shift of the formula to
the left, an increased erythrocyte sedimentation rate (ESR).
In case of purulent meningitis general infectious symptoms are acutely expressed in the first hours
and days of the disease. In case of tuberculous meningitis they are expressed not acutely, gradually
increasing. In patients with viral meningitis general infectious symptoms most definitely appear in the
first days of the disease, but rapidly disappear.
Meningeal syndrome is a complex of symptoms caused by irritation or inflammation process in the
meninges. It is observed in all types of meningitis and consists of general cerebral and meningeal
symptoms. General cerebral symptoms include: headache, vomiting, psychomotor agitation
periodically changed by weakness, impaired consciousness, and seizures. Headaches and vomiting in a
combination with fever constitute pathognomonic triad of primary manifestations of meningitis.
Observing these symptoms, a doctor of any speciality should suspect meningitis and check the presence
of actually meningeal symptoms. і 0. Infectious diseases of the central nervous system | 64
Actually meningeal symptoms are divided into general hyperesthesia and hypersensitivity of the
sense organs, reactive pain phenomena and tonic muscle tension. Manifestations of tonic muscle
tension include a stiff neck, Kernig's and Brudzinski's signs.
The syndrome of inflammatory changes in cerebrospinal fluid is crucial in diagnosing meningitis. In
case of even a slight suspicion of meningitis a lumbar puncture and cerebrospinal fluid analysis have to
be done. According to the results of analysis of cerebrospinal fluid, a conclusion about the clinical form
of meningitis can be made.
 In patients with meningitis spinal fluid flows under high pressure and has various coloring: serous
meningitis gives a transparent opalescent colour, purulent—cloudy, yellowish-green one. In case of
purulent meningitis pleocytosis is pronounced — thousands or tens of thousands of cells in 1 mcl,
mainly neutrophils. In case of serous meningitis pleocytosis is lymphocytic, tens or hundreds of cells in
1 mcl. If a decrease of cytosis accompanied increasing of protein content, this may means encapsulation
of the inflammation and the formation of brain abscess. .
The analysis of glucose in the contents of cerebrospinal fluid also has a great importance. Its marked
decrease is typical for tuberculous meningitis, but is also observed in case of cronic or subacute purulent
meningitis and ' meningitis caused by fungi.
The results of cerebrospinal fluid analysis, its cellular composition, protein and glucose level are
decisive for the diagnosis and etiotropic therapy prescription. The final etiologic diagnosis is made
according to the results of bacteriological, serological and virological analysis of cerebrospinal fluid.
The inoculation of pathogen in vital environments is also used to determine their sensitivity to
antibiotics. Immunological express methods ensure more rapid diagnostics of meningitis etiology,

Meningococcal meningitis
Clinical presentation. The incubation period of meningococcal infection is 2-6 days. The disease
begins acutely with fever up to 38-40° C, a sharp headache with irradiation to the neck and back,
vomiting that does not bring any relief. During the first or second day meningeal signs are observed. At
the beginning of the disease a patient is agitated, and then drowsiness, sopor turning into coma appears.
From the 2nd day of illness herpetic eruptions on the lips and face are observed. In severe cases of
meningococcal meningitis typical hemorrahagic rash develops. Neurological examination does not
show any focal symptoms (in case of a hard course of the disease the signs of III, IV, VII, VIII pair of
cranial nerves lesion are possible). A combination of acute beginning, fever, headache with vomiting in
the first hours of the disease makes a doctor suspect meningitis.
Cerebi o-spinal fluid flows under the high pressure and has cloudy, gray or yellowish-green coloring.
Netrophilic pleocytosis is found (up to tens of thousands of cells in 1 mcl), a high level of protein (up to
1-16 g/l). In the smears of cerebrospinal fluid meningococcus is found in 80 % of cases. It can be seen in
blood smears or washout from the posterior pharyngeal wall too.
The typical sign of meningococcal infection is the presence of haemor- rhagic necrotic rash of
different size on the trunk and lower limbs — from petechia to large hemorrhages with necrosis of the
skin. There is pronounced intoxication and hemorrhagic syndrome with hemorrhages in the mucosa of
the pharynx as well nasal, stomach, uterine bleeding and hemorrhagic in the adrenal gland.
Treatment. Antibacterial therapy should be started in the first hours of the disease — cephalosporins
of III generation, ceftriaxone — 4.0 g/day or meronem 3.0 g/day intravenous injection. All purulent
meningites require prescribing of osmotic diuretics (mannitol, laziks, and dexamethasone) and
deintoxication — reosorbilact or reopolyglucin. Treatment usually includes normalization of vital body
functions.

Secondary purulent meningitis. Pneumococcal meningitis

The pathogen of the disease is gram-positive diplococcus — extracellular pneumococcus. This
meningitis may be either primary or secondary. Most often the disease occurs as complication after
pneumonia, otitus, sinusitis, mastoiditis. The clinical course is hard. The brain tissue is often affected,
the picture of purulent meningoencephalitis can be observed. The typical sign is loss of consciousness
with the development of sopor or coma, convulsions. Theie are signs of cranial nerves lesion, focal
neurological symptoms (mono-, hemiparesis). A frequent phenomenon is swelling of brain tissue with
possible signs of wedging. Bradycardia and low blood pressure are observed. In the peripheral blood
inflammatory changes take place. Cerebrospinal fluid is very cloudy, has a greenish color, its
pathological changes are the same as in case of other purulent meningitis. The pathogen is found in
10. infectious diseases of the cential nervous system | 65
cerebrospinal fluid. The use of broad-spectrum antibiotics and sulfanilamide drugs reduced mortality
from pneumococcal meningitis. However, its treatment is complicated, requires curing of the primary
nidus of infection.
Staphylococcal and streptococcal meningitis
These rrveningites occur as complication of acute and chronic purulent processes or traumatic brain
injuries. The onset is accompanied by chills and fever to 40°С. They characterized meningeal
syndrome, impaired consciousness, and seizures. The peculiarity of staphylococcal meningitis is
frequent abscess formation, blocking of spinal routes and mycosis. The analysis of cerebrospinal fluid
— the detection of infection pathogen — is the crucial point in the diagnostics.
Treatment. In case of purulent meningitis antibacterial therapy should be started in the first hours of
the disease. Intravenous antibiotics injection is advisable, penicillin is chosen (benzylpenicillin
orampicillin sodium salt). The daily dose of benzylpenicillin is 20.000-40.000 units, the drug is injected
every 4 hours. The daily dose of ampicillin is 6-8 grams; the drug is also injected every 4 hours. Taking
into account resistance of bacteria to these drugs, it is recommended to start treatment with
cephalosporins of III generation (ceftriaxone — 4.0 g/day). Gram-negative bacteria and some strains of
pneumo- cocci are sensitive to chloramphenicolum; its dose is 3 g/day.

Serous meningitis
Serous meningitis most often has viral etiology. Its pathogens can be enteroviruses, viruses of
lymphocytic choriomeningitis, simple herpes or herpes zoster, Epstein — Barr virus, epidemic
parotiditis, tick-borne encephalitis. All of them run with a serous inflammation of the soft cerebral
membrane and are accompanied by lymphocytic pleocytosis in cerebrospinal fluid.

Tuberculous meningitis
It occurs ill patients with hematogenic disseminated tuberculosis in case of the presence of primary
tuberculous nidus in the lungs or lymph nodes. People of all ages may get sick, but the disease mostly
affects children aged 2-7 and elderly people as well as patients with immunodeficiency (including
AIDS, alcoholism, drug abuse, poor nutrition). The typical sign of tuberculous meningitis is the
formation of miliary tubercles in the meninges and sero-fibrinous exudate in the subarachnoid space.
The process is almost always localized on the basal surface of the brain, because the cranial nerves are
accustomed to pathological procces. The substance of the brain itself also often suffers.
Clinical presentation. Tuberculous meningitis has a harder course than other forms of serous
meningitis. Symptoms of the disease usually develop gradually. Development of meningeal syndrome
is preceded by prodromal period. Its duration can take up to 2-4 weeks. During this period, a patient
becomes weak, sleepy, and apathetic, he may have subfebril temperature. He quickly gets tired, loses
appetite and weight, has a recurrent headache. The intensity of these symptoms increases with time,
vomiting occurs. Gradually, signs of irritation of the meninges appear: a stiff neck and long back
muscles, Kernig's, Brudzinski's signs. The body temperature increases up to 38-39 °С. With time the
pathological process involves cranial nerves: oculomotor, facial, less frequently — visual and
vestibulocochlear ones. Vegetative disturbances are often observed: excessive sweating, changes in
pulse rate and blood pressure, hypothalamic disorders. There are also focal neurological symptoms:
pathological foot signs, central mono- or hemiparesis. The patient's condition gradually worsens,
deafening proceeds, consciousness impairs, seizures appear. Patients in bed have a characteristic
meningeal posture: the head is thrown to the back; lower limbs are bent at the knee joints.
Diagnosis of tuberculous meningitis is based on the subacute course of desease, clinical
manifestations and results of the analysis of cerebrospinal fluid. It is important to detect tuberculous
nidus, the X-ray investigation of the lungs must be carried out. Spinal fluid is colorless with a pearl
shade and (lows under high pressure. Lymphocytic pleocytosis is found (100-500 cells in 1 mm3). The
amount of glucose (up to 1-2 minol/l) and chlorides (up to 90-100 mmol/l) decreases, protein content
(up to 5-10 g/l) increases. After some hours a delicate fibrous membrane is formed in a tube with
cerebrospinal fluid. A pathogen can be detected there.
Treatment. The course of tuberculous meningitis is lengthy. Mortality reaches 10 %, mainly among
children and the elderly. In the treatment combination of three tuberculostatic drugs is used at least:
isoniazid (300-600 mg/day), rifampicin (450-600 mg), pyrazinamide (1.5-3 g/day). They all have side
effects, the main one is hepatotoxicity. In case of effective therapy after 2-3 months pyrazinamide is
revoked and treatment with isoniazid and rifampicin continues for 10-12 or more months.
Encephalitis

Encephalitis is an inflammatory lesion of the brain tissue of infectious or infectious-allergic origin.

Classification.66Depending
I PART 1: General Neurology
on the brian structure tissue which is mainly lesioned, encephalitis can be
divided into leukoencephalitis — lesion of the white matter of the brain; polioencephalitis — gray
matter of the brain is lesioned; panencephalitis — lesion of the gray arid white matter of the brain.
Polio- and panencephalitis are the most common types.
Depending on the pathogene, encephalitis can be classified as viral and microbial. As for the
localization, there exists stem, cortical, mezencephalitic and diencephalic encephalitis and as for the
course — acute, subacute, chronic, progredient-remitting encephalitis. As for the neurological clinical
presentation, the disease is divided into typical, asymptomatic, abortive, and less frequently —
fulminant encephalitis. Encephalitis is also classified into primary and secondary.
Primary encephalitis is an independent disease characterized lesion of the brain mainly by
neurotropic viruses:
► arboviral encephalitis — tick-borne encephalitis (Russian spring-summer);
► mosquito encephalitis (Japanese, Australian, American, West Nile);
► encephalitis caused by an unknown virus-lethargic;
► herpetic;
► enteroviral encephalitis (Coxsackie, ECHO).

Secondary encephalitis occurs as a complication of an existing infection in the body or local

inflammation, as a result of an open head injury, allergies. Secondary encephalitis may be
parainfectional (in case of measles, rubella, endemic parotitis, chicken pox, flu and other infectious
diseases).

Primary encephalitis Lethargic encephalitis

Etiopathoflenesis.This disease belongs to primary viral polioencephalitis caused by the unknown
pathogen. It is supposed that the patogene is a virus that is contained in the saliva and mucus of the
nasal pharynx. The disease occurs during the cold season. The virus is unstable and quickly dies in the
environment. The carriers of the virus are people who had a hidden form of the disease; infection is
spread by airborne transmission. The causative agent gets to the central nervous system from the
nasopharynx by hematogenous and lymphogenous ways.
Pathology. In the clinical presentation of disease there exist two stages — acute and chronic. The
acute stage is characterized by vascular-inflammatory, infiltrative process. The gray matter such as the
diencephalic area (hypothalamus, gray matter of the third ventricle), subcortical nuclei, brainstem
(nuclei of III pair of cranial nerves, black substance) are affected. The lesion of nervous cells is not very
definite; the changes are reversible except for the black substance where they already have a
degenerative character during the acute stage. The chronic stage is characterized by pronounced
degenerative changes in the black substance.
Clinical presentation. There are two stages of the disease: acute and chronic.
The acute phase of the disease begins with a flu-like phenomena with a temperature icrease up to
38°С. Oculolethargic syndrome is the most typical one. It is characterized by excessive sleepiness,
which is manifested by lethargy or disturbance of the form of sleep: the patient is asleep during the day
and awake at night. Oculomor disorders — diplopia, ptosis, divergent strabismus, paresis of
convergence and look up paresis, Argyll Robertson reverse syndrome — appear. The combination of
sleep disturbance, oculomotor disturbances and thermal reactions is known as Economo triad. The
characteristic vegetative disorders constitute hypei salivation, hyperhidrosis, greasiness and heperemia
of the face. Besides oculolethargic form there exist other forms of epidemic encephalitis: hyperkinetic,
ataxic, vestibular, narcoleptic, psychosensory (hallucinations, delusions), epileptiform, endocrine, and
abortive.
The acute period of epidemic encephalitis lasts a few weeks, sometimes a month or longer, most
patients after regression of symptoms can develop a chronic stage of the disease. It manifests itself by
gradually progressing Parkinson syndrome. Vegetative disorders also take place: hypersalivation,
hyperhidrosis, and greasiness of the face. Some oculomotor disorders are still preserved: convergence
weakness, look up paresis, Argyll Robertson reverse syndrome.
The diagnosis is made basing on medical history and neurological clinical data. In the acute phase of
10. infectious diseases of the cential nervous system | 67
the disease leukocytosis, an increased ESR, hypo- chromic anemia are found in patient's blood. Spinal
fluid is clear, sometimes mild lymphocytic pleocytosis is detected.
Treatment of the acute stage include interferons (laferon 3 mln I) or re- aldiron 1-3 mln U/day).
Dehydration, detoxication, symptomatic methods are used. In case of Parkinson syndrome
antiparkinsonian treatment is prescribed (see "Parkinson's disease").
Tick-borne encephalitis
Etiology. Tick-borne encephalitis is caused by virus. The carriers and containers of the virus are
Ixodes ticks. Infection is spread by transmissive and alimentary ways and differs depending on the
geographic area. The infection is transmitted by the bite of the tick in the park, forest. The carriers
of infection are female ticks, whose bite is usually painless. In the nature the rodents (field mice,
hares, hedgehogs, and some birds) can be virus reservoirs. When biting them, a tick sucks the virus
with the blood of animals, and then, biting a human, transmits him a pathogen. Alimentary way
transmission is possible when consuming raw milk of infected goats.
Pathogenesis. In case of the tick bite skin vessels are the gateway of the infection. In case of the
alimentary transmission way, virus multiplying takes place in the mucosa of the alimentary canal.
The incubation period lasts 3-30 days (more often 7-10 days).
Pathomorphology. The virus is especially tropic to motor structures of the brain — medulla
(motor nuclei of cranial nerves) and cervical part of spinal cord (anterior horns of the cervical
intumescence),
Clinical presentation. The whole period of the disease is divided into acute and chronic stages. After
the incubation period, the patient acutely develops general fatigue, muscle aches, headache, increase of
the body temperature up to 40 °С. There is a pronounced meningeal syndrome, oedema of the brain
tissue. At this stage of the disease the patient may die as a result of brain dislocation. The first period of
fever lasts 3-5 days and then after a few days the repeated increase of temperature may take place.
During the second temperature increase encephalitic symptoms appear: peripheral paralysis of neck
muscles, shoulder girdle, proximal parts of the upper extremities. A characteristic hanging head
symptom is observed — the head is hanging on the chest, the patient can not lift the hands, move them
aside. The development of bulbar syndrome with tongue muscles atrophy, disturbance of swallowing
and speech are possible. After 2 weeks paresis and atrophy of muscles increase. This is a paralytic
period of the acute stage. There are next clinical forms of acute stage: poliomyelitic, meningeal, en-
cephalitic, polyradiculoneuritic and abortive. In case of the favorable disease course a recovery stage
comes, movements are gradually restoring but the atrophy increases. This period may last 2-3 years.
The chronic stage of residual symptoms is characterized by the presence of persistent atrophic paresis
or paralysis, seizures of Kozhevnikov epilepsy. The latter are expressed by constant myoclonic
twitchings in certain groups of muscles, recurrent generalized convulsive seizures.

Diagnostics, The important role in the diagnosis of the disease is played by epidemiological data:
the patient's being in endemic focus of the infection in the spring and summer period, information about
the tick bite. Typical clinical manifestations are very important. Diagnosis of tick-borne encephalitis is
proved by serological tests results and the data of biological experiments on laboratory animals.
Treatment. The patient should be hospitalized to the infectious department as soon as possible.
During three first days of the disease serotherapy is carried out: specific antitick-borne immunoglobulin
is injected intramuscularly in the doze of 3-6 ml 2-3 times a day; prednisolone is injected in the dose of
1 mg/kg. For inhibition of viral RNA replication ribonuclease is injected intramuscularly 30 mg 6 times
a clay during 4-5 days. In addition, it is important to conduct dehydration, detoxification, maintainance
of fluid electrolyte balance. Symptomatic therapy constitutes prescription of anticonvulsant drugs, anti-
cholinesterase drugs, vitamin therapy.

Herpetic encephalitis
Herpetic encephalitis is the most common and severest form of acute encephalitis.
Biology. It is caused by viruses of simple herpes (1 or 2 type). They have tropism for tissues of
ectodermal origin (the skin, mucous membranes, nervous system). The virus can persist for a long time
in a human organism, staying in the latent state, and may get activated under certain conditions.
Encephalitis may occur at the first acquaintance to the virus or in case of exacerbation of chronic
infection. It is spread evenly around the world, does not depend on the season and affects people of all
ages.
Pathomorphology. Herpetic encephalitis is characterized by the lesion of the temporal and inferior
68 I PART 1: General Neurology
frontal lobe of the brain, where necrotic foci of hemorrhagic impregnation are observed.
Clinical presentation. The disease occurs after a prodromal period that is expressed by acute
respiratory viral infection with herpetic eruptions on the mucous membranes. Symptoms of nervous
system lesion are acutely developing. They are accompanied by moderately expressed meningeal
syndrome, which is getting worse. A loss of consciousness and coma can be observed. Focal symptoms
may vary: a central hemiparesis, hemianosmia, signs of cranial nerves lesion. The typical sign is
violation of higher cortical functions: aphasia, amnesia, change of behavior, disturbance of smell and
hearing, hallucinations, partial and generalized convulsive seizures. The course of the disease is
unfavorable. In case of the absence of treatment 80 % of patients have a quick coma with lethal
outcome. The fulminant course of the disease with the rapid development of brain oedema and
subsequent development of brain stem wedging with respiratory standstill is possible. The patients who
survived have a reverse development of symptoms with persistent residual symptoms.
Diagnosis. For making diagnosis combination of acute beginning of the disease and typical clinical
signs occurring is important, It is necessary to investigate the CSF. It flows under the high pressure, its
contents shows lymphocytic or mixed lymphocytic-neutrophilic pleocytosis, a moderate increase of
protein. The contents of glucose is mainly unchanged, in 50 % of herpetic encephalitis erythrocytes are
observed as manifestation of hemorrhagic nature of the lesion. The nature of herpetic encephalitis can
be proved with the help of a polymerase chain reaction (PCR). It allows an accurate detection of the
presence of herpes virus deoxyribonucleic acid (DNA) in cerebrospinal fluid. Changes on CT and MRl
appear not at once. A few days after the beginning of the disease changes can be detected
(hypointensive foci on CT and hyper- intensive — on T2-weighted image on MRl) in the frontotemporal
lobe of the brain, cingulated gyrus and area of the insula (Fig. 10.1).
Treatment. Although the course of herpetic encephalitis is extremely difficult, it is one of the few
variants of encephalitis which have a specific therapy.
Acyclovir (Viroleks, Zovirax) have to be prescribed which inhibits the synthesis of viral DNA. The
drug is prescribed 15 mg/kg every 8 hours intravenously with an isotonic solution during 10-14 days. In
the further treatment is changed for oral administration of the drug 500 mg twice a day during 10 days.
At the same time the antitoxic treatment, dehydration and symptomatic methods are used.

Secondary encephalitis
Flu-associated encephalitis is the infectious-allergic impairment of nervous system. It arises in acute
period of flu. Flu-associated encephalitis is caused by A1, A2, A3, В viruses. Flu virus has toxic
influence on the brain vessels, caused acute capillary toxicosis in different tissues. The lesion of the
nervous system characterizes the severity neurotoxicosis and circulation disturbances.
Clinical presentation. The disease develops on 3-4 day of flu beginning or at the end of the second
week. It starts rapidly with a higher temperature, general brain, meningeal and focal symptoms after the
flu. There is a general weakness, irritability, emotional instability. Psychotic disorders can be observed
such as hallucinations, delirium and generalized convulsive seizures in
severe cases. In some cases focal signs connected with brainstem damage: lesion of III, IV, VI, VII and
XII pairs of cranial nerves, the pyramidal tract and speech centers are involved.
In case of toxic form of the flu associated encephalitis hemorrhagic meningoencephalitis can be
developed. This form has a severe course and usually leads to patients' death. The disease starts with
general brain symptoms such as a headache, sleepiness, a loss of consciousness, phychomotor agitation.
There are changes in liquor as a lymphocytic pleocytosis, an increased protein content and blood traces
in cerebro-spinal fluid.
Treatment. Antitoxic treatment, dehydration and symptomatic methods are used as well as donor
gamma globulinum, in severe cases prednisolonum 30-60 mg intravenous, antibiotics in order to
prevent pneumonia.

Rheumatic encephalitis
In case of rheumatism, the central nervous system is involved in the pathological process along with
rheumatic, joints and heart. The main forms of rheumatic lesions of the brain are: acute rheumatic
meningoencephalitis, encephalopathy rheumatoid10. infectious diseases of the centialsmall
vasculitis, nervous system
chorea.| 69

Pathology. There is a diffuse impairment of the cerebral cortex, subcortical nodes, brainstem and
brain membranes. In the brain the pathology is characterized by vascular changes in the brain such as
endarteritis, vasculitis, periarteritis, and glial lesions.
Small Chorea
Small chorea involves subcortical nodes; there are inflammatory changes which prevail in the
striatum.
Clinic. There are general cerebral symptoms: a headache, fatigue. With time choreic hyperkinesis —
one of the main symptoms — develops. Chorea is characterized by fast polymorphic movements,
non-stereotypic chaotic involuntary movements in different muscular groups with the background of a
low muscular tonus. Grimacing and lip-smacking may be expressed. Such children are called fidgety at
school. The petulance, excessive irritability may occur. A typical symptom is appearance of Gordon-2
— freezing of a straightened leg when the knee reflex is checked. The evidence of rheumatic origin of
disease is subfebrile temperature, the presence of rheumatic carditis, arthritis, rheumatic nodules in
joints, annular erythema on the skin.
Treatment, In case of acute manifestations of rheumatic impairment of the nervous system it is
necessary to conducte specific therapy of rheumatism. Sedative therapy is used for treatment of
hyperkinesis.

Myelitis
Myelitis — inflammation of the spinal cord, usually exciting the white and gray matter.
Inflammation, limited in several segments, referred to as cross- myelitis. In diffuse myelitis
inflammation is localized at several levels of the spinal cord. The disease can be caused by neurotropic
viruses (enteroviruses, herpes viruses), Bacteria (mycobacterium tuberculosis, treponema pallidum),
neuioalergic reactions during vaccination, rarely — by fungi and protista. Clinical syndrome of acute
transverse myelitis can be the first manifestation of multiple sclerosis. Subacute necrotizing myelitis
usually occurs as paraneoplastic syndrome. Almost half the cases can not determine the cause of the
disease. Frequently myelitis inflammations are localized in the lower part of spinal cord's thoracic
section.
Clinic features. Onset can be acute or subacute usually in the background of general inflammation
manifestations: mild fever, general health deterioration, An intensive pain appears at the level of
destruction, after which paresis of the lower trunk and limbs manifests itself, conductive sensitivity
disorders and dysfunction of the pelvic organs occur, sometime there may be decubitus.
Diagnostics. Lumbar puncture determinesVotein increasing and neutrophilic pleocytosis (with
pyogenic infection) or lymphocytic one. CT or MRl usually shows no signs of lesion. Spinal stroke in
the clinical picture resembles myelitis, but the difference is marked by the absence of general
inflammatory manifestations, retaining a deep sensitivity, absence of changes in cerebrospinal fluid.
The presence of Babinsky symptoms, conducting sensitivity disorders and pelvic disorders allow to
differentiate myelitis from acute Guillain — Barre polyradiculoneuropathy. The probability of multiple
sclerosis is low at transverse myelitis with severe gross motor, sensitive and pelvic disordeis but high in
the presence of only moderate motor and sensitivity disorders.

In cases of suspected multiple sclerosis brain MRI is prescribed; it allows to detect distinctive changes
of the disease.
Current and forecast myelitis depends on its etiology and a degree of spinal cord injury. In
favourable cases a partial or nearly complete regression of neurological disorders is observed after the
acute stage.
Treatment. Prednisolone is prescribed 80-120 mg/day for 2-3 weeks followed by a gradual decrease
of the dose, or intravenous methylprednisolone 1000 mg/day for 3-5 days and then switch to oral
prednisolone. Antibiotics are prescribed in the first days of illness to prevent superinfection. Skin care
and monitoring of bladder function are needed. While identifying the causative agent (herpes virus,
tuberculosis, syphilis) is held, etiologic treatment is prescribed. During the regenerative period
therapeutic exercises are important; at severe spasticity baclofen and syrdalud can be effective.

70 I PART 1: General Neurology

 Acute Poliomyelitis
Poliovirus is one of enteroviruses; it is a picornavirus. The incidence of primary infection has been
estremely low in Ukraine since immunization began in 1957 but many patients have residual disability
following infection before the 1950s. Poliomyelitis remains endemic in the tropics, occurring especially
** in late summer and autumn. Poliovirus targets the anterior horn of the spinal cord and the motor nuclei
of the brainstem.
Clinic features. The incubation period is 10-14 days. There are a few forms:
► Asymptomatic (95 %): with resultant immunity.
► Abortive poliomyelitis (4-5 %): a self-limiting illness with gastrointestinal and mild
upper respiratory symptoms and pyrexia.
► Non-paralytic poliomyelitis (0.5 %): features of abortive poliomyelitis with meningism.
Recovery is complete.
Paralytic poliomyelitis (0.1 %). It has such stages: prodromal(1-3 days). The initial features are
fever, myalgia, meningism, gastroinstestinal disorders of this stage poliomyelitis. Then preparalic stage
develops as well as high temperature, sleepiness, meningoradicular symptoms, spasms, changes of
muscular tonus (1-3 days). There is subsequent asymmetrical peripheral paralysis with no sensory
involvement — paralytic stage. Respiratory failure is due to paralysis of the respiratory muscles. The
lower limbs are most commonly affected, especially in children. Bulbar symptoms can occur with
cranial nerve involvement. When paralytic poliomyelitis occurs before puberty, the patient is often left
with a wasted, shortened limb. During 2 years it is possible renewal stage.
Diagnosis of poliomyelitis. Paralytic poliomyelitis is distinguished clinically from Guillain —
Вапё syndrome by the lack of sensory signs and the asymmetry. CSF findings are similar to those
in other viral meningitides (raised protein, an increased number of lymphocytes, and normal
glucose). The virus may be grown from throat swabs, stool, and CSF, and paired serology will
show a rising titl e.
Treatment. Patients with paralytic poliomyelitis should be isolated. The measures include:
intramuscular injection of immunoglobulin, antibiotics for prophylaxis of pneumonia, careful
nursing, as for all paralysed patients, to prevent pressure ulcers. Physiotherapy is used to avoid
deformations.

Neurosyphilis
Syphilis is caused by the motile spirochaete Treponema pallidum. The natural history of untreated
infection is divided into three stages. Neurological involvement occurs in the third stage, which is
typically many years after the initial infection. Neurosyphilis occurs in less than 10 % of all
untreated cases. Penicillins are widely used for the treatment of other infections and thus many
unsuspected cases of syphilis are treated without progressing to stages two and three.
Classification. Neurosyphilis is divided into 2 forms: early (mesodermal) and late
(parechymatous).
There are clinical forms of early mesodermal syphilis:
1. Asymptomatic neurosyphilis.
2. Acute syphilitic meningitis.
3. Chronic basal syphilitic meningitis.
4. Meningovascular syphilis.
5. Gummy brain and the spinal cord.
Late ectodermal (parenchymatous) neurosyphilis:
1. Progressive paralysis
2. Tabes dorsalis.
3. Taboparalysi

72 I PART 1: General Neurology

Early mesodermal forms of neurosyphilis

One of the most common among all the forms of neurosyphilis is asymp- ! tomatic syphilitic
meningitis. During the long interval between the secondary j and tertiary stages of the disease,
neurosyphilis may actively persist but be asymptomatic. It is characterized by the absence of clinical
signs of meningitis. The examination of cerebrospinal fluid reveals positive syphilis serology, lym-
phocytosis (100-1000 in 1 mcl), elevated protein (0.5-2.0 g/l), and reduced glucose.The frequency
of asymptomatic neurosyphilis is 30 % from all forms of neurosyphilis. j
Acute syphilitic meningitis. It is very common among the young people in case of inadequate
treatment. There are 2 clinical forms: acute and chronic. The invariable sign of acute syphilitic
meningitis is a severe headache which appears at night, well-expressed meningeal symptoms. The
dizziness, nausea, vomiting, fear of the light are often observed. As far as the pathological process is
localized on the basal surface of the brain the cranial nervous (II, lll-VI) are surprised. There is
positive is an Argyll Robertson syndrome — the absence of the pupils reaction to the light at the
preserved reaction to convergence and ; accomodation. Such symptoms as myosis, anizokoria, pupil
deformation may ?take place. In cerebrospinal fluid there are lymphocyte pleocytosis, elevated
protein — 0.5-1:2 g/l, positive RW and glucose reducing.
The course of the disease is favourable. In case of adequate treatment the' meningeal syndrome is
liquidated in 2-3 days, CSF normalizes in 7-10 days. Very often this form can be transformed into a
chronic one.
Chronic basal syphilitic meningitis. It develops subacutely, sometimes during several weeks.
The typical features are early cranial nervous lesion: oculomotor nerve (diplopia, ptosis, anisokoria),
peripheral palsy of the facial nerve, atrophy, neuritis of the optic nerve, disorders of the
vestibule-cochlear nerve. The general cerebral and meningeal signs are also observed.
Meningovascular syphilis manifestation results in an obliterate endarteritis and periarteritis. It
can lead to brain and spinal cord infarction. The clinical presentation includes general cerebral signs,
meningeal signs and focal symptoms (hemiparesis, aphasia, sensory disturbances, and generalized
seizures). The symptoms of the disease are manifested 5-30 years after the infection. Pupil
monosyndrome is possible: anizokoria, bilateral pupils deformation, direct Argyll Robertson
syndrome.The course of the disease is very progressive.
Gummy of brain. It is a syphilitic without-vessels granuloma. It creates an individual focus of
lesion. The clinical presentation is very similar to brain tumor. The most typical place of its
localization is fossa interpeduncularis. Clinical features include: hypertensive syndrome: (a
headache, vomiting, chocked discs on the eye ground, epileptic attacks); focal signs (paralysis,
sensory disorders, aphasia, epileptic attacks, agnosia; protein-cell dissociation in cerebrospinal
fluid. The gummy brain is characterized by pupil deformation, slight . pupils reactions, the
decreased knee reflexes.

Late ectodermal forms of neurosyphilis

Talies dorsalis. Nowadays it is a rare form of neurosyphilis. Usually it is observed 15-25 years
after infection, it is the result of posterior columns and posterior roots of spinal cord degeneration.
Some of cranial nerves, paravertebral and spinal ganglions can be injured. There are 3 clinical
stages: neuralgic,
ataxic, paralytic.
Neuralgic stage is characterized by paresthesia (numbness, tingling), One of the most, main
symptoms is pain. It has a lancinating character. It is very severe and appears very sudden. Its
duration is from 1-2 seconds, sometimes longer. Usually it is the first sign of the disease. One of the
typical features is visceral crisis — an attack
10. Infectious diseasesof neurological
of lite pain
central nervous system | 73 in internal organs. The most
common are stomach crisis, also intestinal crisis, heart crisis, liver, and kidney and urine bladder
crisis.
One of the typical and early signs of tabes dorsalis is pupil monosyndrome: anizokoria, bilateral
pupils deformation, direct Argyll Robertson syndrome. Knee and Achiile reflexes are decreasing.
There are lesions of the optic nerve (primary gray atrophy), vestibule-cochlear (dizziness and
decreasing of hearing), oculomotor disorders (ptosis, strabismus), trigeminal neuralgia.
Ataxic stage. The dominating symptoms are disorders of coordination in the extremities and
unstable standing and walking. These symptoms can be explained by disturbances of deep sensation
 because the posterior funicules'* are damaged. Usually the symptoms of lesion dominate in the
lower extremities. The patients feel the flour badly. It is difficult for him to walk in darkness or witli
his eyes closed. The muscle tonus is decreased.
Paralytic stage. It is characterized by severe disorders of motor function. But because of severe
sensitive ataxia and full deafferentiation the patients are not able to stand, walk and even to use their
extremities for the fulfillment of any actions. Thus, that is why the patient looks like paralyzed. The
most common symptoms are: trophic and pelvic disorders (retention of urine and feces, sometimes
incontinence of urine and feces), trophic skin disorders. The possible psychological disturbances;
changes of personality, memory disturbances.
Sometimes tabes dorsalis is associated with progressive paralysis. The last manifests itself as
memory disturbances, insomnia, and euphoria. There are also speech, writing and reading disorders.
This disease is known as taboparalysis.

Progressive paralysis
It is developed 10-20 years after syphilis infection. The main clinical features are memory and
personality disturbances, a loss of acquired skills, positive Argyll Robertson test, paresis of the
extrimities, pelvic disorders, epileptic attacks. There are also an increased protein content (0.45-0.6
g/l), positive Wasserman test in CSF.
Diagnostic. Additional methods of neurosyphilis diagnosis: positive Wasserman test in blood and
CSF; lymphocyte pleocytosis (50-100 cell in 1 rncl) and increased protein content (from 0.5 to 1.5 g/l)
in CSF at meningeal forms. There are also important serologic reactions, such as the reaction of
immobilization of Treponema pallidum, the reaction of immunofluorescention in blood and CSF.
Treponema pallidum DNA can be detected in CSF of patients by the polymerase chain reaction
(sensitivity 60 %).
Treatment. The treatment of neurosyphilis begins with the treatment of early syphilis. Usually
pfenicillin is used as standard treatment. It can be introduced in intravenous or intramuscular
introduction from 2.4 up to 24 mln units (U) per day during 10-14 days. Then 2.4 mln U of Retarpen or
Benzatin- Benzylpenicillin are used once a week during 3 weeks.
The effectiveness of treatment is determined according to the blood tests and CSF examination.
That's why lumbar puncture is made just after penicillin treatment and then every 3 months.

Lyme disease (neurohorreliosis)

The causative agent in Lyme disease is the spirochaete Borrelia burgdorferi, which is transmitted by
the tick Ixodes dammini. The organism is prevalent throughout Europe and North America (e.g. Lyme,
Connecticut, where the disease was first recognized).
Clinical presentation. The clinical course of Lyme disease can be divided into three stages. Stage 1
begins 3-30 days after the tick bite and consists of a relapsing-remitting pyrexia and arthralgia, with a
typical skin lesion (erythema chronicum migrans) developing at the site of the bite. This stage resolves
after about 4 weeks.
Stage 2 occurs a few weeks or months after stage 1 and. consists of neurological (15 %) or cardiac
symptoms (10 %), which can last up to 8 weeks. Neurological manifestations include:
► Subacute lymphocytic meningitis: often mild and self-limiting but can recur if not treated.
► Subacute encephalitis: often mild and self-limiting.
► Cranial nerve involvement.
► Peripheral neuropathy with painful radiculitis.

A patient 74that
I PART
is1:presented
General Neurology
with a unilateral or bilateral periferal palsy of VII cranial nerve, a rash and
a systemic upset, e.g. fever should always be becon- sidered to have possible Lyme disease.
Stage 3 occurs several months or years later and consists of recurrent and often eiosive arthritis.
Signs of diffuse CNS involvement may also develop, with focal encephalitis, seizures, behavioural
disorder and a multiple-sclerosis-like illness.
Diagnosis. Clinical features and epidemiological consideration are indicative. Serological and PCR
techniques give the best probability of making the diagnosis.
Treatment. Treatment comprises oral antibiotics (penicillin or tetracycline) at beginning of desease
with following high-dose intravenous penicillin or cef- triacsone for 14 days in stage 2 and 3. This
shortens the course of neurological illness and prevents further parenchymal damage.
HlVassociated nervous system disorders
The causative agent of AIDS is human immunodeficiency virus which belongs to the family of
retroviruses. The virus is transmitted through sexual contact, particularly among male homosexuals;
parenteral^, when using non sterile syringes for injection (drug addicts, patients not following asepsis
rules) and in case of blood transfusion and transfusion of drugs made of the blood of infected people.
Pathomorphologic studies show that 90 % of AIDS patients have nervous system lesions, although
only 50-70 % of cases show clinical neurologic complications. In 10 % of the patients nervous system
lesions are the first clinical manifestations of the disease.
HIV affects the structures of the central nervous system directly, penetrating perineurally into glial
cells and from endothelial cells of cerebral capillaries which carry antigen CD4 + on the membrane.
Classification. HIV- and AIDS-associated nervous system lesions can be both primary — with HIV
infection affecting the nervous system directly, and secondary — being caused by opportunistic
infections and tumors that develop in patients with AIDS in case of immunodeficiency. In the latter
case focal neurological disorders are caused mainly by three factors: toxoplasmosis, primary
lymphoma and progressive multifocal encephalopathy.
Primary HIV-Related Nervous System Disorders:
► HIV-associated dementia;
► aseptic meningitis;
► HIV-associated vacuolar myelopathies;
► vascular neuroAIDS;
► HIV-associated neuropathies: inflammatory demyelinating polyneuropathy (Guillain — Barre
syndrome), chronic inflammatory demyelinating polyneuropathy.

Secondary HIV-Related Nervous System Disorders:

► Progressive multifocal leukoencephalopathy (PML).
► Toxoplasmatic encephalitis.
► Gerpetic encephalitis.
► Cytomegalovirus (CMV) encephalitis.
► Cryptococcal meningitis.
► CNS lymphoma and Kaposi's sarcoma.

HIV-associated dementia. The morphological substrate of HIV encephalopathy is a primary

lesion of the white matter of the cerebral hemispheres of the brain of the inflammatory and
demyelinating nature, mainly in subcortical structures, caused by multifocal giant cell encephalitis and
progressive diffuse leukoencephalopathy.
The clinical presentation of HIV encephalopathy is characterized by a triad of syndromes: behavioral
changes, cognitive and movement disorders. At the initial stage of the disease a loss of memory,
impaired concentration, slowing and weakening of intellectual activity are detected. Hyperreflexia,
symptoms of oral automatism, tremor and signs of mild ataxia, hipomimia are observed in the
neurological status.
Clear impairments of cognitive function, stiffness, increased drowsiness, negligence, apathy are
gradually appearing, movement disorders (ataxia, hyperkinesis, central paresis) are getting worse.
Convulsive seizures, pelvic disorders may also take place.
The typical clinical and instrumental criteria for diagnosis of HIV encephalopathy ate: in case of CT
or MRl study — cortex atrophy with the widening of subarachnoid spaces and ventricles of the brain,
detection of the subcortical focus in the frontal and parietal lobes. MRl in T2-weighted regime shows
symmetrical75diffuse or multifocal
I PART 2: Special Neurology hyperintensive white matter changes in the periventricular area and
semioval centre not having a mass effect and not accumulating contrast. Investigation of liquor shows
no pathological changes, but in 20 % of cases slight lymphocytic pleocytosis (up to 50 cells in 1 ml), a
moderate increase of protein (500-1000 mg/l), the high titer of antibodies to HIV are observed.
HlV-associated meningitis. The diagnosis of meningitis is based on three syndromes: general
infectious syndrome, membranous syndrome, inflammatory changes of cerebrospinal fluid. However,
in most cases atypical variants of HlV-associated meningitis are observed.
The clinical presentation Is usually not clear, may manifest itself only through a headache of a
hypertension-liquor character. It often camouflages itself as post-flu encephalitis or migraine-like
attacks.
 HlV-associated vacuolar myelopathy may occur independently or combined with AIDS dementia.
Pathomorphological signs are demyelination and spongioid degeneration of the spinal cord, mainly in
the lateral and posterior funiculi on the level of middle and lower thoracic spinal cord segments,
Vacuolar myelopathy is characterized by slowly progressing spastic paraparesis with high tendon
reflexes, pathological foot signs, sensitive ataxia, sensitivity disorders of conductive type with the upper
limit corresponding to the affected segment. Functional disorders of pelvic organs of the central type are
also typical. MRI investigation shows atrophy and area of an increased signal on T2-weighted regime
on the level of the thoracic part of the spinal cord with or without involvement of the cervical part.
Vascular neuro-AIDS. Virus-induced vasculitis of the brain and spinal cord may be observed in
case of neuro-AIDS with subsequent thrombosis development and brain infarction.
Clinical presentation of ischemic strokes caused by neuro-AIDS is characterized by a wavelike
course. The development of acute ischemic stroke is often preceded by transient ischemic attacks.
Repeated ischemic strokes with the appearance of new focal symptoms develop as a result of multiple
vascular lesions. Ischemic strokes in vertebrobasilar system with a severe clinical course are rather
frequently observed. Neurological symptoms are accompanied by consciousness impairments,
alternating syndromes, tetraparesis, and cerebellar ataxia. HIV-infected people often have
transformation of ischemic stroke into hemorrhagic stroke.
Hemorrhagic strokes are characterized by a sudden onset, severe course. Bleeding in the cerebellum
is possible. It increases the risk of intracerebral hemorrhage of thrombocytopenia which frequently
develops in case of AIDS.
Inflammatory polyneuropathy. Patients suffer from numbness, burning pain, paresthesia in the
feet, aggravated by the slightest touch. Such sensitivity disorders are called "burnt foot." The pain gets
worse at night and relieves when putting the legs in cold water. Neurological status shows hypesthesia
of surface sensitivity of "gloves" and "socks" type, a decrease of vibration sensitivity to 5-6 sec.,
hyporeflexia of Achilles reflexes. Knee reflexes are usually preserved. Sometimes there is atrophy,of
small muscles ot foot, trophic and vasomotor impairments in the lower extremities. Some patients have
dominant manifestations of vegetative insufficiency: orthostatic hypotension, liability of pulse rate,
cardiac arrhythmia. Electroneuromyography data detects signs of axonal polyneuropathy.
Other types of peripheral nervous system lesion are also possible — such as acute inflammatory
demyelinating polyneuropathy of Guillain — Barre syndrome type, chronic inflammatory
demyelinating polyneuropathy, neuropathy of the facial nerve,
encephalomyelopolyradiculoneuropathy.
Progressive multifocal leucoencephalopathy (PML). This is a demyelinating disease of the
nervous system, caused mainly by papovavirus JC with replication that takes place in the situation of
immunosuppression. Clinical manifestations of progressive multifocal leukoencephalopathy are
headache, dementia, aphasia, hyperkinesias, progressive paresis, sensory disorders, and epileptiform
attacks. The course is rapidly progressive; the death comes after 6-9 months.
Diagnosis is based on the results of paraclinical examinations: moderate pleocytosis in cerebrospinal
fluid, papovavirus JC identification with the help of the polymerase chain reaction (PCR), CT or MRl
of the brain showing single or multiple foci of low density in deep white matter parts with a serrated
contour, with the predominant localization in the parietal-occipital area. The typical sign is not forming
a mass effect and not accumulating contrast.
Toxoplasmatic encephalitis is the most common cause of mass lesions in patients with AIDS. The
onset of the disease may be acute or subacute in the form of focal or generalized seizures (in one third of
patients). The development of focal neurological symptoms — aphasia, cranial neuropathy, hemi-
paresis, sensory and motor disorders combined with pronounced cerebral symptoms (headache,
disorientation, and consciousness confusion) is very important. CMS lesions are preceded or go
76 I PART 2: Special Neurology
simultaneously with developing lesions of the eye (uveit, focal necrotizing chorioretinitis, and
papillitis) which run without obvious inflammatory reactions.
Diagnosis of toxoplasmatic encephalitis is based on detection of toxoplasma DNA in blood and
liquor with the help of PCR and neurovisualization methods: CT or MRl of the brain. CT or MRl of the
brain show multiple microfocal or ring-shaped foci with peripheral swelling, mainly located in the area
of the basal ganglia and frontal-parietal areas. These foci have a mass effect and can accumulate
contrast in the form of a thin rim in the focal periphery. Gerpetic and cytomegalovirus (CMV)
encephalites are described in infectious diseases.
Cryptococcal meningitis (meningoencephalitis) usually occurs in case of severe
immunodeficiency. The disease has an acute or subacute onset with a headache, fever, nausea,
photophobia, consciousness confusion, loss of weight, fever, temperature increase up to 37.5-38.0°C.
The temperature can last for a long time, periodically fluctuating from a low to a high level. At the same
time there are somatic manifestations (pneumonia, urinary bladder infection, skin lesions). The later
stages of the disease are characterized by convulsive seizures, mental disorders, impaired
consciousness, and cranial nerves lesions.
Neoplasms of the CNS. Primary lymphomas are the most spread. They are found in 2 % of cases,
particularly in a situation of profound immunosuppression. The initial symptoms reflect single or
multiple lesions of the brain parenchyma and intracranial hypertension. A variety of clinical
symptoms is determined by. localization of the process. The typical signs are consciousness
confusion, torpor, a loss of memory, behavior disorders, lesions of cranial nerves, hemiparesis,
aphasia, convulsive seizures.
Diagnosis is based on CT or MRI data, mass lesions .with perofical swelling, the presence of
hyperintensive signal on T2-weighted images and a shift of median brain structures are detected.
Stagnant discs of the optic nerve are observed in the process of fundus investigation.
Treatment. Neuro-AIDS treatment should have a complex pathogenetic character, include fully
treatment of the AIDS and consider the features of central and peripheral nervous system lesions. In
case of primary AIDS specific highly active antiretroviral therapy (HAART), inhibiting the disease
progression and temporarily stabilizing the patient's condition, is prescribed. Today about 20
HAART medicines are used. They include two groups of drugs: (
► drugs that inhibit HIV enzyme-revertase (zidovudine, zaltcitabine, didan- j osine, abacavir,
stavudine, etc.);
► drugs that inhibit another viral enzyme-protease (amprenavir, indinavir, > і and ritovir). A
simultaneous use of different combinations that consists •' of 3 or more drugs steadily reduces the
concentration of virus in the • j blood, increases the number of T-lymphocytes with CD4 +
phenotype in .'1 the blood, helps to reduce the rate of viral replication.
Treatment of secondary neuro-AIDS is based on the use of the specific therapy of nervous system
lesions caused by opportunistic infections.
In case of toxoplasmatic encephalitis pyrimethamine (15-150 mg/day) and sulfadiazine (2-4
mg/day in 4 doses) or clindamycin 1200 mg at a time during 6 weeks, then 600 mg per day lifelong,
are used. Clarithromycin or rovamycine are alternative drugs for clindamycin.
In case of cryptococcal meningitis amphotericin В 0.5-1.0 mg/kg is used for 2 weeks at the same
time with flucytosine 0.5 mg/kg per day intravenously, with further change for diflucan 400 mg/day
for 10-12 weeks.
Tests and typical tasks

1. What are the signs of meningococcal meningitis?

a) fibrillary tremor In muscles, bulbar disturbances
b) paraplegia, fibrillar twitching
c) protein-cellular dissociation in CSF, bulbar disturbances
d) headache, hyperthermia, Kerning's sign
e) paresis of extremities, hyperkinesis

2. What are the features of tuberculous meningitis?

a) respiratory and cardial malfunction, bulbar disturbances
b) lymphocytic pleocytosis, fibrin membrane in CSF
c) neutrophilic pleocytosis, paresis of the extremities
d) positive Wassermann reaction, sensitive disorders
e) protein cellular dissociation in10.
CSF, paraplegia
Infectious diseases of lite central nervous system | 77

3. What are the characteristics of epidemic encephalitis in acute form?

f) pathological somnolence, oculomotor disturbances
g) bulbar disturbances, paralyses of the extremities
h) high muscular tone, hypokinesis
i) low muscle tone, hyperkinesis
j) mannequin's postur e, hypomimia
 4. What are the characteristics of tick-borne encephalitis?
1
a) pathological somnolence , oculomotor disturbances
b) hypomimia, hypokinesis
c) sensitive disorders, ataxia
d) conductive hemihypeshesia, spastic hemiplegia
e) flaccid pareses of upper extremities, bulbar disturbances

5. Indicate main clinical features of tabes dorsalis.

a) bulbar disturbances. Bernard — Homer syndrom
b) meningeal symptoms, papilledema
c) pathological somnolence, oculomotor disturbances
d) sensitive ataxia, the absence of the knee and Achilles reflexes
e) paraplegia, fibrillar twitching
6. A three-year-old child has been treated for purulent otitis for a week. Suddenly the state of the child
became worse. Vomiting, headache, fever, rise in temperature up to;39"C appeared. There was
detected rigidity of the muscles of the back of the head, Kering's symptom on the both sides, general
hypersthesia. Which disease can be suspected?
7. After acute nasopharyngitis through 7 days a twenty-year-old patient had a high temperature 40 'C,
severe headache, vomiting. During the second day meningeal sings and herpetic rash on the lips are
observed Neurological examination does not show any focal symptoms.. The examination of
cerebro-spinal fluid showed that it is cloudy, neutrophil pleocytosis - 5 thousands of cells in 1 mcl,
protein 3 g/l. What is the previous diagnosis?
8. A 25-year-old patient, who works as a lumber-man, at the end of May had chill, high body
temperature, headache. On the 6,h day he also had hiccough, weakness of arms, his head fell on the
chest. In the neurologic status paresis of the arms with atony of muscles and areflexion are detected.
The head is drooping. What is the previous diagnosis? What is the form of disease?
9. A 36-year-old patient with HIV suddenly felt a sharp headache, vomiting; temperature rose up to 40
degrees; roseola rash on the body, lympadenopathy, hepa- tolienal syndrome were occurred.
Neurologocal status: central paresis of mimic muscles and hemiparesis on the right side, meningeal
symptoms. Craniogram shows areas of calcination, positive test with toxoplasmin. What is the
preliminary diagnosis? What examination should be carried out?
10. After tonsillitis a ten-year-old child had general cerebral symptoms: a headache, fatigue. In
neurological status fast polymorphic movements, non-stereotypic chaotic involuntary movements in
different muscular groups against the background of a low muscular tonus were detected Grimacing
and lip-smacking were expressed. The doctor detected freezing of the straightened leg during the
knee reflex check. Which disease can be suspected?

1(52 І PART 2: Special Neurology

 11. CEREBRAL VASCULAR DISEASES. SLOWLY PROGRESSING AND TRANSIENT
DISTURBANCES OF CEREBRAL BLOOD CIRCULATION. BRAIN STROKE

Cerebrovascular disease (CVD), including stroke, is the third leading cause of death in Ukraine and
the leading cause of disability among senior people. Cerebrovascular disease occurs when the blood
vessels supplying the brain with oxygenated blood are damaged or their function is compromised. If the
blood flow is severely restricted, depriving the brain of adequate oxygen even briefly, a stroke can
occur. It has been estimated that every 45 seconds, one person suffers from a stroke, often with
debilitating consequences or even death. One of four men and one of five women over the age of 45
would suffer a stroke.

Anatomy of the cerebral vascular system

Four arteries supply the brain almost exclusively: two internal carotids and two vertebral arteries. The
contributions of blood flow to the brain of these systems in the adult human brain are approximately
three fourths of the total for the carotids, and one fourth for the vertebrals. These vessels originate from
branches stemming out of the aortic arch. Internal carotid and vertebrobasilar arterial systems connect at
the base of the brain by arterial anastomosis and form Circle of Willis (Fig. 11.1). The arrangement of
the brain's arteries into the Circle of Willis creates redundancies or collaterals in the cerebral circulation.
If one part of the circle becomes blocked or stenosed or one of the arteries supplying the circle is
blocked or narrowed, the blood flow from other blood vessels can often preserve cerebral perfusion well
enough to avoid symptoms of ischemia.

Etiology of CVD
Atherosclerosis, arterial hypertension (> 140 mm Hg systolic, > 90 mm Hg diastolic), a combination
of atherosclerosis and arterial hypertension, vasculitis, diabetes mellitus, blood diseases, elevated
plasma fibrinogen, degenera tive changes in the upper cervical spinal cord, heart and vascular pathology
(atrial fibrillation, valvular heart disease, mitral valve prolapse, myocardial infarction, carotid stenosis),
obesity.
Risk Factors for CVD
The risk of stroke increases with age and is higher in men than in women at any age. Risk factors of
CVD include: hyperlipoproteinemia (total cholesterol > 5.0 mmol/l, Low-density lipoprotein (LDL) > 3
mmol/l, High-density lipoprotein (HDL) < 0.9-1.2 minol/l), cigarette smoking, alcohol abuse (> 60 g of
alcohol per day in men, > 40 g in women), drug abuse (amphetamines, heroin, cocaine), sedentary
lifestyle.

Slowly progressing disturbances of cerebral blood circulation (GBC)

Slowly progressing cerebral circulatory disorders of CBC, which lead to gradually increasing diffuse
structural changes associated with cerebral dysfunctions, is also called white matter disease and refers
to a finding on an MRl. The main role in the etiology is assigned to atherosclerosis, arterial hyperten-
sion or their combination, angiocoagulopathies, heart diseases.
Clinical symptoms of slowly progressing cerebral circulatory disorders: headache, vertigo, noise in
the head, altered memory, a decreased mental efficiency and white matter changes on MRI scan.

Treatment. The traditional approach to the treatment of patients with slowly progressing cerebral
circulatory disorders of CBC is the prescription of vasoactive (pentoxifylline — 300 mg/day
intravenous infusion) and nootropic agents (pyracetamum — 20% — 10.0 ml/day intravenous infusion,
citico- 11.
lineCerebral vascular diseases. Slowly progressing and transient disturbances of cerebral blood ... | 79
— 500-1000 mg/day per os or intravenous infusion) in the context of secondary prophylaxis
of worsening in chronical cerebrum diseases.
Transient ischemic attacks (TIA)
A transient ischemic attack is defined as acute focal neurological deficit lasting less than 24
hours. Attacks are usually much shorter, most episodes clearing within 1 hour, only 5 % last longer
than 12 hours. Miller Fisher first described the phenomenology of TIAs as "prodromal fleeting attacks
of paralysis, numbness, tingling, speechlessness, unilateral blindness or dizziness," which preceded
cerebral infarction in patients with the occlusion of the internal carotid artery (ICA).

Signs and symptoms

Transient ischemic attacks usually last a few minutes. Most signs and symptoms disappear within an
hour. The signs and symptoms of TIA resemble those found early in a stroke and may include:
► Sudden weakness, numbness or paralysis in the face, arm or leg, typically on one side of the
body.
► Slurred or garbled speech or difficulty in understanding others.
► Sudden blindness in one or both eyes or double vision.
► Dizziness, a loss of balance or coordination.

Diagnostic tests
All patients with possible TIA should receive a detailed, documented neurologic examination, with
emphasis on cognitive and language function, cranial nerve function, facial and limb strength, sensory
function, deep tendon reflex symmetry, and coordination. This examination can be helpful in
determining whether a patient previously had an unrecognized stroke. It also can serve as a baseline
examination if the patient's neurologic status worsens or neurologic symptoms recur. All patients
should have a baseline electrocardiogram (ECG). A complete blood count with a platelet count should
be obtained to rule out polycythemia; thrombocytopenia, and thrombocytosis. It is helpful to know the
prothrombin time (PT), activated partial thromboplastin time (aPTT), and International Normalized
Ratio (INR) before antiplatelet or anticoagulation therapy is administered; the PT, aPTT, and INR can
be elevated in some hypercoagulable states. The glucose level should be determined to rule out
hypoglycemia or hyperglycemia. Carotid duplex ultrasonography should be performed in a reliable
laboratory, preferably one with validation against the results of cerebral angiography. Alternatively,
cerebral and cervical vessels can be evaluated by magnetic resonance angiography (MRA) with a
contrast medium or by Computed tomography (CT) angiography.

TIA Treatment principles

Ongoing treatment will focus on preventing another TIA or stroke and reducing additional risk
factors for stroke. This may include reducing high blood pressure, the most common risk factor for
stroke, by making changes to the patient's diet and taking blood pressure-lowering medicines. Patients
were recommended taking aspirin (75-100 mg/day) or another antiplatelet medicine (clopidogrel — 75
mg/day) to prevent strokes. It has been shown that people who have had a stroke, a TIA, or an
endarterectomy may benefit from taking aspirin or another antiplatelet medicine, such as aspirin with
extended-release dipyridamole, daily to prevent another stroke. Patients were recommended taking an
anticoagulant medicine, which is commonly called a blood thinner, if a patient have atrial fibrillation,
taking statins (Atorvastatin —10-40 mg/day). Statins can reduce the risk of stroke in people who have
had a TIA. Control diabetes mellitus is required.
A patient who has TIA also needs to make lifestyle modification:
► quitting smoking and avoiding secondhand smoke. People who smoke have a higher risk of
stroke than those who quit;
► maintaining a healthy weight. Being overweight increases risk of developing high blood
pressure, heart problems, and diabetes, which are risk factors for TIA and stroke;
> eating a balanced diet that is low in cholesterol, saturated fats, and salt. Fatty foods may make
hardening of the arteries worse. Increase fruits and vegetables in the diet;
► getting
1(52 Іregular
PART 2: Special Neurology Physical activity significantly lowers a risk of stroke.
exercise

Brain stroke
A stroke is an acute focal or global impairment of brain function resulting from a pathological process
of blood vessels (thrombus, embolus, vessel rupture). Stroke is a major public health problem, ranking
among the top three causes ot death in most countries. It affects the brains of almost a half million
people every year, causing 150.000 deaths. Stroke mortality rises exponentially with age, virtually
doubling every 5 years and death rates are higher among African-Americans.
There are two main kinds of stroke. The most common, an ischemic Stroke, occurs when the artery in
the brain is blocked by a blood clot, usually because of atherosclerosis (the deposition of a plaque on the
inside of artery walls) or cardiogenic embolism. Alternatively, a hemorrhagic stroke can occur when a
portion of the arterial wall weakens and bursts.

Ischemic Stroke
Ischemic stroke is responsible for, 80 percent of all strokes. There are two kinds of ischemic stroke.
The first, a thrombotic stroke, results from a blood clot (thrombus) forming in a vessel inside the brain
and cutting off the blood supply to the tissues served by that vessel.
The second; an embolic stroke, occurs when a clot forms somewhere else in the body, breaks off, and
travels to the brain. The clot can originate in the peripheral artery, in the heart itself, or in the arteries in
the neck or brain. Among people with an abnormal heart rhythm called atrial fibrillation, clots can arise
in the left atrium and travel through the left side of the heart and the aorta and into the brain. When the
clot becomes lodged in the artery, the tissue beyond the blockage is starved of oxygen and begins to die.
Pathophysiology of Ischemic Stroke
Hemodynamic insufficiency: Cerebrovascular autoregulation is normally able to maintain a
relatively constant cerebral blood flow (CBF) of 50-60 mi/ 100 g brain tfssue/mln as long as the mean
arterial pressure (MAP) remains within the range of 50-150 mm Hg. If the MAP falls below 50 mm Hg,
and in certain pathological states (ischemia), autoregulation fails and CBF declines. Major neurological
deficits arise only when CBF falls below the critical ischemia thi eshold — 20 ml/100 g/min.
Hypoperfusion: If adequate CBF is not reestablished, clinically evident neurological dysfunction
ensues — breakdown of cerebral metabolism. Prolonged, severe depression of CBF below the
infarction threshold of 8-10 ml/ 100 g/min causes progressive and irreversible abolition of all cellular
metabolic processes and necrosis. Infarction occurs where hypoperfusion is most severe. The area of
tissue surrounding the zone of infarction in which the CBF lies between the thresholds for ischemia and
infarction is called the ischemic penumbra. Brain tissue in the zone of infarction is irretrievably lost,
while that in the ischemic penumbra is at risk, but potentially recoverable. The longer the ischemia lasts,
the more likely infarction will occur; thus, time is brain.

Stroke clinical forms

1. A completed stroke, which can cause neurological deficits, that are either:
► A minor stroke, when deficits disappear during 21 days
► A major stroke (disabling stroke)
2. A stroke in evolution— a progressive course of stroke development.

Infarct Types Lacunar infarcts ("small deep infarcts")

Lacunes are small (< 1.5 cm in diameter), round or oval infarcts in the subcortical, periventricular
region or brain stem. Classic lacunar syndromes include: purely motor hemiparesis (internal capsule,
corona radiata, pons), contralateral purely sensory deficit (thalamus, internal capsule), ataxic hemi-
paresis (internal capsule, corona radiata, pons), dysarthria witli clumsiness of one hand (clumsy
hand-dysarthria syndrome; internal capsule, pons). The presence of multiple supratentoi ial and
infratentorial lacunes is termed the lacunar state mi is clinically characterized by:
► pseudobulbar palsy;
► small-step gait ("marche a petit pas");
► urinary incontinence;
► and
11. Cerebral vascular diseases.
affective Slowly progressing
disorders (compulsiveand transientcrying).
disturbances of cerebral blood ... | 81

Territorial infarcts are those limited to the distribution of the primary cerebral arteries (CA) —
anterior, medial or posterior (АСА, MCA or PCA). With the exception of striatocapsular infarcts (the
internal capsule, basal ganglia), these infarcts are predominantly cortical. Embolic territorial infarcts
often undergo secondary hemorrhage ("hemorrhagic conversion, hemorrhagic infarction").
 End zone infarcts. Low-flow infarction in the subcortical white matter is due to extracranial
high-grade vessel stenosis and/or inadequate collateral flow.
Border zone infarcts also result from hemodynamic disturbances due to microangiopathy. They are
found at the interface ("watershed") between adjacent vascular territories, and can be either anterior
(MCA-ACA t> contralateral hemiparesis and hemisensory deficit, mainly in the lower limb and sparing
the face, with or without aphasia) or posterior (MCA-PCA P contralateral hemianopsia and cortical
sensory deficit, with or without aphasia).

Stroke Symptoms
The FAST test is an easy way to recognize and remember the most common signs of stroke or TIA.
The FAST test identifies up to 89 % of strokes and TIA's and is recommended by the National Stroke
Association. Each one of the first three letters in FAST stands for a word which patient can use to
uncover a symptom of stroke. Using the FAST test involves asking three simple questions:
► Facial weakness — can the person smile; has his mouth or eyes drooped on one side?
► Arm weakness — can the person raise both arms?
► Speech difficulty — can the person speak clearly and understand what you say?
► Time to act — act FAST and call for an ambulance immediately.
Paralysis or weakness. Paralysis, weakness, and tiredness are the most common effects from stroke.
These effects may involve one side of the body or just the face or an arm or leg. A survivor also may lose
the ability to recognize the need to urinate or to control bladder or bowel muscles, which can result in
not getting to the toilet in time. Constipation can also occur. Incontinence problems are usually
temporary, but they can be emotionally distressing.
Aphasia. At least 25 percent of all stroke suivivors lose the ability to speak, write, or understand
spoken or written language. This,condition can be improved with therapy.
Spatial perception, thinking, and memory. Stroke can damage the areas of the brain that control
memory, spatial relationships, learning, and awareness. Survivors may have significantly shortened
attention spans or find short-term memory problematic. They may lose the ability to learn new tasks,
follow a set of instructions, make plans, or carry out actions in sequential steps.
Mental health changes. Depression, personality changes, and trouble controlling emotions are
common after stroke because of the debilitating emotional effect of the trauma. Strokes can also damage
the frontal cortex and other parts of the brain involved with emotion. Post-stroke depression usually
responds well to antidepressant medications and psychological counseling.
Additional symptoms: trouble seeing in one or both eyes, a loss of balance or coordination, a severe
headache with no known cause.

Stroke Syndromes
Internal Carotid Artery (ICA). Territorial infarcts affect the middle cerebral artery (MCA) more
often than the anterior cerebral artery (АСА). The symptoms includeifjblindness in the ipsilateral eye,
impairment of consciousness, contralateral hemiplegia and hemisensory deficit, homonymous
hemianopsia, conjugate gaze deviation to the side of the lesion, partial Horner syndrome, ICA infarcts in
the dominant hemisphere produce global aphasia.
Anterior cerebral artery (АСА). Contralateral hemiparesis is usually more distal than proximal,
and more prominent in the lower than in the upper limb (sometimes only in the lower limb).
Middle cerebral artery (MCA). Main trunk occlusion produces: contralateral hemiparesis or
hemiplegia with a corresponding hemisensory deficit, homonymous hemianopsia and global aphasia
(the dominant side) or contralateral hemineglect with limb apraxia (the non-dominant side). Occlusion
of the posterior main branch produces Wernicke's or global aphasia (the dominant side) or apraxia and
dyscalculia (the non-dominant side). Anterior branch occlusion on the dominant side additionally
produces Broca's aphasia.
Vertebral Artery-(VA) occlusion produces variable combinations of symptoms and signs,
including: homonymous hemianopsia, dysarthria, dysphagia, unilateral or bilateral limb paralysis with
1(52 І PART 2: Special Neurology
or without sensory deficit, ataxia, drop attacks (due to medullary ischemia), impairment of
consciousness.
Basilar artery (BA) occlusion. Thrombotic occlusion of the BA may be heralded several days by
nonspecific symptoms (unsteadiness, dysarthria, headache, mental changes). BA occlusion causes
impairment of consciousness, ranging from somnolence to coma, mental syndromes (hallucinations,
confabulation, psychosis), quadriparesis, and oculomotor disorders (diplopia, vertical or horizontal gaze
palsy). Pontine infarction produces quadriplegia and mutism with preservation of sensory function and
vertical eye movements (locked-in syndrome).

Ischemic stroke diagnostics

In Table 11.1 lists initial diagnostic checkups recommended by current guidelines for patients with
suspected stroke. These studies help exclude stroke mimics, uncover critical comorbidities (e.g.,
myocardial ischemia), and establish the safety of thrombolytic therapy.
In patients with suspected stroke recommended urgent cranial CT (Fig. 11.2) or alternatively MRI is
recommended. If MRI is used, the inclusion of diffusion weighted imaging (DWI) and T2-weighted
gradient echo sequences are recommended. In patients with TIA, a minor stroke or early spontaneous
recovery immediate diagnostic work-up, including urgent vascular imaging (ultrasound,
CT-angiography, or MR angiography) is recommended.

Patients admitted within 3 hours of a stroke onset may be candidates for intravenous thrombolysis;
CT is usually sutficient to guide routine thrombolysis. Carotid ultrasound, MRA and СТА visualize
carotid stenosis. Cardiac monitoring should be conducted routinely after an acute cerebrovascular
event to screen for serious cardiac arrhythmias. Echocardiography can detect many potential causes
of stroke: transoesophageal echocardiography (TOE) has been claimed to be superior to transthor acic
echocardiography (TTE) for the detection of potential cardiac sources of embolism. For all stroke
patients, blood tests are recommended.

11. Cerebral vascular diseases. Slowly progressing and transient disturbances of cerebral blood ... | 83
 Table 11.1
Emergency diagnostic tests in acute stroke patients (ESO 2000)
1. Bialn Imaging: CT or MRl
2. ECG
3. Laboratory Tests:
complete blood count and platelet count, prothrombin time or INR, partial
thrombin Time (PTT), serum electrolytes, blood glucose, C-reactive protein
(CRP) or sedimentation rate, hepatic and renal chemical analysis.
4. Extracranial and transcranial Duplex/Doppler ultrasound
5. MRA or СТА
6. Diffusion and perfusion MR or perfusion CT
7. Echocardiography (transthoracic and/or transoesophageal)
8 . Chest X-ray
9. Pulse oximetry and arterial blood gas analysis
10. Lumbar puncture
11. EEG
12. Toxicology screen

Ischemic Stroke Treatment

The "time is brain" concept means that treatment of stroke should be considered as an emergency.
Thus, avoiding delay should be the major aim in the prehospital phase of acute stroke care. This has
far-reaching implications in terms of recognition of signs and symptoms of stroke by the patient or by
relatives or bystanders, the nature of first medical contact, and the means of transportation to hospital.
All acute stroke patients require specialist muitidisciplinary care delivered in a stroke unit, and
selected patients will require additional high technology interventions.
The treatment of a patient with stroke is divided in two groups: general and specific treatment.
General treatment refers to treatment strategies aimed at stabilizing the critically ill patient in order to
control systemic problems that may impair stroke recovery; the management of such problems is a
central pad of stroke treatment. General treatment includes respiratory and cardiac care, fluid and
metabolic management, blood pressure control, the prevention and treatment of conditions such as
seizures, venous thromboembolism, dysphagia, aspiration pneumonia, other infections, or pressure
ulceration, and occasionally management of elevated intracranial pressure.
Intermittent monitoring of a neurological status, pulse, blood pressure, temperature and oxygen
saturation is recommended for 72 hours in patients with significant persisting neurological deficits). If
the oxygen saturation falls below 95 %, oxygen should be administered. In patients with a severe stroke
or swallowing problems regular monitoring of fluid balance and electrolytes should be provided.
Normal saline (0.9 %) is recommended for fluid replacement during the first 24 hours after stroke.
Routine blood pressure lowering is not recommended following acute stroke except for patients with
extremely high blood pressures (> 220/120 mm Hg) on repeated measurements, or with severe cardiac
failure, aortic dissection, or hypertensive encephalopathy. Monitoring serum glucose levels and
treatment of serum glucose levels »10 mmol/l with insulin titration are recommended, or if severe
hypoglycaemia < 2.8 mmol/l is diagnosed it should be treated with intravenous infusion of 10-20 %
glucose. Treatment of pyrexia (temperature > 37.5°C) with paracetamol and fanning is recommended.
Specific treatment of Ischemic stroke
Intravenous rtPA (Actilyse) (0.9 mg/kg body weight, maximum 90 mg), with 10 % of the dose given
as a bolus followed by a 60-minute infusion, is recommended within 3 hours of onset of ischemic stroke.
The blood pressures of 185/110 mm Hg or higher is lowered before thrombolysis. Aspirin (160-325
mg loading dose) can be given within 48 hours after ischemic stroke, but that if thrombolytic therapy is
planned or given, aspirin or other antithrombotic therapy should not be initiated within 24 hours.
Early mobilization is recommended to prevent complications such as aspiration pneumonia, deep
vein thrombosis (DVT) and pressure ulcers.
 The low-dose subcutaneous heparin or low molecular weight heparins (5000IU twice daily) should be
considered for patients at high risk of DVT or pulmonary embolism (e.g. due to immobilization, obesity,
diabetes, previous stroke).
Oral dietary supplements are only recommended for non-dysphagic stroke patients who are
malnourished. Early commencement of nasogastric (IMG) feeding (within 48 hours) is useful in stroke
patients with impaired swallowing.
Even with optimal stroke unit care including thrombolysis, less than one third of patients recover
fully from stroke. Rehabilitation aims to enable people with disabilities to reach and maintain optimal
physical, intellectual, psychological and/or social function. Goals of rehabilitation can shift from initial
input to minimize impairment to more complex interventions designed to encourage active
participation.

Hemorrhagic stroke
The second category of stroke, hemorrhagic stroke, occurs when a vessel in or near the brain ruptures
and leaks blood into the brain or surrounding tissues. In this case, the blood pushes against otherwise
healthy brain tissue and compresses it. The increased pressure reduces blood flow into the area, and if
the pressure becomes high enough, it can cause damage to brain cells.
There are two primary kinds of hemorrhagic strokes, named according to the location of the bleeding:
subarachnoid hemorrhage (SAH) occurs when blood floods the space between the brain and the skull
and intracerebral hemorrhage (ICH) happens when an artery inside the brain ruptures, spilling blood
into the surrounding brain tissue.
Pathogenesis of hemorrhagic stroke
Hemorrhagic strokes are often caused by aneurysms, or weakened portions of the artery wall. An
aneurysm may have no symptoms and go unnoticed for years. In many cases, the first sign of an
aneurysm is a stroke.
Subarachnoid Hemorrhage (SAH)
Of non-traumatic SAH, approximately 80 % are due to a ruptured berry aneurysm. Rupture of
arteriovenous malformations (AVMs) is the second most identifiable cause of SAH, accounting for 10
% of cases of SAH. Most of the remaining cases result from rupture.of the following types of pathologic
entities: mycotic aneurysm, angioma, neoplasm and cortical thrombosis.

Symptoms and signs

Signs and symptoms precede ruptured cerebral aneurysm in anywhere from 10-50% of cases.
Premonitory manifestations generally appear 10-20 days prior to rupture. The most common symptoms
are as follows: headache (48 %), dizziness (10 %), orbital pain (7 %), diplopia (4 %), and visual loss (4
%).
The typical presentation of aneurysmal rupture is with a very severe headache of abrupt onset ("the
worst headache of my life"), often initially accompanied by nausea, vomiting, diaphoresis, and
impairment of consciousness. Meningeal syndrome develops: the neck is stiff, and neck flexion is
painful. There may also be focal neurological signs, photophobia, and/or backache. Subarachnoid blood
can be seen on CT or MRI (Fig. 11.3) within 24 hours of the hemorrhage in roughly 90 % of cases. If
SAH is suspected but the CT is negative, a diagnostic lumbar puncture must be performed.

Complications of SAH
Some complications of SAH include the following: hydrocephalus, rebleed- ing, vasospasm, seizures
and cardiac dysfunction.
Hydrocephalus can be an acute or a delayed complication of SAH. Acute obstructive hydrocephalus
complicates 20 % of SAH cases. Clinical risk factors for the development of hydrocephalus include an
increased patient age, the use of antifibrinolytic drugs, left ventricular systolic dysfunction, and sei-
11. Cerebral vascular diseases. Slowly progressing and transient disturbances of cerebral blood ... | 85
zures. Acute hydrocephalus usually occurs within the first 24 hours after hemorrhage but may occur as
late as 7 days afterward. It presents as a relatively abrupt mental status change, including lethargy,
stupor, or coma. CT scan differentiates hydrocephalus from rebleeding.
The incidence of the complication of rebleeding is greatest in the first 2 weeks. The peak is
within 24-48 hours following initial SAH (approximately 6 %), with a rate of 1.5 % per day for the
next 12-13 days. Clinical factois that increase the likelihood of rebleeding include the following:
hypertension, anxiety, agitation, seizures.
Vasospasm. Currently, delayed ischemia from arterial smooth muscle contraction of the large
capacitance vessels at the base of the brain is the leading cause of death and disability following
aneurysmal SAH. Risk factors for vaso- і spasm include the following: lai ger volumes of blood in
the subarachnoid space, a clinically severe SAH, the female gender, young age, and smoking.
Vasospasm may be clinically indistinguishable from rebleeding. Symptoms vaiy with the arterial
territory involved, but patients typically present with a new-onset geneial decrease in
consciousness or focal neurologic deficit. Lethargy, with or without focal neurologic deficit, is a
manifestation of vasospasm, until proven otherwise.
Seizures. Seizures occur in 13-24 % of patients with SAH, commonly in the first 24 hours after
the bleed. They are most common after rupture of middle cerebral artery aneurysms. Generalized,
partial, and complex-partial seizures are observed after SAH. Seizures can lead to an increased
cerebral blood flow, hypertension, and elevated intracranial presure, thereby escalating the risk of
rebleeding and neurologic deterioration.

Cardiac dysfunction. Cardiac dysfunction occurs in a significant number of people with SAH.
Neurogenic sympathetic hyperactivity, as well as increased levels of systemic catecholamines, has
been implicated in SAH-associated cardiac dysfunction. Arrhythmias occur in as many as 90 % of
patients and most commonly include the following: premature ventricular complexes, bradyar-
rhythmias, supraventricular tachycardia.

Intracerebral Hemorrhafle (ICH)

ICH may occur within brain parenchyma or the surrounding meningeal spaces. It accounts for 8-15 %
of all strokes and results from a wide spectrum of disorders. ICH is more likely to result in death or
major disability than ischemic stroke or SAH. ICH and accompanying edema may disrupt or compress
adjacent brain tissue, leading to neurological dysfunction. Substantial displacement of brain
parenchyma may cause elevation of intracranial pressure (ICP) and potentially fatal wedge syndromes.

Pathogenesis
Nontraumatic intracerebral hemorrhage most commonly results from hypertensive damage to blood
vessel walls (hypertension, eclampsia, drug abuse), but it also may be due to autoregulatory dysfunction
with excessive cerebral blood flow (reperfusion injury, hemorrhagic transformation, cold exposure),
rupture of an aneurysm or AVM, adenopathy (cerebral amyloid angiopathy, moyamoya), altered
hemostasis (thrombolysis, anticoagulation, bleeding diathesis), hemorrhagic necrosis (tumor,
infection), or venous outflow obstruction (cerebral venous thrombosis). Non-penetrating and penetrat-
ing cranial trauma are also common causes of intracerebral hemorrhage.
Chronic hypertension produces a small vessel vasculopathy characterized by lipohyalinosis, fibrinoid
necrosis, and development of Charcot — Bouchard aneurysms, affecting penetrating arteries throughout
the brain including len- ticulostriates, thalamoperforators, paramedian branches of the basilar artery,
superior cerebellar
1(52 І PART 2:arteries, and anterior inferior cerebellar arteries.
Special Neurology
Predilection sites for intracerebral• hemorrhage include the basal ganglia (40-50 %), lobar
regions (20-50 %), thalamus (10-15 %), pons (5-12 %), cerebellum (j5-10 %), and other
brainstem sites (1-5 %).
Clinical manifestations. The classic clinical presentation includes the onset of a sudden focal
neurological deficit while the patient is active, which progresses over minutes to hours and are
 determined by the size and location of hemorrhage, but may include the following: hypertension,
fever, or cardiac arrhythmias, nuchal rigidity, subhyaloid retinal hemorrhages, an altered level of
consciousness, anisocoria, focal neurological deficits.
General complications. Intraventricular extension of hemorrhage, hydrocephalus, cerebral
edema, intracranial hypertension, seizures, and hemodynamic changes (often a dangerous
elevation of blood pressure)
Clinical criteria of diagnostics:
► young and middle age of a patient, hypertonic cerebral crises in anamnesis,
► a sudden onset against the background of physical or emotional overtension,
► consiousness disturbances, marked meningeal symptoms,
► hyperemia of the face, midriasis on the lesion side,
► breath is hoarse, irregular, the body temperature is increased,
► blood pressure is extremely high, pulse is tensed,
► there are focal and dyslocation brainstem symptoms.

Hemorrhagic stroke Diagnostics

Brain imaging is a crucial part of the emergent evaluation. CT and MRl (Fig. 11.4) shows the
equal ability to identify the presence of acute ICH, its size and location, and hematoma
enlargement.
CT may be superior at demonstrating associated ventricular extension, whereas MRl is superior
at detecting underlying structural lesions and delineating the amount of perihematomal edema
and herniation.
A CT angiography scan with contrast may identify an associated aneurysm, arteriovenous
malformation, or tumor. CT angiography may provide additional detail in patients with
suspected aneurysm or arteriovenous malformation.
• White cell differential count of peripheral blood is shifted to the left.
► Cerebral spinal fluid is bloody with the presence of changed erythrocytes.
► On fundus — hemorrhages in retina.
► M-echo is shifted on 6-7 mm on Echo-encephaloscopy.

Hemorrhagic stroke treatment

Potential treatments of ICH include stopping or slowing the initial bleeding during the first hours
after onset; removing blood from the parenchyma or ventricles to eliminate both mechanical and
chemical factors that cause brain injury; management of complications of blood in the brain, including
an increased ICP and a decreased cerebral perfusion; and good general supportive management of
patients with severe brain injury. Good clinical practice includes management of airways, oxygenation,
circulation, glucose level, fever, and nutrition, as well as prophylaxis for deep vein thrombosis.
A sudden eruption of an intracranial hemorrhage destroys and displaces brain tissue and can induce
an increase in ICP. The dynamics of ICH after the appearance of the primary lesion includes hematoma
growth, perihe- matomal edema and/or ischemia, hydrocephalus, or secondary intraventricular
hemorrhage (1VH). Blood pressure can be monitored adequately with an automated cuff, whereas
continuous monitoring of systemic arterial pressure should be considered in patients who require
continuous intravenous administration of antihypertensive medications and in patients whose
neurological status is deteriorating. Airway and oxygenation can be assessed per respiratory status and
pulse oximetry. Cardiopulmonary instability in association with an increased ICP is to be avoided to
minimize11. Cerebral vascular diseases. Slowly progressing and transient disturbances of cerebral blood ... | 87
deleterious effects in patients with a limited autoregulatory capacity. The vast majority of ICH patients
are admitted to intensive care units because of their impaired consciousness, elevated blood pressure,
and frequent need for intubation.
 The traditional treatment of SAH from a ruptured cerebral aneurysm included strict blood pressure
control, with fluid restriction and antihypertensive therapy. This approach was associated with a high
rate of morbidity and mortality from the ischemic complications of hypovolemia and hypotension.
Current recommendations advocate the use of antihypertensive agents when the mean arterial
pressure (MAP) exceeds 130 mm Hg. Intravenous beta-blockers, which have a relatively short half-life,
can be titrated easily and do not increase intracranial pressure. Beta-blockers are the agents of choice in
patients without contraindications.
Most clinicians avoid the use of nitrates, such as nitroprusside or nitroglycerin, which elevate ICP.
Calcium channel blockers have a fast onset and lead to a relatively lower increase in ICP than do
nitrates. Angiotensin-converting enzyme inhibitors have a relatively slow onset and are not first-line
agents in the setting of acute SAH.
Patients with signs of increased ICP or herniation should be intubated and hyperventilated. Minute
ventilation should be titrated to achieve a PC02 of 30-35 mm Hg. Avoid excessive hyperventilation,
which may potentiate vasospasm and ischemia.
Other interventions for increased ICP include the following:
► Osmotic agents (mannitol -— 50.0 intravenous), which can decrease ICP dramatically (50 % 30
minutes post administration).
► Loop diuretics (furosemide — 1 % 2.0-4.0 intravenous) also can decrease ICP.
► The use of intravenous steroids (dexamethasone — 8-12 mg intravenous) for decreasing ICP is
controversial but is recommended by some clinics.
► Surgical treatment to prevent rebleeding consists of clipping the ruptured berry aneurysm.
Endovascular treatment (coiling) is an increasingly practiced alternative to surgical clipping. The
neurosurgeon/neuro- interventionalist must be involved early in the care of the patient with an
aneurysmal SAH.
Stroke prevention
The high blood pressure and diabetes should be managed with lifestyle modification and
individualized pharmacological therapy. High blood cholesterol should be managed with lifestyle
modification and a statin. Regular physical activity is recommended. The heavy use of alcohol and
cigarette smoking should be discouraged. Subjects with an elevated body mass index should take a
weight-reducing diet. Low-dose aspirin is recommended in women aged 45 years or more who are not
at increased risk for intracerebral hemorrhage and who have good gastrointestinal tolerance. Aspirin
may be recommended for patients with non-valvular atrial fibrillation (AF) who are younger than 65
years and free of vascular risk factors. Unless contraindicated, either aspirin or an oral anticoagulant
(INR — 2.0-3.0) is be useful for patients with non-valvular AF who are aged 65-75 years and free of
vascular risk factors. Low dose aspirin is be useful for patients with asymptomatic ICA stenosis > 50 %
to reduce their risk of vascular events.
Secondary prevention — reduced stroke recurrence after the first stroke:
► antiplatelet therapy (TIA, mild stroke, atherothrombotic stroke);
► oral anticoagulation (cardiac embolism, arterial dissection);
► endarterectomy (in symptomatic carotid stenosis > 70 % or > 80 %, or after mild strokes);
► the potential utility and indications of carotid angioplasty and stenting in the treatment of carotid
stenosis are currently under intensive study.

Rehabilitation measures include physical, occupational, and speech therapy, as well as

psychological counseling of the patient and family.

Tests and typical tasks

1. Name the main signs of subarachnoid hemorrhage?
a) Kernig symptom, bloody CSF
b) global aphasia, apraxia
1(52 І PART 2:hemiplegia
c) hemianesthesia, Special Neurology
d) bladder disturbances, hemiplegia
e) hemiplegia, nystagmus
2. Indicate the main symptoms of Inner carotid artery thrombosis:
a) bladder disturbances, hemiplegia on the side of thrombosis
b) nystagmus, hemiparesis
 c) dysarthria, dysphonia
d) blindness on the thrombosis side, hemiparesis on the oposite side
e) ver tigo, headMche

3. Name kinds of brain stroke:

a) transient ischemic attacks
b) slowly progressing disturbances of cerebral blood circulation -с) hemorrhagic,
ischemic
d) subdural, epidural hematomas
e) acute, subacute, chronic

4. What are the clinical manifestations of transient ischemic attacks in the carotid system?
a) mono- or hemiparesis, alternated syndromes
b) diplopia, hemiataxia
c) dysphonia, dysarthria, dysphagia
d) segmental hypesthesia, bladder disturbances
e) mono- or hemiparesis, motor or sensitive aphasia
5. Indicateclinical forms of stroke:
*a) a completed stroke, minor stroke, major stroke b) hemorrhagic, ischemic
c.) transient ischemic attacks, subarachnoid hemorrhage
d) slowly progressing disturbances of cerebral blood circulation
e) a mild, moderate, heavy stroke

6. A 45-year-old patient was brought to a clinic in a heavy condition. A knife-like headache with
vomiting appeared suddenly while carrying heavy wardrobe. Convergent strabismus, neck rigidity,
Kernig syrntoms on both sides were found, fhere are no palsies and sensitive disturbances, tendon
reflexes on arms and legs are lowered evenly. What is the previous diagnosis? What should doctor
r
prescribe to diagnosis confirming? _f -я,*!'-*
7. A 55-year-old patient suffered horn diabetes mellitus one morning felt numbness in right limbs and
rambling speedi. He was hospitalised to neurologic department.
In 2 hrs after prescription of vascular drugs symptoms had dissapeared. What diagnosis should be wr
itten? - м
8. A 60-year-old patient is frequently annoyed, complains of headache, dizziness, bad mood. His exam
shows oral automatism reflexes, anisoreflexia of tendon and periosteal reflexes. What disease may be
suspected?
9. A 48-year-old patient felt difficulties while swallowing food, shaking while walking, dizziness. There
were episodes of dizziness at past. Examination showed not impaire conciousness, segmental
hypesthesia on the left part of the face. The left half of the soft palate hangs down. Pharyngeal and
palate reflexes are absent on the left, chokes while swallowing. Hemihypesthesia and increased
reflexes on the right side were found. Coordination in the left arm and leg is impaired. Determine
clinical diagnosis.
10. A patient after an emotional stress suddenly lost consiousness, the face is hyper- aemic, blood
pressure is 250/150 mm Hg, coma, meningeal signs are positive, anisoreflexia of tendon and
periosteal reflexes D > S. What is the presumable diagnosis?

11. Cerebral vascular diseases. Slowly progressing and transient disturbances of cerebral blood ... | 89
 12. HEADACHE. KINDS AND NOSOLOGICAL FORMS OF HEADACHE. MIGRAINE

The definition of "headache" means pain or discomfort sensation localized over the eyebrows up to the
cervico-occipital area. The pain in the face zone is related to the group of facial pains — prosopalgia.
Headache occurs in case of irritation of pain receptors in the skin, subcutaneous tissue, tendon helmet,
vessels and soft tissues of the head, skull periosteum, brain membranes. Arterial walls are particularly
susceptible to irritation, the venous sinuses of the brain membrane and small veins are less susceptible.
Headache is a syndrome with a polyetiological structure. The reasons causing it can be united into the
following groups:
1. Diseases of the circulatory system: acute and chronic brain circulation disorders, arterial
hypertension, abnormalities of brain vessels, vasculitis, etc.
2. Infectious diseases of the brain and its membranes (encephalitis, meningitis), general infectious
diseases that are accompanied by fever.
3. Injuries of the skull and brain.
4. Expansive processes of the brain.
5. Diseases of the eyes, ears, sinuses, teeth, temporal mandibular joint, cervical spine.
6. Somatic diseases (endocrine diseases, lespiratory diseases with respiratory failure and hypercapnia
etc.).
7. Emotional stress and neuroses.
8. Intoxication (alcohol, CO) and use of medicines: nitrates, caffeine, oral contraceptives,
adrenoceptor agonists, some antibacterial drugs (cycloserine, rifampicin), indomethacin,
carbamazepine, ranitidine, etc.
Despite polyetiological nature of cephalgia, headache has several mechanisms of development.
The vascular type of headache. Vascular headache is often represented by the- vasomotor
mechanism connected with dilatation of blood vessels due to a decr ease of systemic blood pressure or
discrepancy of an increased stroke volume of blood and cerebral vascular resistance (in case of an
increased systemic blood pressure). It is characterized by the pulsating nature of cephalgia in the
temporal areas.
If the arterial tone of cerebral vessels is increased ischemia and ischemic hypoxia of the brain may
occur. In this case headache is dull, is perceived by the patient as a feeling of compression, is
accompanied by dizziness, "black spots" in front of the eyes.
The venous variant of the vascular type of headache is caused by vasomotor disorders of the venous
system leading to venous insufficiency and venous stasis phenomena. The venous outflow is most
effective in the upright position, so venous headache occurs or increases in the prone position,-sitting
position with the lowered head or while coughing. The typical symptom of venous insufficiency
headache is a morning headache with the domination of not a pain itself but a feeling of a "heavy", "not
clear" head. The pain is most often localized in the occipital region, where the projection of the
confluence of intracranial venous vessels is located. The pain usually decreases during active
movements of the shoulder girdle and neck area muscles, enhancing the vein tonus.
The vascular variant of headache is observed in case of migraine, vegetative dystony, arterial
hypertension of various origins, cerebral atherosclerosis, vasculitis.
Tension headache is a headache that develops due to scalp muscles and chewing muscles tension.
There are two mechanisms of cephalgia tension development. Emotional stress causes sympathoadrenal
activation which increases neuromuscular stimulation and facilitates scalp muscles tension. The pain
may also be caused by segmental reflex mechanisms that are triggered during prolonged uncomfortable
position or due pathological impulses in case of local inflammation of the eyes, ears and nose or neck
osteochondrosis.
Tension cephalalgia is characterized by a slight or moderate intensity, bilateral localization in the
frontal, occipital, temporal areas, compressing character. Patients describe such pain as a sensation of a
1(52 І PART 2: Special Neurology
"tight hat" ("neurasthenic helmet"). The pain is accompanied by unpleasant sensations when touching
the scalp, combing hair, vivid emotional reactions. The pain lasts from thirty minutes to several days.
Tension cephalgia is facilitated by reflex methods, psychotherapy, sedative and myorelaxant drugs.
Today tension headache is grouped as an independent nosological form if it occurs in response to
acute or chronic emotional stress.
 Tension headache as a nosological form is diagnosed in case of the following diagnostic criteria:
► the duration of the headache is not less than 30 minutes. The pain may have an episodic character
with the duration from 30 minutes to 7 days. Chronic tension headache may occur daily without
subsiding;
► a specific nature of the headache (compressing, tightening, monotonous), low intensity.
Pulsating pain is not typical;
» diffuse localization, always bilateral;
► the pain does not increase during physical activity.

Liquor-dynamic headache occurs in case of an increase or a decrease of production of

cerebrospinal, fluid or impairment of its outflow due to any pathological process.
Intracranial hypertension is characterized by "thrusting" headache accompanied by nausea, vomiting,
dizziness and in case of an expressed swelling of the brain — a loss of consciousness. The pain gets
worse when coughing, sneezing, in the horizontal position, decreases — in the vertical position.
Therefore patients with hypertension sleep with head held high. The typical symptoms that occur during
neurological investigation are pain sensation when pressing or moving the eyeballs (Mann symptom),
the presence of bilateral pyramidal symptoms or asymmetry of tendon reflexes.
The evidence of an increased intracranial pressure is venous stasis in the fundus and optic disc
edema. In the case of such symptoms neuro- visual investigation — CT, MRI — should be applied to the
patient for exclusion the possibility of "expansive processes" (tumor, cyst, abscess of the brain).
Neuralgic pain. This type of pain is related to facial pain (prosopalgia) and arises often in cases of
trigeminal neuralgia. It is characterized by brief paroxysms acutely occurring and following one after
another. Patients describe this pain as shooting, "electric shock". Neuralgic pain is characterized by the
presence of trigger zones. The irritation of trigger zones provokes paroxysms. During the neurological
examination painful sensation when pressing the points of trigeminal nerve exits and hypoesthesia or
hyperesthesia in the area of its branches innervation are observed. Neuralgic headache is characterized
by positive dynamics due to carbamazepine.
Psychogenic mechanism of headache is connected with psychogenic disorders of nociception. It is
observed in the case of hysterical and depressive syndromes. The mechanism is individual for each
patient, is accompanied by various emotional disorders and does not limit the ability to work. It is
characterized by a chronic course.
The most common is a combination of different mechanisms of headache (cephalgia of mixed origin).
Principles of cephalgias treatment:
1. Elimination of the headache cause.
2. Impact on the cephalgia mechanism. In the case of vasomotor cephalgias blockers of alpha- or
beta-adrenoreceptors (sermioji, pyrroxanum, anaprilin, atenolol), calcium antagonists (stngeron,
nimotop) are recommended.

In the case of venous cephalgias venotonic drugs are prescribed (troxevasin, anavenol, endotelon,
aescusan). In the case of intracranial hypertension dehydrating agents should be used: osmotic diuretics
— mannitol, glycerine (in the case of normal osmolal ity); diuretics of another mechanism of action —
furo- semide, hydrochlorothiazide, etc; potassium-sparing diuretics — verospiron, diacarb etc.
Dehydrating methods are often combined with venotonic drugs.
In the case of hypotension bed rest increasing the biood supply, high concentration of salt in the diet
and use of large amounts of liquids are recommended.
Tension cephalgias require prescription of myorelaxants (mydocalm, baclofen, sirdalud, miolastan),
drugs with tranquilizing (benzodiazepines) and anti-depressant (tricyclic antidepressants) actions,
reflex treatment methods -• (neckline massage, acupuncture, physiotherapeutic methods),
12. Headache. Klntls arid nosological forms of headache. Migraine | 91
psychotherapy.
In the case of neuralgic pain carbamazepine drugs (finlepsin, timonil etc.) are recommended.
According to the International classification of diseases, there are three nosological forms of
headache: migraine, tension headache and cluster headache.

Migraine
Migraine, or hemicrania (from Greek: hemi — half, cranium — skull) is a paroxysmal headache of
pulsating character in one half of the head.
 Pathogenesis. In the pathogenesis of migraine an important role is played by hereditary-determined
generalized impairment of vasomotor regulation in the form of an unstable tone of extra- and
intracranial vessels, and metabolic disorders of biologically active substances (serotonin,
prostaglandins, kinins, histamine, substance P, etc.). Serotonin plays a particularly important role in
causing migraine attacks.
Today the trigeminal-vascular theory of migraine pathogenesis, proposed by M.Moskowitz in 1984,
is most commonly used. According to this theory, activation of the trigeminal nerve leads to the
secretion of strong vasodilators (calcitonin* substance P) from its nerve endings. It leads to a decrease of
vascular tone and increase of vascular wall permeability and its swelling. A neurogenic aseptic
inflammation occurs that leads to the excitation of afferent fibers of the trigeminal nerve, innervating
intracerebral vessels. Anatomical features of the trigeminal nerve are the reason of hemicranic
localization of the pain and its irradiation onto the frontal-orbital-temporal area. The basis of activation
of the trigeminal nerve is impairment of central serotonin and catecholamine systems' function.
The course of migraine paroxysm has three phases, determining its clinical picture:
I — vasoconstriction due to an increase of peripheral sympathetic effects and the serotonin level.
Aura develops.
II — dilatation of arteries, arterioles, veins and venules. The oscillation amplitude of vessel walls
intensifies, the level of serotonin decreases and the level of bradykinin — increases. Pulsating headache
appears.
III — perivascular edema clue to an increased histamine level and due to the sympathetic system
depression and a decrease of the serotonin level.
Migraine paroxysm can be caused by emotional stress, physical activity, meteorological factors,
irregular eating (fasting) or certain foods rich in ty- ramine (cocoa, chocolate, nuts, citrus, fruits, smoked
meat, cheeses, etc.), alcohol (especially red wine).
Clinical picture. According to the International classification, migraine is divided into migraine
without aura and migraine with aura.
Aura is a complex of focal neurological symptoms that precede pain paroxysm or occur at the pain
elevation. The nature of neurological symptoms that constitute the aura depends on the vascular pool
(carotid or vertebrobasilar) which is involved in the pathological process.
For the diagnosis of migraine without aura the case history of the patient should include at least 5
migraine attacks that meet the following criteria: the duration of headache paroxysm from 4 to 72 hours;
a predominantly unilater al localization of headache with alternating sides; a pulsating nature of head-
aches, its increase during physical activity; the presence of at least one symptom of an attack: nausea,
vomiting, phonophobia, photophobia.
For the diagnosis of migraine with aura the case history of a patient should include at least 2 attacks,
consisting of cephalgic paroxysm and focal neurological symptoms. These neurological symptoms must
last not longer than 60 minutes and completely disappear, leaving no organic neurological
manifestations. Depending 011 the aura there are such forms of migraine: ophthalmic (migraine with a
typical aura, classic migraine), retinal, ophthalmoplegic, hemiplegia basilar migraine.
Ophthalmic migraine (classic) begins with bright photopsias (zigzags, dots, spots) in the left or right
field of view with the subsequent short-term loss or decrease of visual fields. At the end of aura
pulsating pain develops. The cause of a visual aura is dyscirculation in the pool of the posterior cerebral
artery. The difference of retinal migraine from ophthalmic is based in temporary blindness in one or
both eyes combined with a headache. In this case blindness is connected with dyscirculation in the
branches system of the central artery of retina.
in the case of a hemiplegic aura the headache is accompanied by hemiplegia or hemiparesthesia or
hemihypesthesia.
In the case of basilar migraine the headache is accompanied by systemic dizziness, noise in the ears,
ataxia, dysarthria, paresthesias in hands and legs. The typical start is the appearance of bright light in
front of the eyes and the subsequent development of a blindness within some minutes. One third of
patients have a short-term consciousness impairment.
Ophthalmoplegic migraine is characterized by a combination of headache with oculomotor disorders,
1(52 І PART 2: Special Neurology
unilateral ptosis, diplopia. In the case of such a form of migraine patients should be examined
additionally for exception of vascular anomalies (aneurysms).
A severe complication of migraine is a migraine status — migraine paroxysms occurring one after
another. In the case of the absence of adequate treatment they may lead to brain infarction. Migraine
infarction is accompanied by the emergence of focal neurological symptoms depending on the
localization.
Treatment is divided into treatment of an attack and therapy during the period between attacks aimed
at their prevention.
To treat the attack the drugs of the following groups are used:
► in case of slight attacks nonsteroidal anti-inflammatory drugs, especially aspirin, are used. This
group of drugs leads to the reduction of neurogenic, inflammation, a decrease of pain modulators
synthesis (prostaglandins, kinins) and the activation of antinociceptive mechanisms;
► preparations of ergotamine series. This group of drugs causes significant vasoconstriction due to
affecting the serotonin receptors and alpha- adrenoceptors stimulation (nomigren or
dihydroergotamine);
► selective serotonin agonists (sumatriptan, zolmitriptan) interrupt the migraine attack by
influencing serotonin receptors of cerebral blood vessels, reduce the allocation of substance P from
trigeminal nerve endings and the development of neurogenic inflammation. Sumatriptan is
prescribed in the form of tablets (50-100 mg) or injections of 6 ml subcutaneously.

At the same time drugs of benzodiazepine series can also be prescribed. If necessary, antihistamine
and dehydrative drugs are used
Preventive treatment in the period between attacks is pr escribed to patients with a frequency of
attacks at least twice per month. At first non-medication therapies are used. They include diet with the
restriction of products that contain tyramine, medical gymnastics, balneotherapy, acupuncture etc.
For medical treatment blockers of beta-adrenoreceptors (propranolol, atenolol), calcium channel
blockers (nimodipine, flutiarizin), antidepressants (amitriptyline), serotonin antagonists (methysergide)
are used. The daily prescription of small doses of aspirin (100-325 mg) and antihistamine drugs is
possible. The dose of each drug is chosen individually.

Cluster headache
Previous names — Horton syndrome, head erythromelalgia, Harris' migrainous neuralgia,
"histamine" cephalgia. It is typical mostly for young men.
Clinical basis of cluster cephalgia is constituted by sudden intensive paroxysm, itchy, burning or
cutting pain around the orbital area. The pain has a typical irradiation into the frontal-temporal,
zygomatic area, and sometimes onto the entire half of the face and neck.
On the side of the pain facial skin and sclera get red. Horner's syndrome, lacrimation and nasal
congestion are observed. The pain usually develops at night an hour after falling asleep ("alarm clock"
pain), and occurs every night (hiring several weeks (cluster pain). The duration of the attack usually
does not exceed 2 hours. During a day there may be few attacks, with at least one attack at night.
Exacerbation can last from 4 to 10 weeks, then the pain disappears spontaneously for a few years.
For treatment of cluster headache a combination of antimigraine drugs (see "Migraine"),
anticonvulsants (carbamazepines, clonazepam) and antihistamine drugs is used.

Tests and typical tasks

1. What are the nosological forms of headache?
a) liquor-dynamic headache, neuralgic pain
b) headache caused by arterial hypertension, neuralgic pain
c) headache caused by arterial hypotension, psychogenic headache
d) migraine, tension headache, cluster headache
12. Headache. Klntls arid nosological forms of headache. Migraine | 93
e) psychogenic headache, liquor-dynamic headache

2. What are the clinical forms of migraine?

a) migraine without aura, migraine with aura
b) decompensated, terminal
c) subcompensated, initial
d) acute, chronic
e) psychogenic headache, vestibular
 3. What are the clinical forms of migraine with aura? a) hyperkinetic, atactic
• b) ophthalmic, basilar
c) shaking, hypokinetic
d) vestibular, atactic
e) hypokinetic, aphasic

4. What drugs are used for treatment of migraine attack?

a) anticoagulats, nootropic drugs
b) neuroleptics, hypotensive drugs
. c) non-steroid anti-inflammatory drugs, serotonin agonists
d) sedative drugs, dopamine agonists
e) neuroleptics, antioxidants
»
5. What are the symptoms of cluster headache?
.-a) intensive paroxysm of burning pain around the orbital area
b) headache with oculomotor disorders
c) dull, pressing headache
d) bilateral headache of a compressing character
e) headache accompanying by systemic dizziness.

6. A25-years-old young women has a paroxysmal headache of a pulsating character in the temporal
area. These attacks are accompanied by nausea, photophobia. Bright zigzags in the field of view
appear before headache. A paroxysmal headache began at the age of 15 years. What is the previous
diagnosis?-
7. Apatient suffers from headache in the morning every day. The pain gets worse when coughing,
sneezing, in a horizontal position, decreases - in a vertical position. Sometimes patient, has vomiting
on headache elevation. There is edema of optic discs. Which form of headache can be suspected? t " "
8.A young women suffered from pulsing pain in temporal area since 14 years. Duration of a headache
attack is 30-40 min. I he unilateral ptosis, diplopia appear befoie headache. Which disease can be
suspected?
9.Young men has paroxysm of an intensive, burning pain around the orbital area after
psycho-emotional tension. The pain has a typical irradiation to the frontal- temporal area and
sometimes to the entire half of the neck. It usually develops at night an hour after falling asleep,
occurs every night. On the side of the pain the facial skin and sclera get red. Which form of headache
can be suspected?
10. A 39-years-old man has complaints of a moderate intensity headache of bilateral localization in the
occipital areas and a compressing character in the evening. I he patient describes such pain as a
sensation of a "tight hat". His is a manager by profession. The duration of his working day is 8-9
hours using a personal computer. What is the previous diagnosis? )
І З . CLOSED CRANIOCEREBRAL AND SPINAL TRAUMA

Craniocerebral trauma is the leading cause of death in persons under the age of 50 years. Traumatic
brain injury is steadily becoming more common, mainly because of motor vehicle accidents. In
industrial countries, the .incidence is ca. 8000 cases per million persons per year, of which
approximately a half require hospitalization. The outcomes of traumatic brain injury depend on the type
and extend of the acute (primary) injury and its secondary and late consequences. Craniocerebral injury
occurs: in the case when the head is hit by moving objects, when the head hits immovable hard objects,
during shaking of the body, in the cases of an explosive force action. Traumatic injuries to the bony skull
and the underlying
1(52 І PART 2:brain can be of different types and varying severity, depending on the nature and
Special Neurology
intensity of the causative event. Craniocerebral injuries are either closed(with the dura mater intact) or
open (with a wound extending into the subdural compartment or deeper into the brain parenchyma).
Open brain injuries are associated with the risk of early or late intracranial infection.
Classification of closed craniocerebral trauma
During the 18lh century, the French surgeon and anatomist J.L. Petit defined 3 main forms of closed
craniocerebral injury (CCI): concussion (commotio cerebri), contusion (contusio cerebri), compression
(compressio cerebri). Modern classifications of CCI includes: concussion, contusion — mild, moderate
and severe degrees of severity, — and compression — without contusion or with contusion.
Mild closed craniocerebral injury— brain concussion and brain contusion of a mild degree severity.
Moderate— it is only contusion of a moderate degree severity. Severe — includes contusion of the brain
of a high degree severity and an acute compression of the brain.

Brain concussion
Concussion means a reversible traumatic affection of nervous function without morphological lesion,
that causes dysfunction of the reticular formation of the upper brainstem. Effect inactivating the reticular
formation blocks elect» ical activity to the cerebral cortex, it leads to the disconnection between the
cortex, subcortical structures and brainstem with secondary metabolic, vasomotor and hormonal
disorders.
Clinical manifestations of the brain concussion include transient geneial cerebral symptoms,-
unstable disseminated neurological microsymptomatol- ogy and vegetative disorders.
General cerebral symptoms are characterized by a brief, transient loss of consciousness, usually
lasting no more than 1-20 minutes and sometimes followed by a period of confusion. Headache,
dizziness, nausea, and sometimes vomiting are common accompaniments of concussion in the early
phase. Concussion is accompanied by very brief anterograde or retrograde amnesia. Retrograde amnesia
is amnesia for events occurring before the injury, anterograde amnesia — for events occurring
afterwards.
Disseminated neurological microsymptomalology includes the absence or decreasing of the corneal,
pharyngeal, and cutaneous reflexes, anisocoria, weakness of convergence of eye balls, nystagmus,
Marinesco — Radovici symptom (palmar-chin reflex), asymmetry of deep reflexes, unsteadiness in
Romberg's position, unsteady gait. Sometimes meningeal symptoms can be present.
Vegetative disorders are presented by fluctuations of blood pressure and pulse, hyperhydrosis,
acrocyanosis, astiienisation, sleep disorders, fast fatigue.
Diagnostics. Craniography has to exclude skull fractures, ophthalmoscopy may define papilledema
as sign of the CSF hypertension, examination of liquor in the case of presents of meningeal signs has to
exclude a blood in CSF.
Treatment. Adequate therapy consists of a temporary restriction of activity. Transient restriction of
activity suffices (7-10 days of bed rest), combined with dehydration, antioxidants, vit C, nootropic and
symptomatic medication: sedative, cardiac drugs, analgesics.

Brain contusion
By definition, this type of injur у involves damage of the brain parenchyma.
Clinical manifestations of the brain contusion include general brain symptoms, meningeal
symptoms, steady focal symptoms and vegetative disorders. The main differences between contusion
and concussion of the brain are the presence of focal symptoms in the neurological status, possible
fracture of the base of the sluill, subarachnoid bleeding, morphological lesions on CT or MRl of the
brain.
Contusion causes considerably longer periods of unconsciousness, retro- and anterograde amnesia.
Examination in the acute phase often reveals neurological deficit, which may persist.
Large brain contusions, combined with the associated
13. Closed secondary
craniocerebral brain
and spinal trauma | 95 edema, can cause very rapid
and pronounced increases in intracranial pressure, leading to brain compression and impaction of the
midbrain and diencephalon through the tentorial notch, or of the medulla through the foramen magnum.
The clinical signs of the brainstem wedge are: progressive impairment of consciousness leading to
coma, a dilated pupil, initially only on the side of the expansive lesion, a loss of autonomic regulatory
functions (breathing, temperature, cardiac activity, vascular tone) and death.
The level of impaired consciousness or coma in a brain injured patient is graded numerically with the
use of Glasgow coma scale (GCS). This scale helps to classify the degree of severity of brain injury. The
GCS score is the sum of three scores (eye opening, best verbal response and best motor response). The
maximum score is 15; the minimum is 3. Craniocerebral injury is classified as mild if the GCS score is
14 to 15, moderate if 9 to 13, severe if 3 to 8. Brain contusion of a mild degree of severity is
characterized by impairment of consciousness not longer than 1 hour, focal symptoms' duration about
2-3weeks. A moderate degree of severity means a loss of consciousness for the period from 1 to 6 hours,
regress of focal symptoms in 3-5 weeks. A severe degree — a loss of consciousness tor some days or
weeks, steady focal symptoms in the neurological status, may be signs of brain compression and a
wedge of the midbrain and diencephalon through the tentorial notch, or of the medulla through the
foramen magnum.
Diagnostics. Craniography detects skull fractures, ophthalmoscopy defines papilledema signs of the
CSF hypertension, examination of liquor in the case of presense of meningeal signs shows subarachnoid
bleeding. CT or MRl reveals foci of contusion (Fig.13.1). Parenchymal injuries can be found both
directly underlying on the site of the external blow and at the diametrically opposite location in the
brain. The pathoanatomical findings in foci of brain contusion include ischemic and hemorrhagic tissue
necrosis, small hemorrhages, tears of brain tissue and blood vessels; and secondary brain edema.
Lumbar puncture may show bloody or xanthochromic CSF.
Treatment. Depending on the clinical state, the patient needs to be observed either in an intensive
care unit or in a specialized neurotrauma unit, with frequent examination of vital signs and neurological
functions, decreasing of intracranial pressure on indication. Recent studies have shown a positive effect
of therapeutic hypothermia, in which the patient is cooled down to ca. 34 °С. If the patient survives, MRl
may reveal a permanent injury of the brain parenchyma.

Brain compression
Compression may be caused by subdural, epidural, intracerebral hematomas. Traumatic hematomas
may be located within the brain parenchyma (traumatic intracerebral hematoma) or in the adjacent
meningeal compartments (subdural and epidural hematomas).

Traumatic hematomas
Traumatic hematomas appear when the traumatic injury tears a larger aitery or vein. Intracerebral
hematomas are usually located in the frontal or temporal lobes, They may exert a considerable mass
effect, combined with the surrounding edema, cause sufficient pressure on the brain, leading to the
progressive decline of consciousness and severe neurological deficit.; In such
patients, neurosurgical evacuation of hematoma should be considered, depending on its size and
location.
Brain compression by subdural or epidural hematomas is different from concussion and contusion
because of: an increase of general brain symptoms, appearance of focal and brainstem dislocation
symptoms right after the injury, or after the period of imaginary well being (several hours or days) so
called "lucid space".

Epidural hematoma
Epidural hematoma is generally produced by traumatic tearing of a dural artery, usually the middle
meningeal one. The tear itself is usually the result of a temporal-parietal skull fracture, but sometimes
occurs in the absence of a skull fracture. The blood accumulation is detected between the periosteum
and the dura mater.
Clinical picture. The first manifestations of traumatic hematomas may be brain concussion or brain
contusion symptoms which were attached above. Epidural hematomas may present with a lucid period
immediately following the trauma and a delay before symptoms become evident. Gradual accumulation
of the blood leads to the
112 I PAR compress
Г 2; Special Neurology of intracranial structures.
The arterial hemorrhage can compress the brain very rapidly: a patient who is initially comatose
because of a coexisting brain contusion may fail to emerge from coma because of the development of an
epidural hematoma in minutes or hours after injury (compression with contusion). An initially awake or
only transiently unconscious patient with supposed concussion may lapse into coma after a lucid period
lasting minutes or hours (compression without contusion).
The side of the hematoma can often be assessed by clinical examination: fixed and dilation of the
ipsilateral pupil, the eye will be positioned down and out, while hemiparesis is contralateral to the
hematoma.
 Diagnostics. When an acute epidural hematoma is suspected, a CT or MRI should be performed
immediately to confirm the diagnosis. Hematoma is usually seen as a hyperdense, biconvex zone
that is sharply demarcated from the adjacent brain tissue (Fig. 13.2). Plain radiograms of the skull
might reveal a skull fracture.

Treatment. Once diagnosed, it must be neurosurgical^ evacuated immediately to prevent the

brainstem wedge in and death. Patients often well recover if they don't have any other
accompanying brain injuries and if hematoma has been removed early enough.

Subdural hematoma
Subdural hematoma can be acute, subacute, or chronic. Blood accumulation is between the dura
mater and the arachnoid and arises because of a bridging vein's tear. Clinical examination alone
does not enable a clear-cut distinction between subdural and epidural hematomas: subdural
hematoma is characterized by a rapidly progressive decline of consciousness, ipsilateral pupillary
dilatation, and contralateral hemiparesis.
Chronic subdural hematoma may arise after mild traumatic brain injuiy or even after a relatively
trivial blow to the head, of which the patient may no longer have any recollection. A few weeks or
(rarely) months after the causative event, the patient begins to suffer from an increasingly severe
headache, fluctuating disturbances of consciousness, confusion, and ultimately progressive
somnolence. Hemiparesis, if present, is usually mild and signs of intracranial hypertension are
usually absent.

The diagnosis is established by CT or MRI. Subdural hematoma is typically seen as a hyperdense or

isodense area (depending on the time elapsed since the traumatic event), either crescent shaped or
closely applied to the skull unlike epidural hematoma, a subdural hematoma is poorly demarcated from
the underlying brain tissue.

The treatment is neurosurgical. Therapeutic anticoagulation is a risk factor for the development of a
chronic subdural hematoma.
Complications of traumatic brain injury. Frequent late complications of severe traumatic brain
injury include neurasthenic phenomena, mental and personality changes, and symptomatic epilepsy.
Focal brain lesions cause deficits according to their localization. Posttraumatic epilepsy is seen within
two years in 80 % of the patients with brain injury in anamnesis, but it can also arise many years after the
initial trauma in rare cases. The severity of posttraumatic mental and personality deficits is positively
correlated with the length of the initial loss of consciousness and with the duration of retrograde and
anterograde amnesia around the time of the injury. Both short- and long- term memory are impaired.
The patient has difficulties to cope with complex tasks and situations and is easily get tired. Impatience,
irritability, diminished initiative, poor concentration, apathy characterize the patient's behavior. Rarer
posttraumatic phenomena include malresorptive hydrocephalus. It arises most commonly after a
traumatic subarachnoid hemorrhage and consists of an impairment of CSF flow and resorption due to
adhesions of the arachnoid and of the arachnoid granulations.

13. Closed craniocerebral and spinal trauma | 97

Spinal trauma
Traumatic spinal cord lesions are usually due to fractures and dislocations of the spine causing
displacement of fragments of a bone and/or intervertebral disk. The spinal cord can also be compressed
by a traumatic hemorrhage in the spinal canal or direct traumatic compression in the absence of a
fracture. Clinical signs of spinal cord trauma depend on the level and severity of the lesion.
Spinal cord trauma can be classified like this: concussion, contusion, compression, hemorrhage into
the grey matter of the spinal cord (hematomyelia) or in the adjacent meningeal compartments
(hematorrhachis).
Spinal cord concussion. This is spinal cord injury arising at the moment of a spinal trauma. The
manifestations may be purely sensory (transient pares- thesis), but are more often mixed motor
(transient mild paresis of the extremities) and sensory, and there may initially be a complete spinal cord
transaction syndrome identical to that of the acute shock phase seen in other patients with irreversible,
complete spinal cord transection syndrome (conductive disorders of sensitivity and paresis of
extremities below the injured area with impairment of pelvic oigans function). The distinctive feature is
that spinal cord concussion resolves spontaneously and completely, usually within minutes, hours and.
at most, within 3 days.
Spinal cord contusion. This term refers to the traumatic destruction of spinal cord tissue by direct
mechanical compression or hemorrhage, which may be the result of: a dislocated vertebral fracture, a
free bony fragment, a herniated intervertebral disk, or a repositioned subluxation of vertebra. The initial
clinical presentation is usually that of partial or complete spinal cord transaction syndrome (depending
on the extent of the lesion), including stable segmentary and conductive sensor disorders, bladder
dysfunction, paraparesis (paraplegia) or tetraparesis (tetraplegia) with following trophic disorders. The
transection syndrome usually improves no more than partially. Early recovery of sensation seems to be
a favorable prognostic sign for motor recovery.
Spinal cord compression. The spinal cord can be mechanically compressed by a herniated
intervertebral disk, a bone fragment, or a spinal epidural hematoma (hematorrhachis). Spinal epidural
hematoma arises spontaneously, as a complication of trauma. Intense local back pain is felt at first (root
pain), meningeal symptoms, followed within hours or days by neurological signs of spinal cord
involvement. In the case of compression of the spinal cords occur paresthesia, conductive disorders of
sensitivity and mild paresis of extremities below the injured area with the impairment of pelvic organs
function.
Hemalomyelia is a confluent hematoma in the central region of the spinal cord (grey matter), usually
extending over several segments. It is typically manifested as a partial spinal cord transection syndrome,
often with a dissociated sensory deficit in the dermatomes of the affected segments, segmentary motor
disorders, or else a Brown-Sequard syndrome. The most common cause of this trauma is a fall onto the
buttocks, diving into shallow water. The neurologic deficit commonly worsens over the next hours and
days, while its clinically determined level rises. Local pain is common. The lower cervical cord is a
preferred site of injury.
Diagnostic of acute spinal cord trauma includes a gentle, nontraumatic neurological examination to
determine the level of the lesion; plain radiography of the spine of the suspected level of injury to
identify fractures and dislocations of the vertebral column and assess damage to the intraspinal
structures. A lumbar puncture in the case of spinal epidural hematoma or hematomyelia reveals bloody
or xanthochromic CSF. MRI documents ongoing compression of the spinal cord, extent, and type of
spinal cord 98 I PAR Г 2: Special Neurology
injury.
Treatment of spinal cord trauma The patient must be correctly positioned, and repositioned every 2
hours, to prevent decubitus sores. It is also essential to prevent over distension of the bladder, cystitis.
This is accomplished at first by regular catheterization of the bladder with strict aseptic technique.
Immediate neurosurgical exploration via laminectomy is indicated as an emergency measure in a patient
with a complete or incomplete spinal cord transection syndrome. Immediate improvement can follow
evacuation of a spinal epidural hematoma or the removal of a bone fragment hindering blood flow to the
spinal cord. It is necessary to treat post-traumatic spinal pain. Rehabilitation begins at the time of injury.
 Tests and typical tasks

1. Indicate the clinical sign of the brain concussion

a) pareses and paralyses
b) agnosia
c) loss of consciousness
d) increasing, of the corneal reflexes
e) motor aphasia

2. In which form of craniocerebral injury "lucid space" is observed?

a) epidural hematoma
b) brain concussion
c) brain contusion
d) intracerebral hematoma
e) subarachnoid hemorrhage

3 What kind of craniocerebral injury is not accompanied by focal signs?

a) brain contusion
b) intracerebral hematoma
c) epidural hematoma
d) subdual hematoma
e) brain concussion
4. What additional diagnostic method confirms subarachnoid hemorrhage?
a) ophtalmoscopy
b) lumbar punction
c) echoencephaloscopy
d) electroencephalography
e) cranial x-ray examination

5. Which method is the most important in the diagnostics of contusion of the spinal cord?
a) CT of the spinal cord
b) ultrasound
c) evoked potentials
d) electromyography
e) MRI of the spinal cord

6. During a head trauma a patient lost consciousness. In 5 min he didn't remember the events before and
after the trauma. On examination there are anisoreflexia of the knee reflexes, weakness of
convergence of the eye balls, decreasing of the corneal reflexes, there are present oral automatism
reflexes. What is the preliminary diagnosis?

7. In 3 hours after a closed craniocerebral injury with a brief, transient impairment of consciousness
lasting not more than 2 min and disseminated neurological microsymptoms a patient suddenly lost
consciousness. By examination the dilatation of the right pupil and hemiparesis on the left side are
revealed. What is the preliminary diagnosis?

8. During a head trauma a patient lost consciousness, then he felt a headache, nausea, numbness and
weakness in the right hand. On examination - central paresis of the right hand, decreasing of the
corneal reflexes. What is the preliminary diagnosis?
13. Closed craniocerebral and spinal trauma | 99

9. A patient with craniocerebral trauma was admitted to the hospital with a headache, vomiting,
psychomotor irritation, neck stiffness. Focal neurological deficit is absent. What additional
examination is necessary?

10. After a head trauma a patient suffers from a sharp headache with vomiting, pain while moving the
eye balls and sleep disorders. Meningeal and vegetative disorders are present. Focal neurological
symptoms are absent. What is the preliminary diagnosis?
 14. BRAIN AND SPINAL CORD TUMORS. SYRINGOMYELIA

Brain Tumors
Tumors of the nervous system occur at any age and account for 10 % of all oncological diseases in
adults and 20 % in children. Mortality due to intracranial tumors is the same as from stroke. In children,
primary brain tumor is the second most widespread type of cancer after leukemia and constitutes 22 %
of all oncological diseases in children.
The classification of brain tumors with regard to topographic anatomy is based on the relation to the
cerebellar tentorium. There are supratentorialtumors (of cerebral hemispheres and of the basal surface
of the anterior and middle cranial fossa) and subtentorial tumors (of the cerebellum, brainstem, fourth
ventricle, cerebellopontine angle, posterior cranial fossa). This distribution is due to the peculiarities of
a surgical approach, as well as the clinical presentation: the predominance of focal symptoms over
general symptoms in supratentorial tumors and vice versa in subtentorial tumors. In adults, two thirds of
tumors have supratentorial localization; in children, in contrast, two thirds of tumors are subtentorial
tumors.
There are also primaiyml metastatic brain tumors. Primary tumors, depending on tiie maturity of the
cells and growth rate, are divided into benign and malignant iumors. However, limited intracranial
space, possibility of brain compression, as well as the difficulty of approach during surgical
interventions make benign tumors semimalignant. Malignant brain tumors rarely metastasize outside
the boundaries of the brain, but brain metastases of extracerebral tumors are quite common, especially in
cancer of the lung, breast, kidney and skin (melanoma).
Theie aie extracerebral tumors (neuromas, meningiomas, pituitary adenomas, etc.) and intracerebral
tumors.
According to the modern histogenetic classification, there are 10 groups of brain tumois. The most
common are: neuroectodermal tumors (astrocytoma, glioblastoma, oligodendroglioma, ependymoma,
choroid plexus tumors), tumors of cranial and spinal nerves (neurinoma, neurofibroma), and brain
membrane tumors (meningioma, bone and cartilage tumors, lipoma, chondrosarcoma, brain membrane
sarcomatosis, melanocytoma, hemangioblastoma).
Biology of some tumors in children (craniopharyngioma, meduloblastoma, teratoma, dermoid cyst) is
associated with dysembryogenesis. The most common cause of tumors are acquired single mutations in
somatic cells that occur under the influence of unfavorable environmental factors, viral infections,
trauma, intoxication. Some tumors are hormone-dependent.
Brain tumors have infiltrative, diffuse (glioma) or expansive, nodular (meningioma, neurinoma)
growth. In infiltrative growth the tumor invades the surrounding structures. Expansive growth is a
growth of conglomerate, delimitation of the medulla, and its compression.
Characteristics of some brain tumors. Among neuroectodermal tumors, gliomas are the most
common ones. The most frequent is astrocytic glial tumor — astrocytoma. It can have both infiltrative
and expansive growth. This is a benign tumor that grows slowly, but its malignant transformation is pos-
sible. In adults, it is localized in the hemispheres of the brain; in children — in the hemispheres of the
cerebellum, brainstem, optic nerves.
Glioblastoma is a malignant glial tumor. It occurs in adults, mostly men, in the temporal lobe of the
brain. It has a large number of vessels and rapid stroke-like progress. There may be hemorrhage into the
tumor. Life expectancy does not exceed 9-12 months.
Ependymoma is the tumor of ependyma cells, lining the ventricles of the brain and the central spinal
canal. Intracranial localization occurs at a young age; the tumor grows into the ventricular cavity.
13. Closed craniocerebral and spinal trauma | 100
 Choroid plexus papilloma grows from the neuroepithelium of choroid plexus of lateral or fourth
ventricle in children. It is a benign tumor with an expansive growth. '
About 20 % of central nervous system tumors occurring in children are medulloblastomas. This is an
embryonal tumor of the vermis and the hemispheres of the cerebellum, growing into the cavity of the
fourth ventricle. It has a rapid infiltrative growth and metastasis in cerebrospinal fluid pathways.
Neurinoma is a benign tumor of the cranial (usually pair VIII) or spinal nerves. It has a slow growth.
Meningioma is a benign extracerebral tumor originating from the cells of the dura mater or the
arachnoidea mater of the brain.

Pituitary adenoma is a benign tumor of the adenohypophysis It may be either honnonally active with
an infiltrative growth, or inactive. The characteristic features are neuroendocr ine disorders
(acromegaly, Cushing's syndrome, gonadal dysfunction), the enlargement of the sella turcica,
bitemporal hemianopsia.
Clinical presentation of brain tumors includes general and focal neurologic symptoms. General
symptoms are the result of an increased intracranial pressure, which occurs due to the growth of the
tumor, compression of the cerebrospinal fluid pathways, and disorder of the venous blood outflow and

development of brain edema. The severity of hypertensive syndrome depends on the location of the
tumor. General symptoms are predominant in subtentorial tumors. Supratentorial tumors are located
away from the cerebrospinal fluid pathways and therefore in the early stages often only focal symptoms
appear. The classic triad of symptoms of intracranial hypertension: headache, vomiting, papilledema
(disk edema of the optic nerves).
Headache is bursting and diffuse, occurs at night and morning, and is aggravated by coughing,
exertion. Vomiting appears at the height of headache without previous nausea, is not related to food
intake, and often leads to reduction in headache. Papilledema is shown with an increase in their size,
bulging, indistinct outlines, venous distensibility, hemorrhages in the fundus. Prolonged papilledema
leads to a loss of vision due to the development of secondary atrophy of the optic nerves. The general
symptoms also include dizziness, seizures, psychiatric disorders. The characteristic signs are a lack of
initiative, apathy, decreased attention, stupor. In infants, liquor hypertension is shown by an increased
head circumference, separation of cranial sutures, bulging of the fontanelle.
Focal symptoms are divided into primary (caused by the direct effect of the tumor on the brain
substance, and shown by the symptoms of irritation or a loss of function), and secondary (which in turn
are divided into adjacent and distant symptoms). Adjacent symptoms are related to the compression of
the surrounding areas of the brain, swelling, and an impaired blood flow in them.
Distant symptoms are caused by swelling and dislocation of the brain into natural orifices or reserve
space of the skull in the late stages of tumor development. For example, cerebellar tonsils herniation in
the foramen magnum.
Focal symptoms of supratentorial tumors. Frontal lobe tumors are characterized by psychiatric
disorders, which are referred to as "frontal mind" (reduced criticism and self-criticism, tendency to flat
jokes, untidiness, dis- inhibition of sexual and food instincts, loss of social skills, lower IQ). Lesions of
the basal paits of the frontal lobes manifest themselves in aggressiveness and dysphoria, while
convexital lesions are shown by euphoria. Apathy abulia syndrome (a lack of will to act) is possible,
with the inhibition of mental processes, inertia, aspontaneity. Tumors of the frontal lobe are also
characterized by frontal ataxia in the opposite extremities with astasia (standing dysfunction) and abasia
(walking dysfunction).
Jacksonian seizures indicate irritation, and the central monoparesis indicates damage to the precentral
gyrus. There is a possibility of occurrence of Yan- ishevsky grasping symptom in the wrist opposite to
the lesion side and reflexes of oral automatism. Tumors in the left hemisphere result in motor aphasia
and agraphia.1(52Damage of Neurology
І PART 2: Special the basal surface of the frontal lobe is characterized by ophthalmoscopic Foster
Kennedy syndrome: on the affected side occurs primary atrophy of the optic nerve due to compression
of the optic nerve by the tumor; on the opposite side occurs papilledema caused by liquor hypertension.
Temporal lobe tumors are characterized by seizures with olfactory, gustatory, auditory hallucinations;
a feeling of derealization ("deja vu" (already seen) or "jamais vu" (never seen)), complex partial seizures
with the impairment of consciousness and automatisms, homonymous upper quadrant hemianopsia,
temporal ataxia. Damage of the left superior temporal gyrus (Wernicke's center) leads to sensory
aphasia. Large tumors of the temporal lobe very quickly lead to the development of symptoms of
displacement and wedgeing.
Parietal lobe tumors irritating the postcentral gyrus cause sensitive Jackson seizures in the form
of paresthesias in limited parts of the body. If the tumor invades the postcentral gyrus, the
symptoms of irritation are replaced by loss of functions (loss of superficial and deep sensitivity).
Damage of the superior parietal lobule leads to astereognosis. Apraxia is characteristic of tumors
in the supramarginal gyrus. Autotopagnosia is a sign of right hemisphere damage. Localization of
the lesion in the deep parts of the parietal lobe leads to lower quadrant hemianopsia.

If occipital lobe tumor irritates its medial surface, there appear simple visual hallucinations
(photopsia) in the form of light flashes and lines in the opposite visual fields. In the event of damage of
this part of the brain occurs homonymous quadrant hemianopsia. Damage of the outer surface of the
occipital lobe is accompanied by visual agnosia. The occurrence of metamorfopsy (a distorted
perception of shape and size of objects) is possible.
Corpus callosum tumors are characterized by mental disorders and reduced memory.
Focal symptoms of subtentorial tumors are preceded by liquor hypertension. The most common are
tumors of the cerebellum and fundus of the fourth ventricle, as well as acoustic neurinoma.
Cerebellum tumors in 70 % of cases occur in children. In the case ot damage ot the cerebellar vermis,
on both sides occur statico-locomotory ataxia, muscular hypotonia, nystagmus, asynergy, a forced head
position with a forward lean. Localization of tumors in the cerebellar hemisphere leads to dynamic
ataxia with muscular atony of the limbs on the affected side, scanning speech, nystagmus.
Fourth ventricle tumors are accompanied by bouts of liquorodynamic disorders in the form of a
sudden headache, vomiting due to the irritation of the vomiting center in the medulla, disorders of
consciousness, respiratory and cardiac failure.
Among cerebellopontine angle tumors, acoustic neurinoma is the most common one. An early
symptom is noise in the ear with a subsequent hearing loss and vestibular disorders (vertigo,
nystagmus), signs of raducular damage to VII and, over time, V and VI pairs of cranial nerves. If the
tumor grows in the caudal direction, the process involves the roots of IX, X, XI and XII pairs of cranial
nerves. Widening of the internal auditory canal is observed in a combination with osteoporosis of the
apex of the temporal bone pyramid.
Diagnosis of brain tumors includes, in addition to neurological examination, a study of the eye
fundus, visual acuity, visual fields, hearing and vestibular function. The most informative are CT and
MRl studies of the brain, particularly with the use of a contrast medium. Computed tomography is not
informative for small tumors of the posterior cranial fossa (especially in the brain stem). In this case, the
most sensitive method for diagnosis ot brain tumors is used — MRl with gadolinium contrast. In recent
years, the method of positron-emission tomography is being introduced, which gives the oppor- trinity
to study metabolic processes in the brain and tumors by analyzing the distribution of short-lived
radionuclides in the brain. To study the histological structure of tumors, stereotactic biopsy is used.
In supratentorial tumors, electroencephalography shows focal slow-wave activity.
Echoencephalography detects the displacement of midline structures of the brain, indicating the
presence of tumor with its hemispheric localization.
Treatment of brain tumors is mostly surgical.
Radical resection is possible in the cases of extracerebral tumors (meningioma, neurinoma, pituitary
adenoma), as well as in some forms of gliomas. Partial resection of tumor is carried out for
non-resectable tumors because of their location and an infiltrative growth with the aim of
decompression in the cases of liquor hypertension to reduce the tumor to the size required for radiation
therapy.
In the cases of non-radical resection or malignant tumors, chemotherapy is used. Radiation therapy
provides a remote irradiation of the tumor and is used for the treatment of pituitary adenomas, some
forms of glioma, tumors of the base of the skull, pineal gland, brain stem, ependymoma,
medulloblastoma, and the presence of metastases.
Medication therapy of brain tumors is symptomatic and aimed at reducing intracranial hypertension
and cerebral edema. For this purpose, osmotic diuretics, saluretics, corticosteroids and, if necessary,
anticonvulsants are used,
Spinal cord tumors
Tumors of the spinal cord occur 8-10 times less often than brain tumors.
Classification. Tumors of the spinal cord are divided into intramedullary (intraspinal), and
extramedullary (outside the spinal cord matter). Based on the relation to the dura mater, extramedullary
tumors are divided into subdural (Fig. 14.4) and extradural. Extramedullary tumors account for 80 %
and intramedullary — only for 20 % of all spinal tumors.
Depending on the location, there are tumors of the cervical spinal cord (18 % of all spinal cord
tumors), thoracic (66 %), lumbar (15 %) and sacral і (1 %). Tumors of the spinal cord may be the
primary and secondary (metastatic) ones. Metastatic tumors are extradural^ located metastasis of lung
cancer ! or sarcoma, breast cancer, prostate or kidney cancer, as well as melanoma and lymphoma. Also,
there are extradural tumors which grow from the spine or surrounding soft tissues.
Extramedullar subdural tumors are usually benign. 75 % of them are meningiomas and neurinomas.
Meningioma grows from the dura mater. Neurinoma accounts for 20-30 % of all spinal cord tumors and
grows from Schwann cells of the posterior spinal roots, which causes early radicular pain.
Predominant among the intramedullary tumors are gliomas (ependymoma, astrocytoma and
glioblastoma) located at the cervical or lumbar intumescence. They grow from structures of a spinal
cord segment, therefore they debut with segmental disorders.
Ependymoma accounts for approximately 20 % of all spinal tumors and grows from the ependyma of
the walls of the spinal cord central canal at the level of the cervical or lumbar intumescence, but may
also be located in the terminal liquor cistern, growing around the roots of the cauda equina. Glio-
blastoma is an immature malignant glioma with rapid infiltrative growth, which makes it inoperable.
A progressive growth of any spinal tumor over time leads to the damage to the spinal cord
cross-section, but the initial stages of extra- and intramedullary tumors differ significantly.
Clinical presentation. The development of extramedullar tumor consists of the following stages:
radicular, damage to a half of the spinal cord cross- section, a total transverse damage of the spinal cord.
The radicular stage is clearly manifested in the neurinoma of the posterior (sensitive) spinal nerve
root. An early clinical sign is radicular pain, followed by disorder of all kinds of sensitivity by the
segmental type in the area of innerva-
tion of the affected nerve root and the impairment or disappearance of reflexes. Radicular pain increases
during coughing (cough impulse symptom), sneezing, straining, physical exertion, a sudden tilt of the
head (Neri's radicular sign). Spinous process sign (Razdolsky's sign) is associated with root irritation:
tapping on the spinous processes of vertebrae induces pain and paresthesias in the lower pail of the
body. This sign helps to determine the localization of the tumor..
Hemisection of the spinal cord(Brown-Sequard syndrome) is characterized by an impaired superficial
sensitivity of the conductive type on the opposite side and by the segmental type on the affected side,
disorders of deep sensitivity of the conductive type on the affected side, as well as central paresis on the
side of and below the location of the tumor. Comression of the lateral cord by the tumor results in the
gradual spread of conductive disorders of the superficial sensitivity from the bottom up because of the
eccentric location of the longest conductive tracts in the spinothalamic pathway.
Transection of the spinal cord is characterized by disorder of all kinds of sensitivity of the conductive
type below the tumor, movement disorders, disorders of pelvic organs and trophism. The patient is
threatened by bedsores and urosepsis.
Ginical presentation of intramedullary tumors has a different sequence of development, which also
consists of three stages: segmental stage, transection of the spinal cord, radicular pain. Intramedullary
tumors grow from the gray matter of the spinal cord, and the segmental stage is characterized by dis-
sociated segmental disorder of superficial sensitivity at the level of the tumor.
Transection of the spinal cord occurs when the tumor invades tiie white matter. Segmental sensory
disorders are replaced by conduction disorders; motor and trophic disorders and pelvic organs
dysfunction appear. Only at the stage of radicular pain, when the tumor extends beyond the spinal cord
to the roots, radicular pain appears. 14. Brain arid spinal cord tumors. Syringomyelia | 103
Diagnosis of spinal cord tumors requires X-ray of the spine to detect destruction of vertebral bodies
and arches, vertebral osteoporosis at the tumor level, compression fractures and dislocations due to
gross destr uctive changes, and intervertebral foramen widening. The most informative are the CT of the
spine and MRI of the spinal cord. With unavailability of CT and MRI, liquorody- namic testing is
performed, which allows during the lumbar puncture to detect disorder of passability of cerebrospinal
fluid pathways. Studies of cerebrospinal fluid reveals elevated levels of protein in the cerebrospinal
fluid and normal or slightly increased number of cells (protein-cell dissociation). Myelography using
contrast agents allows determining the boundaries of the tumor.
Treatment. Spinal cord tumors can only be treated surgically. The effectiveness of the surgery
depends 011 tumor histology and its stage of development. The radical removal of extramedullar benign
tumors (neurinomas, meningiomas) is possible. A total removal of intramedullary tumors is sometimes
possible in cases of ependymomas, much more difficult for astrocytomas, and almost impossible for
glioblastomas. In malignant tumors, radiotherapy and chemotherapy are used. Symptomatic therapy is
aimed at preventing urosepsis, bedsores, and vascular disorders.

Syringomyelia
Syringomyelia is a chronic, slowly developing disease of young and middle- aged people, which is
characterized by the formation of cavities in the spinal cord, mainly at the level of cervical
intumescence. The frequency of syringomyelia is 2.5—9.3 % of all organic lesions of the nervous
system. The highest incidence occurs among persons who are engaged in a heavy physical labor. Men
suffer 2-4 times more often than women. A disease onset is between the ages of 10-35.
Macroscopically, on cross-sections of the spinal cord, cavities of different diameters (from 1-1.5 cm
to barely noticeable) are visible. They are located in the canalis centralis and distributed mainly to the
posterior horns and posterior funiculus.

At the present time, the hydrodynamic theory of the formation of cavities of syringomyelia is
dominant. According to this theory, patients with various abnormalities in the foramen magnum suffer
from the obstruction of cerebrospinal fluid outflow from the IV ventricle into the subarachnoid space
of the spinal cord. Under pressure, cerebrospinal fluid enters the central channel, expands it
(hydromyelia) and forms a cavity. Various abnormalities occur in 80 % of patients with syringomyelia.
Often the development of syringomyelia is associated with Arnold — Chiari malformation I. This
abnormality includes hypoplasia of the posterior cranial fossa and descent of the cerebellar tonsils
below the foramen magnum.
Defects in the development of the spinal cord are also accompanied by the impaired development of
other organs and tissues. This is manifested in the | signs of status dysraphicus. They may include
curvature of the spine, funnel chest, deformation of hands and feet, extra nipples, high palate, abnormal
teeth, etc.
Unlike true syringomyelia, a variety of necrotic, ischemic and adhesive processes in the spinal cord
can also lead to the formation of gliornatosis with forming of cavities. In such cases we speak of
secondary syringomyelia. This I is possible after hematorrhacliis (bleeding in tlie spinal cord), spinal
cord injury, necrotic myelitis, benign spinal tumors, etc.
Clinical presentation. The classical manifestation of syringomyelia is the ! disease of the entire gray
matter at the level of cervicothoracicus of the spinal 1 cord. This presupposes the development of a
typical triad of symptoms:
► segmental dissociated type of sensory disorder,
► peripheral paresis of the upper extremities,

► segmental vegetative (trophic and vascular) disorders.

Sensory disorders are the most characteristic of the disease. This type of I disorders is also called
"syringomyelitic". It is characterized by a loss of pain і and temperature sensitivity in the arms and
upper body, while maintaining
104 | PART tactile and kinesthetic senses ("jacket" sensory loss).
2: Special Neurology
"Deep" pain may occur in different locations. It has a dull, aching quality, sometimes
accompanied by paresthesias, often with hyperpathia. Pain may occur long before the emergence of
objective signs of disease. Sometimes patients initially consult with a surgeon or a traumatologist on
wounds or burns that do not hurt, but heal poorly.
Movement disorders in the upper limbs are manifested in flaccid, atrophic paresis, predominantly
localized in distal aspects. They are characterized by a weight loss of small muscles of hands with the
formation of "a clawed hand" or "a monkey paw". Fibrillar twitchings are often observed in atrophied
muscles.
Tendon and periosteal reflexes are reduced or lacking.
 The spread of the cavity to the lateral funiculus is accompanied by the signs of destruction of
conducting motor and sensory paths — spastic paraparesis of the legs, conductive sensory disorders. A
rarely occurring lumbo-sacral form of the disease is characterized by the same sensory and motor
disorders in the legs.
Vegetative-trophic disorders are varied. There may be sweating disturbances in the form of
hyperhidrosis (loss commonly, anhidrosis) on the face, arms and torso. There may be peripheral
circulatory disorders, manifesting in hyperemia and acrocyanosis. Over time, vegetative-trophic
disorders are increasing. Symptoms include dry, flaky skin; hyperkeratosis with deep cracks or poorly
healing ulcers. The patients may experience hypo- or hyperpigmen- tation, nail crumbling and breaking,
trophic disturbances of bone and articular apparatus in the form of kyphoscoliosis of the thoracic spine,
arthrosis, osteoarthropathy, pathological dislocation of joints, cheiromegaly (the enlargement of hands
and fingers). Horner's syndrome is often observed.
Syringomyelia is often accompanied by syringobulbia, which can itself be a manifestation of the
disease. The process permeates to the medulla oblongata and pons. Nuclei of cranial nerves are affected,
most commonly pairs V, IX, X, XII. Patients experience aching facial pain, temperature and pain
hypesthesia on,the face in Zelder zones while maintaining the tactile sense, nystagmus is revealed.
Often there are signs of bulbar syndrome — violated phonation, articulation, swallowing, atrophy of the
tongue with fibrillar twitching in the muscles.
Diagnostics. The most informational method of visualizing the spinal cord with suspected
syringomyelia is MRl study, which shows syringomyelic cavities.
Treatment of patients with syringomyelia should be comprehensive, uninterrupted and include the
use of amino acids, protein, vitamins, and nootropic and anticholinesterase drugs. It is desirable to
conduct treatment courses 2-3 times a year. The indications for neurosurgical treatment are: rapid
progression of the disease, augmentation of liquorodynamic disorders, craniovertebial anomalies.
Surgical treatment includes bypass surgery with cerebrospinal fluid drainage into other cavities and
decompression of craniovertebral junction.
The prognosis for survival is relatively favorable; prognosis for recovery is poor.

Tests and typical tasks

1. What tumor belongs to supratentorial?
a) parietal lobes' tumors
b) spinal cord tumors
c) medullar tumors
d) cerebellar tumors
e) IV ventricle tumors

2. What tumor belongs to subtentorial?

a) frontal lobes' tumors
b) III ventricle tumors
c) medullar tumors
d) temporal lobes' tumors
e) occipital lobes' tumors

3. Indicate the hypophyseal tumor.

a) meningebma
b) astrocytoma
c) meclulloblastoma
d) adenoma
e) neurinoma

4. What tumor is most commonly localized in pontinocerebellar angle?

14. Brain arid spinal cord tumors. Syringomyelia | 105
a) meningeoma
b) neurinoma
c) astrocytoma
d) medulloblastoma
e) ependimoma
5. Indicate additional diagnostic: method foe spinal cord tumors.
a) echoencephaloscopy
 b) angiography
c) electroencephalography
d) electromyography
e) myelographia
6.During 3 months a patient has been suffering from a headache, especially in the morning and at
night. Sometimes he has vomiting. Gradually unsteadiness at walk, intense tremor at finger-nasal and
heel-to-shin test fulfilled by the right limbs and nystagmus at a look to the right have appeared. The
patient is staggering right in Romberg posture. Establish the clinical diagnosis. Describe the possible
fundus changes.
7. A patient developed a girdling pain in his right thoracic half at the level of Th7-Th8 segments. Some
time later there appeared weakness in his right foot with the increase of muscle tone and reflexes;
there was a conduction disturbance of superficial sensitivity from ThlO level on the left and
segmental one at Th7-Th8 level on the right. The liquor examination revealed protein-cell
dissociation. What is the clinical diagnosis?
8.During last 4 months a woman of 40 years old suffers from a headache and periodical short-term
clonic spasm attacks in her right leg. She did not pay necessary attention to these attacks. She applied
for medical assistance only when during the last week there appeared weakness in the right leg.
Central paresis of the right leg was defined. On the eye-ground there are edema optic nerve discs
Which disease should be suspected and how is it possible to prove it?
9. Few months patient complained of a pain in his back and was treated as radiculitis. Because of the
inefficiency of the treatment lumbar puncture was fi ilfilled. There were found 2 cells/ 1 mm3 and 6
grams/liter protein in patient's cerebrospinal fluid Wl rat kind of pathology is it?
10. A patient has gradually had the impairment of pain and temperature arms sensiv- ity. Arm muscles
have grown weak and thin. At neurological examination there were detected pain and temperature
sensivity hypesthesia, preserved tactile sen- sivity in segments both sides C5-TI, left sided Horner's
syndrome, arms paresis with muscle atrophy, deep reflexes areflexia: Define the preliminary
diagnosis. What additional research methods should prove this diagnosis?

106 | PART 2: Special Neurology

 15. EPILEPSY. NONEPILEPTIC PAROXYSMAL STATES

Epilepsy is a chronic disease of the brain. The basic signs include recurrent spontaneous unprovoked
paroxysmal attacks with motor, sensor/ or psychic phenomena which are the result of hypersynchronic
discharges in the neurons of the cortex.
The word "epilepsy" is of Greek origin and has the same root as the verb "epilambano," which means
"to seize" or "to attack." About 1 % of population suffers from epilepsy, in the world it's about
30.000.000 people. In 75 % cases epilepsy starts in childhood or the juvenile period. Many famous
people (Pythagoras, Democritus, Alexander of Macedon, Julius Caesar, Avicenna, Napoleon
Bonaparte, Vincent Van Gogh, Moliere, Leo Tolstoy) suffered from epilepsy. But epileptic seizures,
especially in childhood, happen much more often than the disease itself. Incomplete processes of
myelinization, increased cerebral hydrophilia, metabolism lability and susceptibility to stimulation
generalization in children contribute to this pathology. About 2 % of adults have a seizure at some point
in their life. Two thirds of these people never have another one. Seizures can be among the most
dramatic foi ms of nervous system dysfunction. Seizures have many different causes and
manifestations.
Etiology. Epileptic seizures can be produced by structural lesions in the brain: scar, tumor, congenital
malformations; by metabolic disturbances, toxic influences or may be genetic predisposition to epileptic
seizures in the absence of a structural lesion.
Pathogenesis of epilepsy. Epilepsy reflects the abnormal functioning of cerebral neurons. In general,
a neuron receives both excitatory and inhibitory influences from other neurons, and fires an action
potential only when the overall effect of the excitatory postsynaptic potentials outweighs that of the
inhibitory postsynaptic potentials. Intraneuronal recordings from epileptic foci have revealed a
membrane depolarization of abnormally high amplitude that piovokes the firing of a series of action
potentials at high frequency, followed by hyperpolarization. This type of electrical event is called a
paroxysmal depolarization shift. The following processes play an important role in neuronal
depolarization and repolarization: calcium and sodium influx and potassium efflux, excitatory amino
acids such as glutamate, and inhibitory neurotrans- mitters such as gamrna-ammhubjric acid (GABA).
These processes are the basis of various kinds of anticonvulsant therapy.
Epileptic focus. It is the pathologic neurons system, which is able to spontaneous periodic
self-excitement and less dependent on external factors.
By etiology epilepsies divided into 3 forms.
Idiopathic epilepsy. There is no underlying cause other than a hereditary predisposition.
Symptomatic epilepsy. It is considered to be the consequence of a known or suspected disorder of the
central nervous system (due to organic disturbance of the brain).
Cryptogenic epilepsy. The term refers to a disorder whose cause is unidentified, hidden. It is presumed
to be symptomatic, but there is no clear evidence of an etiological factor. The cryptogenic epilepsy
often does not have well defined electro-clinical characteristics.

Characteristics of idiopathic forms of epilepsy

The main reason of idiopathic forms is hereditaiy canalopathy with diffused membrane instability.
Idiopathic epilepsies are defined by age-related onset (starts in childhood or teenagers' years), usually
characterized by general seizures, the absence of neurological symptomatic, the absence of intellectual
disturbances and abnormal
107 I PART EEG and MRl and have a benign prognosis. If one parent suffers from
2: Special Nourology
idiopathic epilepsy, the child's risk is 1:25.

Characteristics of symptomatic epilepsy

Reason. The perinatal period— hypoxia of the fetus and new-born, cortical dysplasia, anomaly of the
brain, delivery trauma, toxemia of the pregnant, infectious diseases of the fetus and new-born.
The postnatal period— craniocerebral injury, neuroinfections, neurointoxi- cations, artery-venous
malformations, stroke.
In adults— most often cause is cerebrovascular pathology of the brain. Patients with symptomatic
epilepsy usually have partial seizures.
Most characteristically for symptomatic epilepsy are partial seizures, focal symptoms in the
neurological status and focal lesions on MRl, focal abnormalities on the EEG, intellectual disturbances.
Epileptic reaction is the response of the brain to the strong external and internal damaging factors
(such as electro-shock, insulin shock, brain hypoxia, severe alcohol intoxication, metabolic disorders:
hypocalcaemia, hypoglycemia, hyponatremia. In children, fever can provoke a seizure.
International classification of epileptic seizures is based on the clinical expression of the seizure and
the electroencephalographic picture during and between seizures. The main division in this
classification is into partial and generalized seizures (Table 15.1).

Table. 15.1
International classification of epileptic seizures (1981)

I. Partial seizures (seizures with local onset)

A. Simple partial seizures (consciousness not impaired)
1. With motor symptoms
2. With somatosensory or special sensory symptoms
3. Willi autonomic symptoms
4. With psychic symptoms
B. Complex partial seizures (with impairment of consciousness), beginning as simple
partial seizures and progressing to impairment of consciousness or with impainvent of
consciousness at onset
1. With motor symptoms
2. With somatosensory or special sensory symptoms
3. With autonomic symptoms
4. With psychic symptoms
5. With automatisms
C. Partial seizures secondaiy generalized
II. Generalized seizures (bilaterally symmetrical and without local onset)
A. Absence seizures
1. Typical absence seizures
2. Atypical absence seizures
B Myoclonic seizures
C. Clonic seizures
D. Tonic seizures
E. Топіс-сіопіс seizures
F. Atonic seizures
G. Infantile spasms
III. Unclassified epileptic seizures (inadequate or incomplete data)

Clinical characteristic of epileptic seizures

The epileptic seizure occurs suddenly. Usually it is very brief, most of them end in 1 to 3-5 minutes.
The main features ot epileptic seizures are spontaneous (less dependent on external surrounding),
regular (exact interval between seizures), stereotype, appearance in the same time of beginning,
progressively worse, development of cognitive and personality disorders, and very often patient feels
well after seizures.
Partial seizures. The abnormal electrical discharges start in a localized area of the brain. The
symptoms are dependent on which part of the brain is affected. These discharges may remain localized,
or they may spread to other parts of the brain and then the seizures become generalized (secondary
generalized seizures). Partial seizures are divided into two groups, simple partial seizures where
19fi I PART 2: Special Neurology
consciousness is maintained, patient completely remembers fits and complex partial seizures starts as
simple partial with the following impairment of consciousness without convulsion. The patient has
impaired consciousness, but not a complete loss. Organic symptoms in the neurologic status, focal
abnormalities on the EEG (focal slow or sharp waves' discharges from the presumed site), local lesions
on MRl, intellectual disturbances are present.
Partial seizures arise from a local area of the brain, In motor seizures, the focus is in the primary motor
cortex. There are twitchings, starting in a distal part of the extremity, or in the face (motor Jackson's).
The twitching may remain localized, or spread up the whole extremity and even involve the extremities
and face from the one side. Sensory seizures have their focus in the postcentral gyrus (the primary
 sensory cortex). There might be feelings of tingling, pins and needles, cold or heat, or numbness of a
limb (somatosensor or sensor Jackson's). Sometimes there may be strange feelings with visual signs, or
hearing or smelling sensations. Autonomic seizures are associated with the focus in the temporal lobe.
There may be: a strange feeling in the stomach rising up to the throat and head, palpitations, sweating
or flushing. The psychic symptoms may consist of changes in mood, memory, or thought
(thinking).There may be distorted perceptions of time (time seems to pass too slowly or too fast), light,
sound or space. Illusional seizures — difficulty in accepting reality objects (objects seem larger or
smaller than usually — syndrome of "Alice in Wonderland"), derealization feeling "dej& vu — ever
seen", "jamais vu — never seen". Sometimes the seizure occurs with complex hallucinations (music,
scenes) or with automatisms: pulling of the clothes, chewing, lip smacking, or repeated aimless
movements. Seizure with psychical disorders ї— a result of discharge in tire secondary and tertiary
associative cortex area, leads to dysfunctions of consciousness.
Secondary generalized seizures. With partial onset and following generalized tonic-clonic seizure
called secondary generalized (spreading of the excitement from source with participation of reticular
formation and subcortex). The name of the focal part of this seizure is aura.
Generalized seizures are generalized from the onset (i.e., primary generalized seizures), starting in
both hemispheres of the brain simultaneously.
General signs of generalized seizures — a complet loss of consciousness, the patient doesn't
remember fits at all, neurological symptoms and intellectual disturbances are absent, normal EEG and
MRI.
Tonic-clonic seizure. In such a seizure the patient loses consciousness, falls down, sometimes with
a scream, and develops a generalized stiffness (the tonic phase). Br eathing stops, as all the muscles of
the trunk are in spasm, and the patient becomes cyanotic, the head is retracted, the arms flexed and the
legs extended. During this phase the patient might bite his tongue. This tonic phase is followed by the
clonic piiase, when the muscles alternately contract and relax, resulting in clonic movements. At the
end of this phase the patient might pass urine. When the jerking stops and the patient regains
consciousness, he does not remember what happened, and may find himself on the floor in a strange
position. Often he falls into a deep sleep. The frequency of the seizure may vary from one a day to one
a month or once a year, or even once every few years. The tonic phase or the clonic phase can
predominate in the seizure, or may be only one of them.
Absence seizure. The word "absence" is of French origin. It means absent.
Typical absences begin in school-aged children around age 7 (childhood absence) or around ages
12-14 (juvenile absence) and can occur many times a day. These are short periods of consciousness loss
lasting only few seconds (not more than half a minute). They are of sudden onset, usually with no, or
only minimal motor manifestations. There is a blank stare, brief upward rotation of the eyes and an
interruption of ongoing activity. The patient is unresponsive when spoken to. It is suddenly over, and the
patient continues what he was doing before the seizure. The patient does not remember these seizures.
Seizures often occur when patients are sitting quietly, can be precipitated by hyperventilation, and rarely
occur during exercises. Neurologic and cognitive examination results are usually normal. EEG
characteristics of absences-— generalized three per second spike-and-wave discharges.

Infantile spasms are characterized by a sudden flexion and adduction of tlie arms and forward
flexion of the trunk. Seizures last a few seconds and recur many times a day. They occur only in the first
years of life, then are replaced by other types of seizures. Developmental defects are usually
present.
Atonic seizures occur most often in children, usually as part of Lennox —- Gastaut syndrome.
Atonic seizures are characterized by a brief, complete loss of muscle tone and consciousness. Children
fall down, risking trauma, particularly head injury.
Myoclonic seizures are characterized by quick symmetric
15. Epilepsy. muscular
Noneplleptic paroxysmal statesmovements,
| 109 either occurring in
one limb, or more widespread and bilateral. They may be single jerks, or jerks repeated.
Status Epilepticus
Status epilepticus — a single seizure lasting for more than 30 min or multiple seizures that are
so frequent, that there is no recovery of consciousness between them: This is a neurological
emergency. Seizures must be terminated as rapidly as possible to prevent permanent brain damage
as well as death from hypoxia, acidosis and secondary renal damage. Causes of epileptic status can
be: antiepileptic drugs withdrawal or noncompliance (subtherapeutic drug level), abstinence
syndrome, sleep deprivation, metabolic disturbances etc.

Diagnostics of epilepsy
Knowing if a person is having a seizure and diagnosing the type of seizure or epilepsy syndrome
can be difficult. There are many other disorders that can cause changes in behavior and can be
confused with epilepsy. Careful consideration of the medical history of a patient can deliver the
first hypothesis about the localization of a focus. Causal risk factors such as head injury or
encephalitis, infantile febrile convulsions or family history of epileptic seizures, age of onset of
first signs of epilepsy, and additional cognitive impairments repotted by the patient have to be
analyzed.
Electroencephalography (EEG) is still the only method that delivers functional proof of the
epileptic nature of the episodes in question. Evidence of brain potentials (spikes/sharp waves)
typical for epilepsy can be achieved in up to 90 % of patients by using repeated interictal (between
seizures) routine EEGs, including sleep registrations. Prolonged video and EEG recording can
"catch" and help to identify the fit.
A paroxysmal depolarization shift of neurons occurring in the midst of a population is reflected
on the EEG by spikes followed by a slow wave (spikes/ sharp waves). Such complexes may be the
initial EEG correlate of a clinically observable seizure. Generalized spike-and-wave discharges
are seen in primary generalized seizures. Three per second spike-and-slow wave patterns are char-
acteristic of absence seizures. Focal spike or spike-and-wave dischar ges may be seen in partial
epilepsies. Synchronous discharges may occur in active seizures.
CT and MRI of the brain may reveal focal str uctural lesions — potential causes of epilepsy,
including tumors, cortical dysgenesis, or mesial temporal sclerosis.

Treatment of Epilepsy
The ultimate goals in treating epilepsy are: to strive for a complete freedom from seizures, have little
to no side effects from medications, be able to follow an easy regimen so that compliance with treatment
can be maintained. About 75-80 % of people with epilepsy can be managed easily with one drug. This is
called monotherapy, and it prevents interaction between drugs, ensures good compliance and is also
cost-effective. However, the remaining 20-25 % of patients may require multiple drugs. Epilepsy
medications do not cure epilepsy; the medications can only control the frequency and severity of
seizures. The patient has to maintain a "seizure diary" where every seizure is recorded and reported to
the treating physician periodically. Each type of epilepsy has a drug that usually proves to be most
effective (Table. 15.2). Some medications lessen the sodium influx, others potentiate GABA-ergic
inhibition, and others selectively block calcium channels.
The best drug for a specific type of epilepsy should be started in a low dose, once or twice daily,
depending upon the nature of the drug. Dose escalation should always be under medical guidance and
needs to be undertaken slowly during follow-up, until either the seizures are controlled or undesirable
side-effects appear. Antiepileptic drug treatment should be continued till the patient has been totally
seizure-free for a minimum of three
110 І РАПТ 2: Special Neurologyyears. After successful completion of the course of treatment, the
drug should not be stopped abruptly; it should be withdrawn slowly over a period of several months.
When medications are clearly ineffective, the other types of therapy that can be considered brain
surgery, and vagal nerve stimulation. There are no special dietary restrictions for epilepsy. Special diets
such as the high fat diet known as "ke- togenic diet" has been of some help in children with intractable
epilepsy. The ketogenic diet is based on high-fat, low carbo-hydrate, and low-protein meals. The
ketogenic diet is named because of the production of ketones by the breakdown of fatty acids. The most
common version of the diet involves long-chain triglycerides. These are present in whole cream, butter,
and fatty meats,

Treatment of status epilepticus

Treatment begins with: secure airway, turn patient sideways, check vital signs, pulse oximetry,
cardiac monitor, control of blood glucose, electrolytes
Table. 15.2
Antiepileptic Drugs
Type of seizures Drug of choose Alternative Contra - Indication
Drugs
Partial seizures, secondary Carbamazepine Topiramate
generalized seizures Valproic acid Phenytoin
Oxcacarbazeplne Levetiracetam
Lamotrlgine Gabapentin
Clonazepam
Phenobarbital
Primary generalized Valproic acid Topiramate Ethosuximide
convulsive seizures Lamotrlgine Phenobarbital
Levetiracetam
Carbamazepine
Phenytoin
Clonazepam
Absence Ethosuximide Topiramate Phenytoin
Valproic acid Clonazepam Carbamazepine
Lamotrlgine Levetiracetam Gabapentin
Mioclonic seizures Valproic acid Lamotrigine Phenytoin
Clonazepam Carbamazepine
Topiramate Gabapentin
Levetiracetam
Phenobarbital

(K, Na, Ca), blood levels of the patient's antiseizure medication (if applicable), toxicology screen.
Status epilepticus should first be terminated with a bolus injection of benzodiazepine (diazepam 10-20
mg or lorazepam 2-4 mg). If a seizure is ongoing, repeat benzodiazepine injection in 3-5 min. If an
effect is absent, phenytoin should be infused at a dose of 15-18 mg/kg. An alternative to phenytoin is
valproate or phenobarbital. If seizures still recur or continue, the patient must be intubated, artificially
ventilated, and treated with thiopental or propofol.

Nonepileptic paroxysmal stales

Nonepileptic seizures are provoked by a temporary disorder or stress. It can be metabolic disorders
(hypocalcemia, hypoglycemia, hyponatremia, hyperglycemia, Hypomagnesemia, hypernatremja),
cardiovascular disorders (syncope), brain hypoxia, drug toxicity (camphor, cocaine and other CNS
stimulants, cyclosporine) ot drug withdrawal syndromes (alcohol, anesthetics). In children fever can
provoke seizures.
Febrile seizures occur, by definition, with fever and in the absence of intracranial infection. They
affect about 4 % of children aged 3 months to 5 years. Benign febrile seizures are brief, solitary, and
generalized tonic-clonic in appearance. Complicated febrile
15. Epilepsy. seizures
Noneplleptic paroxysmal are
statesfocal,
| 111 last > 15 min, or recur s 2
times in < 24 h. Overall, 2 % of patients with febrile seizures develop a subsequent seizure disorder.
However, incidence of seizure disorders and risk of recurrent febrile seizures are much greater among
children with complicated febrile seizures, preexisting neurologic abnormalities, onset before the age of
1 year, or a family history of seizure disorders.
Syncope should be distinguished from seizures. Hypotension, hypovolemia, cardiac arrhythmia, a
sudden increase of the vagal tone, and sometimes an extreme startling can cause syncope. The
commonest cause of syncope is prolonged standing, especially in the heat. Syncope is usually preceded
by a sense of light dizziness or weakness. The fall from syncope is a swooning collapse. The patient with
epileptic convulsions is usually rigid and falls swift. After the syncopal episode perfusion is usually
restored but the patient may feel himself weak for minutes. After epileptic seizure, by contrast, there is a
post-ictal period of stupor lasting from several minutes to nearly an hour. High blood pressure is
common with epileptic convulsions, low — with syncope. Biting ai\d injury of the tongue or lips is
common with a epilepsy, rare in syncope. A patient with a full bladder may urinate during either kind of
spell, but it is a bit more common with generalized epileptic seizure.

Tests and typical tasks

1. Indicate the kinds of generalized seizures.
a) motor Jackson's, sensor Jackson's seizure
b) adversive, automatisms
c) absences, tonic-clonic seizure
d) seizure with psychic symptoms, automatisms
e) autonomic, Kozhevnikov's epilepsy
2. Where Is the epileptic lesion in a seizure with motor aura?
a) In the postcentral gyrus
b) In the'precentral gyrus
c) in the parietal lobe
d) In the occipital lobe
e) in the temporal lobe

3. What symptoms are common for all primary generalized seizures?

a) visual hallucinations
b) automatisms
c) sensory symptoms
d) motor seizures
e) loss of consciousness

4. What additional diagnostic method can prove the diagnosis of epilepsy?

a) electroencephalography
b) echoencephaloscopy
c) radioisotope method
d) pneumoencephalography
e) myelography

5. Prescribere medicine for treatment of status epilepticus.

a) ethosuximide
b) carbamazepine
c) levetiracetam
d) diazepam
e) lamotrigine

6. Apatient is developing simple visual hallucinations (photopsy). Then he becomes unconscious and
there appears a generalized seizure with tonic-clonic convulsions lasting for 3 minutes. Where is the
epileptic focus localized? Name the seizure type.

7. A patient being conscious develops clonic convulsions in his left foot which radiate into left arm and
left half of the 112
face. The seizure lasts for 2 minutes. Where is the epileptic focus localized? Name the
I PAR r 2. Special Neurology
seizure type.
8. A 29-years-old woman presents with stereotyped sensation disorders that have been occurring for 3
yeai s. She complains of strange feeling that everything is very familiar (cjerealisation feeling "deja
vu"). This fits have a sudden onset and lasting only a few minutes. What is the name of such
disturbances?
9. A 40-years-old woman presents with multiple tonic-clonic convulsions lasting for 50 minutes, there is
no recovery of consciousness between them. What diagnosis should be written?
10. A 10-years-old patient has short sudden periods of a loss of consciousness without convulsions
lasting only a few seconds with a blank stare, brief upward rotation of the eyes and an interruption of
ongoing activity. This fits occur many times a day. The patient has no memory of these seizures.
Neurologic and cognitive examination results are normal. On EEG during the seizure three per
second spike- and-wave discharges are generalized. What diagnosis should be written?

15. Epilepsy. Noneplleptic paroxysmal states | 113

 16. DISEASES OF THE PERIPHERAL NERVOUS SYSTEM. VERTEBROGENIC
AFFECTIONS OF THE PERIPHERAL NERVOUS SYSTEM. MONONEUROPATHIES AND
POLYNEUROPATHIES

Peripheral nervous system (PNS) diseases amount about 50 % of all adult diseases and take the first
place in the prevalence. The PNS includes the spinal ganglions, anterior and posterior roots, mixed
spinal nerves, that are formed by root confluense and plexuses (cervical, brachial, lumbal and sacral).
Peripheral nerves are formed from the plexuses, which contain motor, sensory and vegetative fibers.
Cranial nerves are also a part of PNS, but not all of them are mixed.
Classification. Depending on the location of the lesion of the peripheral nervous system diseases are
divided into: neuropathy — damage of a nerve; neuralgia, polyneuropathy — multiple lesions o'f the
nerves; radiculopathy — spinal roots damage; plexitis — plexus lesion; ganglionitis — lesion of gan-
glions.
Etiology. Etiological factors of PNS lesions are various. They are: osteochondrosis, maldevelopments,
infections, intoxications, traumas, metabolic disorders, genetic predisposition. In most cases the disease
occurs when the combined effects of several adverse factors are presented. Mononeuropathy often
occurs when there is a compression of the nerve by fibrous or osteal- muscular formations. Then it is
called a tunnel neuropathy.
Pathogenesis. At bottom of the most neuropathies are ischemic, toxic, metabolic,
compression-ischemic disoders. Multiple lesions of the roots and nerves are often due to
infectious-allergic, toxic, discirculatory, dysmetabolic mechanisms. The main role in the development
of radiculopathy belongs directly to the compression mechanical influence of the intervertebral disk
hernia.
Pathomorphology. There are demyelinating and axonal damages of the peripheral nervous system
structures. In the first type a periaxonal process or segmental demyelinization develops. Axial cylinder
remains intact for a long time. In the second one there is an axonal degeneration. When regeneration
occurs, continual growth of nerve fibers from the proximal part of the axial cylinder takes place in the
PNS with the speed to 0.5-1 mm per clay.
Vertebral lesions of the peripheral nervous system
Among the lesipns of the spine, which cause the development of neurological syndromes,
osteochondrosis mostly occurs. Osteochondrosis is a progressive, degenerative lesion of the
intervertebral disc with reactive changes in adjacent vertebral bodies. Degenerative process
predominates in the lumbar or cervical part of the spine, less frequently in the thoracic part, but often it
is diffuse.
There are reflex and compressive (radicular and radicular-vascular) syndromes in the clinical
presentation. The early stages of the osteochondrosis are characterized by reflex syndromes. They are
manifested by muscular- tonic, vegetative-vascular and neurodystrophic disorders. Reflex syndromes
are caused by the irritation of the pain receptors of the fibrous ring and capsules, intervertebral joints.
Nociceptive impulses activation in the posterior horn leads to a reflex tension of the muscles by
increasing of the anterior horn activity. A reflex muscular tonic syndrome takes place. Impulse diffusion
to the lateral horn causes reflex vasomotor or vegetative-trophic disorders. Pain is one of the major
clinical manifestations of spinal neurological lesion.
Compressive syndromes consist of discogenic radicular and radicular-vascular syndromes. They
appear as a result of the compression of the spinal root and its vessels, hernial evaginations (Fig. 16.1).
Vertebral lesions can lead to the development of the neurological syndromes: radiculopathy —
compression of the spinal root; radiculoischemia — compression of the spinal root and its vessels as
114 І РАПТ 2: Special Neurology
well; myelopathy — vascular compression of the spinal cord. A combined lesion of the roots and the
spinal cord — myeloradiculopathy may occur.
Cervical vertebral syndromes usually do not appear after the influence of the intervertebral discs
hernias, that are rare in the cervical region.They are caused by the changes in the bone structure
(osteophytes) and vertebral discs arthrosis. The most commonly the lower cervical vertebrae are
affected, which have the highest mobility. Vertebral arteries and sympathetic trunk are located near the
osteo-articular apparatus in the cervical region.
Reflex syndromes
Cervicalgia is a pain in the cervical part of the spine, constant or paroxysmal, often caused by
osteochondrosis of the lower cervical vertebrae.

Often the pain spreads to the occipital region and the shoulder girdle and increaces during moving or
turning a head. During deep palpation tenderness of the neck muscles paravertebraly and pain of the
cervical spinous processes are determined.
Cervicocranialgia is a pain in the neck, irradiating to the head and is accompanied by vasomotor and
degenerative disorders. There is headache, co- chleovestibulary, visual and acoustic disturbances.
Vertigo increases during sudden head movements or its rotation. Sometimes these disorders can be
combined with acoustic impairment and drop-attacks.
Cervicobrachialgia — is a reflex syndrome, that is accompanied by constant pain, not only in the
neck, but also in the proximal upper extremity and shoulder girdle. Musculotonic, neurodystrophic and
neurovascular disorders of the upper extremities develop. The most common are: scapulohumeral peri-
arthrosis, shoulder-wrist syndrome, shoulder epicondylosis.
Cervical radiculopathy (radiculitis). The main symptom is pain that rises suddenly after an
awkward movement or exercise that is called lumbago. The pain increases by movements of the head,
irradiating to the shoulder girdle, arm, suprascapular region. The position of the head may be compelled,
the head is bent to the affected side. The most often affected roots are C6-C7.
(Figure segmental innervation). In the area of innervation of the affected roots hypoesthesia or
anesthesia, muscle hypotonia of the upper extremity and shoulder girdle are revealed. Tendon and
periosteal reflexes might be decreased. Vegetative-vascular disorders may occur.

Thoracic vertebral syndromes

Reflex syndromes in the thoracic part of the spine are called thoracalgia. This is the constant pain in
the thorax, that increases from time to time during sharp movements, coughing and deep inspiration.
Vertebrogenic thoracalgia occurs comparatively rare, because the thoracic disc hernias are not wide-
spread. Pain in the thorax with degenerative changes of the spine is most often caused by the lesions of
costovertebral and costolumbar joints and their capsules.
Thoracic radiculopathy is relatively rare. Neurological symptoms are usually determined in the area
of the affected spinal root innervation. The constant symptom is a pain. Sensory disoders are manifested
16. Diseases of 11 іе peripheral nervous system. Vertebrogenlc affections of the peripheral nervous system.... | 115
by segmental anesthesia or, more frequently, hypesthesia.

Lumhar-sacral vertebral syndromes

Reflex syndromes
In clinical practice, the most common are lumbago and lumbalgia.
 Lumbago is an acute lumbar pain, which appears suddenly during exercise or any other movement.
The pain in the lumbar muscles is retained for a few minutes or hours. The pain is very sharp, binds and
does not allow doing even a slight movement in the lumbar region. If an attack occurs during lifting a
weight, the patient can not straighten up. There is a marked limitation of the motrons in the lumbar,
moderate tension and muscle pain, flattening of the lumbar lordosis and kyphosis, sometimes with
scoliosis. Lumbago lasts for a few days and then decrease.
Lumbalgia is a subacute or chronic lumbar pain that occurs gradually after physical activity,
prolonged staying in an uncomfortable position, hypothermia. Pain in the lumbar muscles has an aching,
dull character and enhances by changing the position of the body, an extended position while sitting or
walking. The lumbar part of the spine, as in lumbago, can be deformed, but has lesser extention. There is
a flattening of the lumbar lordosis, limitation of the movements in the spine, slight pain of paravertebral
points in the lumbar region. The sensitivity and reflexes are not violated.
Lumboischialgia is a pain in the lumbar region, which extends to the thigh, lower limb, and
sometimes — on both lower limbs. The pain never descents down on the foot and doesn't reach the toes
— that is characteristic feature of the radicular lesions. Often the pain in the lumbar region is enhanced
with the minimal movement of the trunk by sneezing, coughing, prolonged staying in the sitting or
standing positions. Often it is combined with numbness of the lower limb, especially foot, feeling hot or
chill in it. Movements of the lumbar part of the spine can be greatly limited by bending. There is a
muscle tension, changes in spine configuration (an increased of the lumbar lordosis or scoliosis).
Neuroosteofibrosis segments are determined in the area of lumbar region and lower limbs, which
manifests itself by different syndromes: a piriform muscle, coxal periarthritis and knee joint'
periarthritis.

Compressive syndromes
These syndromes include radicular and radicular-vascular syndromes. In contrast to the reflex
symptoms, the compressive symptoms have a loss of roots' function.
Radicular syndrome is a discogenic lumbosacral radiculopathy (radiculitis). The lesion of roots in
this level is clinically manifested by sensitive (pain, paresthesia, and anesthesia), motor (paresis of
certain muscle groups) disorders, a decrease or loss of tendon reflexes. There are also muscle-tonic,
vegetative-vascular and neurodystrophic disoders.
Clinical manifestations of the radicular syndrome depend on the localization of intervertebral disc
hernias. Most of them are observed at the level of LIV- LV and LV-SI intervertebral discs, which is
associated with the greatest, load on this part of the human spine. So, often L5 and S1 roots are
compressed. The main clinical syndromes of L5 radix lesion are pain and numbness in the upper part of
the thigh, irradiating to the lateral surface of the hip, the anterior surface of the tibia and foot into the
thumb. There are weakness and hypotrophy of the thumbs extensors, hypoesthesia in this radix
innervation area. The knees and Achilles reflexes are not changed.
When there is S1 root lesion syndrome, the most common complaint is pain in the gluteal region,
extending to the posterior surface of the thigh, tibia, lateral surface of the toot, irradiating to the heel, the
little toe. The muscle tone of the thigh, the back of the hip and tibia is decreased. There is feeling of
weakness in the thumbs flexors, sometimes in the foot. Achilles reflex is often decreased or disappeared.
There is a slight hypalgesia in the area of S1 root innervation. In the acute phase the pain during
palpation of the paravertebral points and the spinous processes of the lumbar part of the spine is
determined. Also there are symptoms of tension (Lasegtie, Bekhterev, Neri, Dejerine, Sikkar, planting,
etc.).
Lasegue Symptom. The pain appears or increases in the lumbar region and along the sciatic nerve of
a patient lying on his back during lifting a straight leg. The pain disappears or reduces during bending
the leg in the knee joint.
Neri Symptom is116the pain increasing in the lumbar region during passive flexion of the neck (bringing
І РАПТ 2: Special Neurology
the chin to the chest) in a patient lying on his back.
Dejerine Symptom is the pain increasing in the lumbar region during coughing and sneezing.
The diagnosis of the cervical reflex syndromes, cervical radiculopathy are established on the basis of
clinical manifestations of the disease, and X-ray results. Pain syndrome in the thorax can be caused by
different factors: tuberculous spondylitis, a spinal cord tumor, ankylosing spondylitis (Bekhterev
disease), tumors of the mediastinum, esophagus, stomach ulcer, diseases of the pancreas, kidneys,
lungs, and pleura. Only after detailed examination of the patient and exclusion of these diseases, a
vertebral thoracic radiculopathy (radiculitis) can be diagnosed. Pain in the lumbosacral region may also
occur in various diseases, which should be excluded. First of all among them there are tumors,
inflammatory processes of the spinal column and pelvis, spinal arachnoiditis, tuberculous spondylitis.
Additional diagnostic techniques are study of cerebrospinal fluid, spinal X-rays, CT scans, MRI of the
spinal cord.
Treatment. In the acute phase staying in bed and the rest is required. A patient should be placed on a
firm bed, orthopedic mattress. Variety of anesthetic ointments, which are rubbed into the painful areas,
is required. Also non-steroidal anti-inflammatory drugs are used: diclofenac sodium — 3 ml, ksefokam
(8 my) — 2 ml intramuscular, meloxicam — 7.5 mg twice a day in tablets or 1.5 ml intramuscularly, and
also fintepsin — 200 mg.
To reduce the swelling of the radix, dehydration substances are used: fu- rosemide — 40 mg,
hypothiazide — 25 mg per day for 3-4 days, aminophyl- )ins —10 ml of 2.4 % solution intravenously.
When there are reflex muscular- tonic syndromes, midokalm — 50 mg, sirdalud — 2-4 mg three times
a day should be used. Vitamins of group B, biogenic stimulators subcutaneously for 10-15 days are
prescribed in the chronic recurrent course of the disease.
Among methods of physiotherapy electrophoresis of novocaine, magneto- and diadynamic, massage,
exercise therapy is used. After the elimination of acute symptoms, orthopedic treatment — stretching of
the spine — is applied.
In the chronic phase of disease treatment in a sanatorium, mud packs, radon baths, paraffin-ozokerite
applications are recommended. For persistent pain and severe motor disorders surgical treatment is
used.
Prophylaxis. Physical education and sport is very important against hypokinesia. It is necessary to
avoid hypothermia, sudden movements during work connected with a significant load on the spine and
spinal roots tension, lifting heavyweight.

Herpes zoster
Herpes zoster is characterized by the appearance of the vesicles on the skin located along the affected
dermatome in the form of a chain in the region of segmental innervation. This disease is caused by the
herpes virus Varicella zoster, which is similar by its antigenic structure to the causative agent of the
chicken pox. It affects the sensitive ganglions of spinal or cranial nerves and skin of the appropriate
dermatome, where the vesicular rash appears. Inflammation may also be spread to the appropriate
posterior spinal radices, the meninges, sometimes to the brain substance. The most common herpes rush
appears on the trunk, in the area of the thoracic ganglions and roots innervation. Often the rash arises on
the face, in the zone of the trigeminal nerve innervation, especially its upper branch (Fig. 16.2).
Clinical signs. The disease begins with the appearance of the general infectious symptoms. Suddenly
the patient feels general malaise, headache, and fever. After 2-3 days sharp pain, paresthesia or itching,
redness of the skin with the formation of papules arranged In groups appears in certain dermatome.
Sensitivity can be affected by segmental type. Anesthesia or hypoesthe- sia may also appear. In 2-3
days papules turn into vesicles, filled with serous fluid. In a few days vesicles become dry and form
yellow-brown crusts, which fall off at the end of the third week of the illness. In the region of the rash
unstable pigmentation is left on the skin. In some cases, especially in elderly patients, neuralgic pain in
the affected segment may persist for several months (postherpetic neuralgia). When there is a rash on
the face in the area of the first branch of the trigeminal nerve innervation and the cornea such complica-
tions as iritis, ulcerative keratitis and blindness are possible.
Treatment should be etiopathogenetic. Medicines acting on the nuclear structure of the virus
include: Valacyclovir (Valtrex) —1000 mg 3 times a day for 7 days, Acyclovir (Viroleks, Zovirax) —
800 mg 5 times a day orally for 7-10 days. From antiherpetic drugs are also used metisazon — 0.2 g 3-4
times a day, bromnaftohinon — 0.1 g 2-3 times a day. Interferon is used intranasally, in the conjunctival
sac; it enhances the production of the antiviral protein, which enhances the body's resistance to the
viruses. 16.Gamma
Diseases of 11 globulin —system.
іе peripheral nervous 3 ml Vertebrogenlc affections of the peripheral
intramuscularly at nervous system.... of
intervals | 117 2-3 days (three infusions),

immunoglobulin against the virus Varicella Zoster are also used for treatment. Broad-spectrum antibiot-
ics, nonsteroidal anti-inflammatory drugs (indomethacin or voltaren — 0.025 g 3 times a day, movalis
— 7.5 mg two times a day) can be also applied. In severe disease types hormonal therapy is needed
(hydrocortisone, prednisolone).
Brachial plexitis

In the etiology of the brachial plexus lesions various factors have a great importance: plexus trauma
after clavicle fracture, dislocation of the brachial joint, injury, compression of the plexus between the
clavicle and the 1st rib or the brachial caput that can emerge after operation using inhalation anesthesia
with target the hands fixed under the head. Sometimes the brachial plexus is compressed by the cervical
ribs, calluses after clavicle fr-actures, contraction of the scalenus muscles (skalenus syndrome).
Clinical signs. All the plexus is affected rarely, more common is the damage to its superior or inferior
trunk. Therefore, there are superior, inferior and total syndromes of brachial plexus lesions.
Superior Uuchenne — Erl) paralysis occurs when there is the primary stem lesion of the upper
trunk of brachial plexus, formed by spinal nerves of the C5-C6 segments. By its affection the function of
the proximal arm is lost only witli the atrophy of the proximal muscles group (Fig. 16.3). The patient can
not raise his hand in the brachial joint, abduct it from the body and bend it at the elbow joint. The biceps
refiex is absent and carporadial reflex is de- creaseed. Sensory disoders are observed 011 the lateral
surface of the upper arm and forearm. The pain occurs after pressing on the supraclavicular fossa.
Inferior Dejerine-Klumpke paralysis occurs in the lesions of the inferior trunk of the brachial plexus,
whose fibers exit from the C8-T1 segments and provide innervation to the distal part of the hand.
Movements of wrists and fingers are disturbed. There is muscle atrophy in the hand, the absence of the
triceps retlex and sensitivity disorders on the medial surface of the forearm, in the region of the hand and
fingers. Pain by pressing the plexus in the infraclavicular fossa, Bernard — Horner syndrome on the
affected side is observed.

Fig. 16.3. Duchenne — Erb paralysis

Total brachial plexitis is caused by the total brachial plexus lesion and is accompanied by peripheral
paralysis and sensitivity disorders of the upper extremity.

Multiple lesions of spinal roots and nerves

Acute demyelinating polyradiculoneuropathy of Guillain — Barre
Biology and pathogenesis. Allergic reactions and autoimmune shift play a leading role in the
disease's development. Variety of infections, intoxications, endocrine and metabolic disorders, cooling
may precede the disease. In some patients antibodies to myelin of peripheral nerves are determined.
Clinical presentation. The disease starts from general weakness, a slight increase of body
temperature. Paresthesia (numbness, tingling) in fingers and toes, pain in the extremities along the
peripheral nerves may also appear. Later weakness in the lower extremities appears which than spreads
to the upper extremities and progresses to paralysis. Motor disorders include both distal and proximal
parts of the extremities. Tendon reflexes are decreased or can be absent, muscle atrophy is developed.
118 І РАПТ 2: Special Neurology
The cranial nerves are often affected, especially the facial, bulbar nerves and rarely oculomotor.
Sensitive disorders are slightly manifested — mild hypalgesia of "gloves and sock" type. Nerve trunks
are sensitive when pressed; the tension symptoms (Lasegue, Neri etc) may appear. In the cerebrospinal
fluid protein-cell dissociation is determined, which remains during 2-3 months.
The disease progresses during 2-4 weeks, after which comes relatively steady period, and then
improvement and regression of neurological deficit begins. First of all sensitivity improves, later —
motor functions, and finally reflex disorders disappear. Full recovery in a mild form can occur in 2-3
months. In most cases disease has a favorable course with recovery of function, sometimes persistent
motor disorders may remain. Clinical forms of disease have an especially unfavorable course, which
leads to the ascending type of Landry paralysis with the spread of flaccid paralysis to the muscles of the
body, extremities, bulbar group of muscle. Even using modern resuscitation measure the severest
disease may have fatal outcome.
Diagnosis of Guillain — Ваггё poliradiculoneuropathy is established on the basis of acute onset of
the disease with the development of flaccid tetraparesis of both distal and proximal part of the
extremities with involvements of cranial nerves, relatively mild sensitive disorders and typical
protein-cells dissociation in the cerebrospinal fluid.
Treatment. Urgent hospitalization of patients to the neurological department is required; when vital
functions are disturbed — to the intensive care unit. If the patient has acute respiratory insufficiency,
tracheal intubation should be performed, and the patient should be transferred to APV. Basic methods of
treatment are program plasmapheresis and'intravenous immunoglobulin class G pulse-therapy (0.4 g/kg
per day) daily during 5 days. Antihistamines, vitamins of group B, neuromidin, prozerin are also used.
In the recovery period, massage, remedial gymnastics, balneotherapy, mud and paraffin therapy,
electrical stimulation of the muscles, acupuncture are prescribed. The use of corticosteroids is
ineffective.

Polyneuritis, polyneuropathy
Polyneuritis is the multiple lesions of peripheral nerves of infectious or infectious-allergic etiology.
Multiple lesions of peripheral nerves of non inflammatory, but dystrophic nature are indicated by the
term "polyneuropathy". But even when polyneuritis is caused by infection agents, lesions of the main
nerve trunks have often non inflammatory but neuroallergic nature. Therefore in clinical practice a term
"polyneuropathy" is often used.
In clinical presentation we may determine bilaterally symmetrical dysfunction of motor, sensitive and
vegetative functions in the distal part of extremities: peripheral paresis of upper and lower extremities,
disturbance of all sensitive types of "gloves and socks" type, pain along the nerve trunks, and
vegeto-trophic disorders in form of cyanosis, hypertrichosis of the skin of the hands and feet, brittle of
nails, hair loss (Fig. 16.4).
The main causes in the development of polyneuropathy are exogenic and endogenic intoxications.
Exogenic inti oxications include alcoholic intoxications, intoxication of organophosphorus compounds,
lead, mercury, arsenic, thallium etc.

Fig. 16.4. Distal localization of motor, sensitive and vegetative

dysfunctions in patient with polyneuropathy

16. Diseases of 11 іе peripheral nervous system. Vertebrogenlc affections of the peripheral nervous system.... | 119

Endogenic intoxications develop in patients with diabetes mellitus, uremia, diffuse connective tissue
diseases and vasculitis, malignant neoplasms, Polyneuropathy occur in patients with infection diseases
(diphtheria, leprosy, borreliosis, HIV infections), and also there are genetic forms of polyneuropathy.
Alcoholic polyneuropathy. The disease appears in patients with alcoholism and may be combined
with injuries of internal organs, first of all liver and stomach. As a result, vitamin malabsorption in the
digestive tract leads to their deficit, especially of thiamine. Its insufficiency leads to accumulation of
lactic and pyruvic acids in the tissue, which leads to destruction of the myelin and degeneration of
axons.
Clinical presentation. Alcoholic polyneuropathy often develops subacute. The patient usually
suffers from paresthesia, burning pain in the distal part of extremities, pain by palpation of nerve trunks,
gastrocnemius muscle by its pressing. Father paresis of extremities with hypotension and atrophy of the
paralyzed muscle is developed. Often functions of feet and toes extensors are fallen out, gait of steppage
type appears. There may also appear hypesthesia, anesthesia of the polyneuritic type, sometimes
combined with paresthesia. Disorders of proprioceptive sensitivity lead to the development of sensitive
ataxia. Vasomotor, trophic and secretory disorders are detected very often: hyper hidrosis, swelling of
the distal part of the extremities, cyanosis and change in the skin temperature.
A characteristic feature of alcoholic polyneuropathy is its combination with amnestic syndrome,
described by S. S. Korsakov (Korsakov's syndrome). It manifests itself as a loss of memory 011 current
events, confabulation, disorientation in space and time. Regression of pathological symptoms may
occur in the case of a full rejection of .alcoholic drinks and diligent treatment.
Treatment. В group vitamin, thiamin — 5—10 ml 5 % solution intramus- culary, ascorbic and
nicotinic acid, anabolic steroids are prescribed. In the recovery period biostimulants (aloe, body
vitreous, plazmol), anticholinesterase agents (galantamine, prozerin, neuromidin), physical therapy are
used.
Diabetic polyneuropathy appears in almost 90 % of patients with diabetes mellitus. As a result of
present deficit of insulin, utilization of glucose by peripheral nerves is impaired.
Clinical presentation. In some cases polyneuropathy develops early, flows subclinically and may
exist for many years. It is clinically manifested by reduced vibration sensitivity, a loss of knee and
Achilles reflexes, it is not accompanied by pain.
The second version of the diabetic polyneuropathy is characterized by the acute or subacute
development of the damage to individual nerve trunks. The damage to the femoral, sciatic, ulnar or
median nerves, which is accompanied by paresis of the muscle groups, pain, sensitive disorders takes
place more often. There is also damage to cranial nerves: oculomotor, trigeminal, abducent.
The third version of diabetic polyneuropathy is manifested by multiple lesions of peripheral nerves of
the extremities with the development of motor and sensitive disorders, mostly in the legs. Often there
are no tendon reflexes. Nerve trunks are painful on palpation. Trophic disorders are often observed.
Treatment. The correction of carbohydrate metabolism is provided with diet, hypoglycemic drugs.
Analgetics, gauglioblocators, В group vitamins, nicotic acid, espalipon (berlition), and finlepsin are
used in the treatment of this kind of neuropathy.
Lesions of individual spinal nerves
Peripheral spinal nerves are mixed: they include motor, sensitive and vegetative fibres. Therefore
pathology of nerves causes disorders of these three functions. We can observe paresis or paralysis of the
muscles with their atrophy and hypotension, a loss of sensitivity in the autonomic region of the
mononeurotic peripheric type, also vegeteto-trophic disorders. The causes of nerves' lesions often are
trauma, hurts, compression of the nerve by surrounding tissues — tunnel mononeuropathy (injuries to
peripheral nerves in anatomic osteo-fibrosis and fibro-muscular canals, aponeurotic rima and introits in
ligaments). Permanent microtrauma — professional, sports, domestic, by medical manipulation
promotes the development of mononeuropathy canals.

Mononeuropathy of the upper extremities

Neuropathy of the radial nerve (Fig. 16.5) may appear in different injuries: in fractures of humerus,
compression of the nerve during surgery or using a crutch, during a sleep, especially the "alcoholic".
Radial nerve may be affected by prolonged lead poisoning. There may appear typical picture of hanging
120 І РАПТ 2: Special Neurology
brush as a result of paresis or paralysis of the extensors of the hand, main phalanges of 11—V fingers
and final phalanx of the thumb. Adduction of the thumb is disturbed and leads to the suppination of the
hand and forearm. As a result of high radial nerve lesion paresis of triceps shoulder muscles appears,
Fig. 16.5. Neuropathy of the radial nerve: hand drop.
The sensitive disorder zone is shown darker

16. Diseases of 11 іе peripheral nervous system. Vertebrogenlc affections of the peripheral nervous system.... | 121
triceps reflex is fallen out, the patient is not able to straighten the arm at the elbow. Sensitive disorders in
the autonomic region: the area of the dorsum of the finger I and the area between I and II metacarpal
bones. Pain and autonomic disorders are atypical.
Neuropathy of the ulnar nerve (Fig. 16.6) is caused by a fracture of the condyle and the medial
epicondyle of the humerus, compression of the nerve in the cubital canal. Affection of the ulnar nerve is
characterized by weakness of the hand and muscles flexors, which adductor it to the ulnar side, the
flexor end-phalanges of the IV and V fingers. Hand gets typical look of the "claw hand" fingers in the
proximal part of the phalanx sharply straighten, but in the middle and final ones are bent. Atrophy of the
small intercostals muscles and hypothenar is appeared. The patient can not unite all fingers and fan out
II, III, IV and V. In the area of innervations of ulnar nerve disorders of all sensitive types, vasomotor and
trophic disorders may be observed.

Fig. 16.6. Neuropathy of the ulnar nerve: clawhand. The sensitive

disorder zone is shown darker

Neuropathy of the median nerve (Fig. 16.7.). Compression of

the nerve may be observed in the metacarpal canal in people of
individual professions (porter, milkmaid, bricklayer). Numbness of
l-lll fingers appears, rarely — of all fingers of the hand, intensifies at night, in the horizontal position of
the patient, also when the upper extremity is raised By palpation or percussion of the transverse
ligament of the affected upper extremities paresthesias in fingers are intensified (Tinel sign). Pronation
of the hand, its flexion and also flexion of I, II and partly III fingers, extension of the middle phalanges
of the

122 І РАПТ 2: Special Neurology

 Fig. 16.7. Neuropathy of median nerve: monkey hand. Sensitive
disorder zone is shown darker

II and III fingers are disturbed or just impossible. As a result atrophy of

the thenar of I finger is assigned in one surface with the II finger, and
the hand acquires the form as a "monkey hand". Sensitive disorders are
detected in the innervations zone: on the radial part of the palm, on the
palmar surface of the IHII fingers and half of the IV finger. For large
quantities of sympathetic fibres composed of the median nerve when it is affected brute trophic,
secretory and vasomotor disturbances, hyperpathia appear, and often may lead to causalgia.
Lower extremity mononeuropathy. Neuropathy of the femoral nerve may be caused by fractures of
femoral bones, inflammation in the pelvic area, diabetes mellitus. The patient is unable to stand. Leg
extension in the knee becomes impossible, knee-jerk falls out. Sensitive disorders are observed on the
median surface of the tibia and anterior surface of femur. It may be a positive Wasserman's tension
symptom.
Neuropathy ot sciatic nerve is observed in injury, hip fracture, trauma of the nerve. Paralysis of the
foot and fingers appears, Achilles reflex is fallen out. It is difficult to bend legs in a knee and ankle
joints, and it leads to disturbance of walking. On palpation pain along nerve in points Valle appears,
Lassegue's symptom may be positive. The sensitivity on the posterior surface of hip, posrerolateral
surface of the tibia, on the foot and fingers is disturbed. Pronounced vegeto-trophic disorders appear. By
a significant damage to the nerve pronounced pain, causalgia usually often appears.
Neuropathy of the peroneal nerve (Fig. 16.8) can also be of traumatic or compression-ischemic
origin. Impingement of the peroneal nerve is occurred

16. Diseases of 11 іе peripheral nervous system. Vertebrogenlc affections of the peripheral nervous system.... | 123
 Fig. 16.8. Neuropathy of the peroneal nerve: a
dangling foot

between the fibula and the fibrous edge of the long fibular muscle during the work in the squatting
position. It is characterized by paresis of all muscles, extending to the foot and toes. It leads to a
dangling foot and the changing of a gait (steppage). The patient is unable to stand on the heel, but can
stay on his/her toes. The reflexes are stable. Sensitivity is damaged on the dorsum of the foot and the
outer surface of the tibia. Vegetato-trophic disorders are uncommon.
Neuropathy of the tibial nerve (Fig. 16.9) occurs in injury, nerve compres- ' sion in the osteo-fibrous
tarsal canal which is located behind and below the inner ankle. If the tibial nerve is damaged, paralysis
of the flexors of the foot and toes is developed, making plantar flexion of the foot and toes difficult or
impossible. Ankle reflex disappears. The patient is unable to stand on his/her toes, but can stand on the
heel. On the back surface of the leg and foot the sensitive disorders and autonomic-tropiiic significant
breaches take place. The main symptom of tarsal canal syndrome is a pain in the sole and toes that ap-
pears during the walk or at night.
Treatment. B.group vitamins, biostimulators, anticholinergic drugs are prescribed. Massage,
physiotherapy, physical therapy remedies, surgery treatment are applied.

Fig. 16.9. Neuropathy of the tibial nerve: pes

calcaneus

124 І РАПТ 2: Special Neurology

Neuralgia and neuropathy of cranial nerves. Neuralgia of trigeminal nerve
Etiology. Neuralgia of trigeminal nerve may be caused by the compression of the radix of trigeminal
nerve by aneurysm, voluminous process in the cerebellopontinus angulus, residual periods of
meningitis, inflammatory processes of undemasal additional sinuses or teeth, contraction of suborbital
or mandibular canals.
Clinical presentation. In the onset of darting, very intense pain is in the face region appears. Attack lasts
for several seconds to 1-2 minutes. On the height of paroxysm there may be twitching of mimic muscle
— painful tic. This pathology is characterized by the presence of a trigger zone — hypersensitive
region, little irritation of which (taking food, shaving and etc.) causes typical paroxysm (Fig. 16.10).
During palpation, pain in the place of exit points of affected radix is appeared. Symptoms of sensitive
disorders are absent.
Diagnosis of typical cases has no difficulties. The major task of diagnostic is to determine the cause of
trigeminal nerve's affection and treat it.
Treatment. Carbamazepine is prescribed in dose 600-800 mg for reduction of painful syndrome, in
resistant cases — other antispasm drugs (diphenine, depakine, lamotridgine). Tricycle antidepressants
are used (amytriptiline 25 mg 3 times a day), locally anaesthetics. Among physiotherapy methods one
can use.Novocaine phonophoresis, a low-frequency laser with the irradiation of each of trigger spot. For
removal of painful paroxysm, acupuncture is used.
Fig. 16.10. Trigger zones localization at the presence of trigeminal nerve neuralgia

16. Diseases of 11 іе peripheral nervous system. Vertebrogenlc affections of the peripheral nervous system.... | 125
Neuropathy of the facial nerve
It may appear because of local cooling, often in a combination with neuroviruses and common
infections. But often — it is canal syndrome, caused by nerve compression, its ischemia and swelling in
narrow bone canal of the facial nerve. As a secondary disease, neuropathy of the facial nerve may
develop from the injuries of the ear, tumor in the region of cerebellopontinus angulus, in fractures and
fissure in basic of skull, parotitis, etc.
The clinical presentation of the disease is characterized by the appearance of facial asymmetry —
peripheric paresis or paralysis of mimic muscles. On the affected side one can observe flattened or
absent skin folds on the forehead, enlarged palpebral fissure, flattened nasolabial folds, lower angulus
mouth (Fig. 16.11). This disorder is especially visible when you want to do active movement by the
mimic muscle. Eyebrow keeps down, doesn't make horizontal fold on the forehead. Eye doesn't close.

Fig. 16.11. Leftside neuropathy of facial nerve

When you try to close the eye, eye-bulb on the affected side passes up and out and you see white striae
of sclera (Bell's phenomenon). Lacrimation is defined on the affected side. On frowning, vertical folds
are formed on the relevant side. On the barring teeth, mouth pulls on healthy side. The patient cannot
inflate cheeks or whistle. During the meal food is stuck between cheek and teeth. Superciliary, corneal
and conjunctival reflexes are decreased or absent.
Depending on the affected level of nerve in region of its canal to described picture, additional
symptoms are affixed. Higher affection of the facial nerve to place origin of the fibres of major nervus
petrosus is characterized by lacrimation decrease, xerophthalmus on the affected side. In the case of the
affection in the area between major nervus petrosus and nervus stapedius disorders of taste combination
with higher sensitivity to sounds (hyperacusia) can be observed, lacrimation is possible. If the nerve is
affected lower stapedius, but higher than the origin of chorda thympani, disorder of taste on the anterior
2/3 tongue with lacrimation is observed, but without hyperacusia. Major facial neuropathy, when treated
adequately, ends with full recovery in 2-3 weeks, while in the severe course of disease recovery period
lasts more than 1-2 month. In some cases contracture of paralysis muscle may appear.
Diagnosis. It is nessesary to exclude affected nucleus of facial nerve, which may cause peripheral
paralysis of mimic face muscle, but accompanied by central hemiparesis on the opposite side
(alternating syndrome Millard — Gubler). This pathology develops in acute disturbance of posterior
circulation, brainstem encephalitis, tumors of the brainstem. Affection of facial nerve nucleus may be
caused by the pontine form of poliomyelitis. Pathology of the facial nerve in the case of the tumor of
angulus cerebellopontinus is accompanied by vestibule-cochlear, trigeminal and abducent nerves'
lesions.
Treatment. In acute period, anti-inflammatory therapy is prescribed. Corticosteroids — prednisolone
60 mg a day during1263-4 days
І РАПТ is taken
2: Special Neurology with next lowing of the dose. In the event of paresis of the mimic
muscle, mild hormones are not used. For recovery microcirculation in the trunk of the nerve pentoxifyl-
line (trental) is used 300 mg a day intravenous infusions for 10 days. Since day 5-7 of disease,
hymnastics for the mimic muscle, 2 week-point massage, acupuncture are prescribed.
Tests and typical tasks

1. Indicate the signs of the posterior root lesion

a) sharp pain, segmental anaesthesia
b) fascicular twitching, peripheral paresis of the extremities
c) high reflexes, central paresis of the extremities
d) dissociative anesthesia, the herpetic rash
e) conductive type of sensitive disorders

2. What are the clinical signs of the facial nerve neuropathy?

a) enophthalmos
b) central mimic muscles paresis
c) peripheral mimic muscles paresis
d) peripheral masticatory muscles paresis
e) normal supraorbital reflex

3. What are the characteristic symptoms of ulnar nerve neuropathy?

a) drop hand
b) absence of triceps reflex
c) hyperreflexia of triceps reflex
d) IV and V fingers flexion disturbances
e) l-lll fingers flexion disturbances

4. What are the main symptoms of polyneuropathies?

a) dissociative sensitive disorders of the hands and feet
b) central paresis of extremities
c) pathologic reflexes and conductive sensitive disorders
d) disorders of sphincters function
e) peripheral paresis and sensitive disorders of hands and feet

5. What are the symptoms of lumboischialgia?

a) muscle atrophy and areflexia
b) back pain and tension of back muscles
c) conductive anesthesia and paresthesia
d) peripheral paresis and sensitive disorders
e) central paresis of the extremities
6. After the shoulder trauma patient points to the
weakness of muscles-extensors of
the right hand and fingers, the hand droop. Determine the pathology. і
7. A patient complains of attacks of sharp pain of short duration in the left half of the upper lip and
upper jaw after overcooling. Pain spread over all half of the face. What is the name of such
disturbance?
8 After clavicle fracture patient has muscle weakness in the distal part of the left hand, hypotrophy of
the left hand muscles, pain in the hand. Define the pathology.
9, A patient has a headache and vesicular rash in the skin of the right part of the forehead. What is the
name of such disturbance?
10. A patient complains about acute pain in the cervical part of the spinal cord, that enhances during
the head movements. Muscles of the neck are tense, the head movements are limited, reflex and
sensitivity are normal. What pathology has supposed?
16. Diseases of 11 іе peripheral nervous system. Vertebrogenlc affections of the peripheral nervous system.... | 127
17. DEMYELINATING DISEASES OF THE NERVOUS SYSTEM. MULTIPLE SCLEROSIS.
ACUTE DISSEMINATEDENCEPIIALOMYELITIS. AMYOTROPHIC LATERAL
SCLEROSIS

Demyelinating diseases of the nervous system include disorders with de- myelination — myelin
destruction — as the primary characteristic feature of pathogenesis. Myelin provides insulation for
axons and is necessary for saltatory conduction. Central nervous system (CNS) myelin is produced
by oligodendrocytes. It is composed of tightly wrapped lipid bilayers with specialized protein
constituents. Myelin basic protein (MBP) is its main component and makes up 30 percent of CNS
myelin proteins. MBP is responsible for compaction at the major dense line (Fig. 17.1),

Fig. 17.1. Myelin membrane: A — the axon,

1 — the major dense line,
2 — the intraperiod line

The presence of myelin is essential to maintain conduction velocity. Its loss or damage can lead to
significantly slower conduction or conduction block.

Multiple sclerosis
Multiple sclerosis (MS) is an inflammatory disease that affects the myelin sheath of the CNS,
specifically, the brain and the spinal cord. MS is characterized by dissemination in space and in time. It
is relapsing or progressive disorder of CNS white matter, which is a major cause of disability in young
adults, Pathologically, it is characterized by:

► multifocal areas of demyelination

► a loss of oligodendrocytes
► astrogliosis (sclerotic plaques)
► relative preservation of axons
History. MS is now known to be a common malady even though it was first recognized as a distinct
clinicopathological entity around 150 years ago. A lack of clear medical reports before the early 1800s is
sometimes interpreted as the evidence that MS is a relatively new disease. However, there are some
earlier precise observation and description of MS. Saint Lidwina of Schiedam (1380-1433) developed a
relapsing neurological disorder at the age of 18 and may be the first case of clinically described MS.
Augustus d'Este, nephew of Queen Victoria and illegitimate grandson of George III, had MS in his
second decade of the 19lh century. History knows a lot of well-known people who suffered from MS.
Poet Heinrich Heine, Victorian writer Margaret Gatty, naturalist W. I\l. P. Barbellion (B. F. Cummings)
documented in their diaries growing progressively worsening up to the time of death.
Ch. P. Ollivier d'Angiers was the first to report MS clinical case in the medical literature in 1824.1
Shortly thereafter, Robert Carswell illustrated a case of what is now clearly recognizable as MS in his
atlas of anatomical pathology. At approximately the same time, in the 1830s, Jean Cruveilhier published
pathological and clinical descriptions of MS. E. F. Alfred Vulpian first suggested the rubric of "sclerose
en plaque" in 1866. Jean M. Charcot was primarily responsible for establishing MS as a unique and
recognizable syndrome, describing the clinical spectrum and the histological appearance.
 Epidemiology. MS is not a rare disease. It affects 2.5 millions worldwide and approximately 250.000
to 350.000 in the United States alone. There are more than 19.000 MS patients in Ukraine. MS is not
evenly distributed throughout the world. Racial or ethnic origin plays an important, but not exclusive,
role in determining susceptibility to the disease. Persons of northern European descent have a strong
predisposition for MS. Other races and ethnic populations are resistant to a different extent. MS is rare
in tropical areas, virtually unknown among black Africans but occurs in African-Americans at half the
rate of the whites, possibly due to racial admixture or environmental (actors.
MS prevalence per 100.000 inhabitants is very high in Shetland Islands (129.0), Olmsted County,
USA (122.0), some less but also high in Iceland (99.4), Sardinia (69.0), Seattle, USA (69.0), in Hobart,
Tasmania (68.0). The prevalence is less than 5 cases per 100.000 in tropical areas: Malta (4.0), Seoul,
South Korea (2.0), Okinawa, Japan (1.9), Hong Kong (0.8). Migration from high to low prevalence
before the age of 15 lowers the MS risk, whereas migration after this age does not affect the risk.
Migration from low to high prevalence areas increases the risk of MS, but the effect of age is less clear.
The disease is acquired before or at the time of puberty. Symptoms usually begin during young
adulthood, with the peak onset at the age 24. Approximately 0.3 percent of MS cases are diagnosed
before the age 15. Women are affected nearly twice as often as men.
Etiology. Most clinicians hold the opinion that MS is a multifactorial disease. It results in the
genetically susceptible subject of the activation of the immune system by different viral agents, thereby
initiating a pathogenetic cascade that eventually leads to destruction of the myelin sheath. Thus,
according to the contemporary conception three components which determine the etiology of MS are:
> Viral infection
> Inherited immunogenetic susceptibility
> Geographic factors (were described above).

Infection. Viral infection is the most plausible, because of the elevation of viral titers and long latent
period. Serum samples of MS patients may contain higher titers of antibodies to the various infectious
organisms: adenovirus. canine distemper virus, herpes simplex viruses (HSV). human herpes virus 6
(HHV6), influenza, measles and varicella zoster virus (VZV), Chlamydia pneumoniae, cytomegalovirus
(CMV), Epstein — Barr virus (EBV). The most valid candidates forth role of the trigger of
immunopathological changes in the presence of MS are EBV and HHV6. Virus may be involved in the
pathogenesis of MS in several ways: transient, recurrent or persistent CNS viral infection or one outside
the CNS.
Heredity. Epidemiological findings support a polygenic hereditary piedis- position to MS. A number
of candidate genes have been investigated. The only definitive genetic association in MS is with the
serologically defined human leukocyte antigen (HLA) DR15, DQ6. This is one of the DR2 haplotypes.
Other susceptibility genes likely contribute the T-cell receptor variable beta region, the IgG heavy-chain
variable region (especially the VH2-5 gene), the MBP coding gene, the CTLA-4 gene on chromosome
2q33, the interleukin-1 ra associated gene in concurrence with the HLA-DR15 haplotype.
Pathogenesis. The precise pathogenetic mechanism of MS remains controversial, but many
neurologists believe that alterations of the immune system are responsible.
Autoimmunity. Transient or persistent infection outside the CNS may activate autoreactive 1" cells
(T4 lymphocytes) by the formation of a three- molecule complex. Low levels of autoreactive T and В
cells persist even in normal individuals. Autoimmune disorders occur when the tolerance of these cells
toward their antigen is broken. Blood-brain barrier (BBB) leakage alone may break tolerance. As a
result of this alteration of the BBB, immunoactive lymphocytes penetrate into the brain parenchyma.
Moreover, an infection or injury may release CNS antigens into the periphery, where they may activate
corresponding autoreactive cells.
Once the immunoactive cells and substances have penetrated into the brain parenchyma, the
mechanism by which they injure the myelin sheath is various. After reactivation they secrete the
17. Demyelinating diseases of tlie nervous system. Multiple sclerosis,... | 129
cytokines which cause inflammation and edema in the brain. Next phase may be demyelination.
Besides, there are demonstrations of a T cells (CD8 cytotoxic cells) attacking the myelin sheath.
Myelin is destroyed and eventually replaced by a glial scar. It is like scars which gave MS its name.
Remyelination occurs even in the earliest lesions, but is generally relatively inefficient. Myelin
destruction releases a lot of its components, in particular MBP and others neuroproteins, which may
enter bloodstream via the permeable BBB and provoke the next circle of autoimmune reactions.
Pathology. As MS is a disease of the myelin sheath, lesions are found almost exclusively in the white
matter but, because myelinated fibers are present in gray masses, lesions may be seen in gray matter as
well. On gross sections of the brain, sclerous plaques appear as yellowish, slightly shrunken, areas that
are dense glial scars. MS lesions vary considerably in size, ranging from only a few millimeters to
plaques involving almost the entire centrum semi- ovale.
Certain parts ot the nervous system are preferentially involved in MS. At first, it is cerebral
hemispheres: the anterior angles of the lateral ventricles, the corpus callosum and other periventricular
areas are almost affected. Lesions of the white matter may extend into the cortex. Next typical
localization is the spinal cord, especially the cervical portion. The lateral and posterior funiculus is the
preferable location of the plaques. The brainstem and cerebellum are eventually affected in many cases.
And at last, optic nerves aye frequently involved.
The course of MS varies greatly from one individual to another, but two basic types of the course can
be identified: relapsing-remitting (RR) and chronic progressive. The first one occurs in 66-85 %
patients most common when onset is under the age 25. Well-defined relapses are separated by periods
of a nearly complete recovery with or without residual symptoms and without progress during
remission. Chronic progressive type can be divided into three subtypes: primary, secondary
progressive and progressively remitting-relaps- ing MS. Primary progressive (PP) MS is found in 5-15
% of patients, generally when disease onset is over the age 40 and with progressive worsening from the
disease onset. Secondaiy progressive (SP) MS is seen in over 50 % of cases 6-10 years after onset:
initially remitting-relapsing, later disease looks like chronically progressive with recurrences, mild
remissions, and plateau phases may occur. The progressively remitting-relapsing subtype happens
rarely, a complete remission may or may not occur after relapses, symptoms tend to worsening from
one relapse to the next.
Definitions of stages. A relapse is the appearance of a new neurological disturbance, or the
reappearance of one previously present, lasting at least 24 hours. All such disturbances arising within a
one-month period are counted as a single relapse. The relapse rale is the number of relapses per year.
Clear improvement of neurological function is termed remission.
Clinical picture. MS is characterized by multiple symptoms and signs of brain and spinal cord
dysfunction which reflect the dysfunction of the particular areas of the nervous system involved and are
not specific for this disease. Typical MS manifestations include paralysis, paresthesiae, optic neuritis
(retrobulbar neuritis), ataxia, diplopia, and bladder dysfunction.
Pyramidal tract lesion results in central hemi-, para-, tetrapai esises which a t e common for MS and
cause weakness, spasticity and hyperreflexia. Involvement is often asymmetrical and mainly of the
legs, especially in the early stage of the disease. Spasticity makes its first appearance in the form of
extensor spasms; flexor spasms develop later. The latter are often painful, cause frequent falls, and in
severe cases can cause flexion contractures. Exercises or heat frequently worsen deficits. Many patients
complain of abnormal fatigue.
Sensory manifestations. Episodic or continuous paresthesias are common, particularly in the early
stage of the disease. Diminished vibration and proprioceptive sensation in the distal extremities can be
find. As the disease progresses, such positive phenomena usually recede and are replaced by sensory
deficits affecting all sensory modalities.
The useless hand syndrome is a very specific MS symptom and is only rarely caused by other
disorders. Patients with the sensory useless hand may note subjective numbness and a loss of
discriminatory and proprioceptive function, resulting in difficulties of writing, typing, buttoning
clothes, and holding objects, especially when not looking at their hand, This syndrome usually remits
over several months. Many MS patients have Lhermitte's sign, an electric or coldlike paresthesia
trending from the nuchal region down the spine, sometimes as far as the legs, on flexion of the neck.
Pain iri MS patients most often appears in the form of trigeminal neuralgia, a severe pain in the limbs,
tonic spasms, or backaches, sometimes with radiation in a radicular pattern. Other painful phenomena
include flexor spasms due to spasticity, contractures, and dysuria due to urinary tract infection.
Visual impairment in MS is usually due to optic neuritis. The most common manifestations are a
visual loss in one 130
eye, periocular
І РАПТ pain, especially with eye movement, a central scotoma that evolves
2: Special Neurology
over a few days. Bilateral simultaneous optic neuritis is uncommon in MS adults, physical exercises,
high ambient temperature, menstruation, or cigarette smoking can aggravate existing of visual problems
(Uhthoffs phenomenon). Most patients begin to recover within 2 weeks, and a significant visual
recovery is common.
Optic neuritis frequently may be an initial symptom of MS, but even if its clinical history is negative,
the evidence of optic nerve involvement is often fixed on funduscopic examination — partial atrophy—
or by visual evoked potentials.
Cerebellar pathways are frequently involved during the course of MS. The manifestations include
dysmetria, adiadochokinesia, action tremor with terminal accentuation — intention tremor, a loss of
balance, "jiggling" gait, an ataxic dysarthria with imprecise articulation, scanning speech, Classical
cerebellar disorders are described as Charcot's triad: intention tremor with finger-nasal and heel-to-shin
tests, scanning speech and nystagmus.
Urinary urgency — frequent and urgent incontinence, due to detrusor hy- perreflexia or
detrusor-sphincter dyssynergia, results from spinal cord lesions arid is frequently encountered in MS
patients. Symptoms of bladder dysfunction may be transient and occur with an exacerbation but are
commonly persistent. The resulting bowel and bladder dysfunction in MS may lead to hydronephrosis
and chronic renal failure if untreated.
Cranial nerve impairment may be seen with lesions that affect brainstem nuclei or exiting and
entering fibers. Usually this occurs in association with other symptoms. The oculomotor, abducens,
trigeminal, facial nerve may be involved. Diplopia is commonly due to internuclear ophthalmoplegia.
Impairment of cranial nerves IX, X, and XII generally appears late in the course of some patients.
Cognitive disorders ate defined up to 70 percent of MS patients. The problems are often subtle and
may not be detected on standard mental status evaluation. Its degree mainly depends on the total lesion
load seen on MRI, as well as brain atrophy, enlarged ventricles, and thinning of the corpus callosum also
portend symptoms of cognitive dysfunction. Depressive disorders occur among people with MS at rates
higher than in most other populations with chronic neurologic disorders.
The diagnosis of MS is based on the demonstration of white matter lesions disseminated in time and
space in the absence of another identifiable explanation.
At least two attacks and evidence of two separate CNS lesions (clinical or paraclinical) are required
foi the designation of clinically definite IVIS MS remains a clinical diagnosis, although MRl, evoked
potentials, and CSF examination can help clarify less certain cases.
MRl of the head is the most sensitive study for MS and is more useful than computed tomography.
Demyelinated lesions have the appearance of focal areas of an increased T2-weighted and a decreased
T1 -weighted signal, their size are larger than 6 mm, an oval shape, with locations in the periventricular
area, corpus callosum, and posterior fossa. MRl of the spinal cord shows discrete lesions in about 80
percent of clinically definite MS patients.

Gadolinium enhancement, indicating blood-brain barrier disruption, can show acute lesions. FLAIR
imaging is especially helpful for evaluating periventricular lesions that may be unnoticed on regular
T2-weighted scans.
Brain atrophy very often accompanies growing progressively worsening of the disease.
Additional innovative MRI techniques are also useful in the diagnosis of MS. MR spectroscopy gives
the information about the biochemical constituents of the imaged substance. In MS the
N-acetylaspartate (NAA)/creatinine ratio is decreased in theareas of axonal or a neuronal loss.
Diffusion-weighted imaging can show early stages of MS plaque formation.
"Positive CSF' is defined by either the presence of oligoclonal hands as a result of intrathecal IgG
production different from any such bands in serum, detected by isoelectric focusing, or by a raised IgG
index.

17. Demyelinating diseases of tlie nervous system. Multiple sclerosis,... | 131

 Evoked potentials (EP) are summed cortical electrical responses to peripheral sensory stimulation.
They can be used to localize sites of pathology and measure conduction velocity along sensory
pathways. Visual EP and somatosensory EP can detect subclinical lesions of demyelinatioiij in that way
provide evidence of multifocality.
Degrees of severity. The following scale is commonly used in clinical practice. It distinguishes 5
degrees of severity of MS, from slight (I) to serious (V):
I — there are no complaints, but organic neurological symptoms are shown,
II — there are some complaints, or ganic neurological symptoms are shown, but the patient's ability to
work is preserved,
III — evident neurological symptoms, a patient can move with the help of a chain on the distance of
200-300 m, is able to help himself at home but the ability to work is lost,
IV — severe neurological symptoms, is able to help himself only at home,
V — a patient can't move, needs the help of other people in everyday life.
In world's practice the Expanded Disability Status Scale (EDSS) is used. It provide for division into
10 steps in 8 systems: pyramidal, cerebellar, brainstem, sensory, bowel and bladder, visual, cerebral
and other.
Treatments focuses on three areas:
► Treatment of acute exacerbations and hastening of their-recovery,
► altering the natural history of MS,
► and providing symptomatic relief of current symptoms by enhancing of physical abilities and
preventing or treatment of complications.

Acute exacerbations. Corticosteroids are the most commonly used medicines for MS. The current
recommendation is to treat disabling attacks with 500 to 1000 mg of intravenous methylprednisolone
per day for 3 to 5 days with or without a short tapering dose of oral corticosteroids.
Altering the course of RR MS should be carried out protractedly and uninterruptedly.
Beta-interferons are a class of peptides that have antiviral and immunoregulatory functions. In MS they
allow to reduce the frequency and severity of relapses, show a good effect on MRI measures of the disease
activity. There are interferon-beta 1b (Betaferon) with subcutaneous injection every other day and
interferon-beta 1a (Avonex/Rebif) once-weekly intramuscular or subcutaneous three times weekly
injections. The main side effect is fever-like syndrome after injections.
Glatiramer acefate has a similar to interferons effect. It is a synthetic mixture of polypeptides
produced by the random combinations of four amino acids that are frequent in MBP. Glatiramer acetate
reduces new lesion formation and volumes, its side effects are relatively minor compared to
interferons. Co- paxon is administered daily subcutaneously.
Altering the course of SP MS. Treatment approaches aimed to affect the natural course of the
disease are available for these patients. Interferon-beta 1b may have a beneficial effect on the overall
outcome of SP MS. Intravenous steroids may also have long-term effects on disease progression when
given regularly. Mitoxantrone may stabilize or improve the course of disease with every 3 month
administration of intravenous medication. The greatest concern regarding last medication is its cardiac
toxicity. Repeated MRl show a positive effect in the treated patients.
Symptomatic treatment. Spasticity requires prescription of baclofen. Doses should be escalated
slowly to prevent the occurrence of adverse side effects, up to 120 mg/day. Symptoms of a hyperreflexic
bladder are often manageable with anticholinergics. For treating fatigue, medications are only partially
effective. Amantadine in the dose of 100 rng is the standard initial treatment. Paroxysmal symptoms are
highly responsive to rmedical
132 I PAR treatment. A small dose of carbamazepine is often very effective.
2. Special Neurology
Emotional Incontinence may be amenable to a low dose of tricyclic antidepressants.

Acuta Disseminated Encephalomyelitis (ADEM)

ADEM is a monophasic inflammatory demyelinating disorder that characteristically begins within 6
weeks of an antigenic challenge such as infection or immunization. It occurs more often in the young
and causes the rapid development of multifocal or focal neurological deficits. Perivenous inflammation,
edema, and demyelination are the pathological hallmarks of ADEM. These lesions commonly enlarge
and coalesce, forming lesions pathologically indistinguishable from MS.
 Epidemiology and risk factors. ADEM can occur at any age but perhaps because of the higher
frequency of immunization and exposure to new antigens, it is most common during childhood. Unlike
MS, both sexes are affected with equal frequency. No association has been noted with pregnancy.
Clinical features. A prodrome of headache, low-grade fever, myalgias, and malaise often precede the
onset of ADEM by a few days. The most frequent clinical signs are motor deficit, followed by sensory
deficits, brainstem signs, and cerebellar signs. CSF findings are variable, normal results were present in
up to 20 percent of patients. Oligoclonal bands were positive in over 60 percent.
Neurological symptoms develop rapidly in the acute phase and are commonly associated with
encephalopathy, stupor, coma, meningismus, and seizures. Peak severity occurs within several days,
and recovery may begin soon afterward. The prognosis for recovery is often quite good.
Treatment with intravenous methylprednisolone leads to a stop of the disease progression and allows
sooner recovery, just as with MS. Plasma exchange can be tried in those with severe deficits and little
response to corticosteroids. Intravenous immunoglobulin (IVIG) has also been used successfully.
Amyotrophic lateral sclerosis (ALS)
ALS is the most frequent of motor neuron diseases — devastating neurological disorders of adults.
ALS is a fatal motor neuron degenerative syndrome inherited in approximately 10 % of people, of
which 20-25 % are associated with a mutation of the gene superoxide dismutase. The sporadic and
familial forms of ALS are clinically and pathologically similar. ALS was described for the first time in
1874 by J. M. Charcot and still referred to as Charcot's disease in many parts of the world.
Etiology. The cause of ALS is remained unknown. Many causes have been proposed, including
toxicity from excessive excitation of the motor neuron excitation transmitters, such as glutamate, free
radical mediators, mitochondrial dysfunction, cytokine abnormalities, trophic factor deprivation and so
on.
A high rate of detection of enterovirus nucleic acid in the neuronal cytosol within the grey matter of
the spinal cord ot patients with ALS suggests an association between persistent enterovirus and ALS.
Pathology. ALS is a primary disorder of motor cells and their axons. The hallmark of ALS is atrophy,
degeneration, and a loss of motor neurons in the lower brainstem and anterior horn of the spinal cord,
followed by glial replacement. There is a loss of pyramidal cells from the motor cortex of the prefrontal
gyrus and large myelinated fibers of the anterior and lateral columns of the spinal cord, the brainstem,
arid the cerebrum.
Epidemiology and Risk Factors. Sporadic ALS has a worldwide incidence of 1 to 2 in 100.000
persons. Clusters of the disease have been identified, particularly in the Kii peninsula of Japan and the
Mariana Islands. The incidence is greatest between the ages of 50 and 70 years. The male-to-female
ratio is about 1.3:1. There is recent evidence of association between increased dietary fat and cigarette
smoking.
Clinical picture. ALS is a syndrome of upper and lower motor neuron dysfunction at several levels
of the neuraxis without the involvement of other neurological systems. Generally the patient seeks
attention because of symptomatic weakness, although a history of!fasciculations! muscle cramping, and
atrophy before weakness is apparent. There is the presence of hyperreflexia in segmental regions where
muscles are hypotrophic without sensory disturbances in the same zones. Limb involvement occurs
more often than bulbar involvement, and the upper limbs are more often affected than the lower limbs in
sporadic ALS. Inverse proportion can be seen in familiar ALS.
Primary lateral sclerosis and progressive pseudobulbar palsy are both syndromes confined to the
upper motor neuron, whereas progressive muscular atrophy and progressive bulbar palsy affect only the
lower motor neuron. If the upper motor neurons are affected prepotently, the symptoms will be
primarily clumsiness, stiffness, and fatigue, while lower motor neuron lesion will present as weakness
or atrophy and occasionally fasciculations. Bulbar symptoms occur with including hoarseness, slurring
of speech, choking on liquids, and difficulty initiating swallowing. Fasciculations, especially of the
tongue, are considered by some to bediseases
17. Demyelinating specifically
of tlie nervousassociated with ALS.
system. Multiple sclerosis,... | 133
There is a wide variation in disease progression and duration, neither of which can be accurately
predicted from the age or level of onset, although generally elderly patients have a shorter survival. ALS
remains a fatal disease, with a mean survival of 3 years after the onset of symptoms.
Diagnosis. Clinical picture with symptoms of upper and lower motor neuron damage simultaneously
always are presumably for ALS. Electromyography (EMG) is used as additional method. EMG features
commonly include fibrillations, positive sharp waves, and complex repetitive discharges that indicate
denervation.
 Treatment. Only benzothiazole riluzole has been shown to extend lifespan. Riluzole acts to block
voltage-activated sodium channels and to protect against glutamate toxicity. Standard dosage is 50 mg
twice daily. Drugs such as baclofen, quinine, and phenytoin can be used to reduce cramping; anticho-
linergic agents can be used to control sialorrhea. Amitriptyline or selective serotonin reuptake inhibitors
may help control pseudobulbar symptoms in some patients.

Tests and typical tasks

I. Indicate the main causes of multiple sclerosis.
a) viral infection and inherited predisposition
b) disturbances in blood circulation and copper metabolism
c) bacterial or fungal infection
d) high arterial blood pressure and hypercholesterolemia
emotional stresses and hypodynamia

2 What structures are damaged in multiple sclerosis most frequently?

a) brain grey matter
b) peripheral nerves
c) the brain and spinal cord white matter
d) spinal cord grey matter
e) brain membranes

3. Indicate changes of motor system in multiple sclerosis.

a) central paresis
b) paresis of masticatory muscles
c) jackson epilepsy
d) peripheral paresis
e) fasciculations
f) muscles atrophy

4. What drugs are used in the acute stage of multiple sclerosis?

a) antibiotics
b) corticosteroids
c) cytostatics
d) immunomodulators
e) coagulants

5. What structures of the nervous system are damaged in amyotrophic lateral sclerosis?
a) spinal cord anterior and posterior roots
b) peripheral nerves and ganglions
c) sensitive pathways and the cerebellum
d) pyramidal tracts, spinal cord anterior horns
e) the nervous plexus and vertebral nervous nodes

6. Apatient lias lower spastic paresis, abdominal reflexes and vibration are absent, frequency and urge
incontinence is present. What structures are damaged? What disease are such symptoms typical for ?
7.Against a background of catarrh of the upper respiratory tracts and high temperature, there was an
acute appearance of vomiting, meningeal signs, paresises of the extremities, bulbar disorders. What
disease can be suspected?

8. During examination of 2:a Special

134 І РАПТ patient there was detected mixed upper paraparesis with atrophy of the arms
Neurology
and shoulder girdle, increased reflexes of hands, fibrillary twitches in muscles. What structures were
affected? What disease can be suspected?

9. A 26-years-old woman has a decreased visual acuity on the right eye. She had effective treatment of
retrobulbar neuritis. A year later she started suffering from diplopia, weakness in the legs, staggering
gait, difficult urination. I he patient doesn't lead right eyeball outwards, has horizontal nystagmus,
scanning speech, central tetraparesis, pathologic Bablnski's sign on the both sides, intention tremor
 and missing the mark with coordination tests, reduction of vibration in the extremities. There is
atrophy of disks on the eye-ground. Determine topical diagnosis. Give the clinical diagnosis.

10. A 16 years-old teenager on the third day of fever and high temperature had a headache,
psychomotor agitation. On the fifth day the patient had weakness in leg; and arms, dysphagia, urinary
retention. There were detected strabimus, moderate ptosis, tetraparesis with a low muscular tonus,
painfulness of peripheral nerves, distal hyperstesia, moderate meningeal signs. What is the clinical
diagnosis? What additional methods of examination must be carried out?

17. Demyelinating diseases of tlie nervous system. Multiple sclerosis,... | 135

 18. HEREDITARY DISEASES OF THE NEUROMUSCULAR
APPARATUS. MYASTHENIA AND MYASTHENIC SYNDROMES. HEREDITARY
DISEASES WITH LESION OF THE PYRAMIDAL, EXTRAPYRAMIDAL AND
COORDINATIVE SYSTEMS

Hereditary diseases at the neuromuscular apparatus

Hereditary diseases of the neuromuscular apparatus constitute a large group of inherited maladies
which mark out especially progressive muscular dystrophies, spinal muscular atrophies and hereditary
neuropathies.

Progressive muscular dystrophies

The muscular dystrophies (MD) are a group of progressive hereditary degenerative diseases of
skeletal muscles. W.H.Erb in 1891 formulated the clinical and histological concept of a group of
diseases caused by primary degeneration of muscle, which he named muscular dystrophies. In the
history of dystrophies, the most notable event was the discovery by Louis M. Kurikel in 1986 of the
dystrophin gene and its protein product. Since then there has been an extraordinary accumulation of
molecular- genetic, ultrastructural, and biochemical information about muscular dystrophies, which has
greatly broadened our understanding of their mechanisms.
Etiology and pathogenesis. Muscular dystrophies are a group of hereditary diseases caused by
abnormalities of the dystrophin-associated membrane complex which play a vital role in maintaining the
normal structure and function of muscle cells. In muscular dystrophy of the Duchenne or Becker type,
for example, a genetic defect leads to a deficient or absent expression of the structural protein
dystrophin; these diseases are thus referred to as dystrophinopathies. A particular protein involved also
determines whether the disease will affect all muscles or will preferentially affect oculopharyngeal
muscles, the limb girdles, or the distal limb muscles.
Duclmnne pseudohypertrophic muscular dystrophies
In 1855, the French neurologist Guillaume B. A. Duchenne described the progressive muscular
atrophy of childhood that now bears his name. This is the most frequent and best known of the early
onset muscular dystrophies. It begins in early childhood and runs a relatively rapid course. The
incidence is in the range of 13 to 33 per 100.000 yearly or about 1 in 3.300 live male births. There is a
strong familial liability as the disease is transrfiitted as an X-linked recessive trait (Xp21.1
chromosome), occurring almost exclusively in males. Approximately 30 percent of patients have no
family history of the disease and these represent spontaneous mutations.
Clinical features. Duchenne MD is usually recognized by the third year of life and almost always
before the sixth year. Delivery and early childhood development are normal. The child's movements
become clumsy, incr easing difficulty in climbing stairs, walking, jumping and running. The proximal
muscles of the pelvic girdle are symmetrically involved at first, less and later involved the shoulder
girdle. A loss of muscle fibers leads to clinically evident muscle atrophy. In some muscles, however, the
replacement of muscle fibers by fat and connective tissue can compensate for the lost muscle volume or
even produce pseudohypertrophy, particularly in the calves and buttocks.
The weakness of the hip muscles leads to a bilateral gait abnormality, with a characteristic waddling
appearance. To stand up from the lying position the affected boys adopt the Gower's manoeuvre (stairs):
turn themselves prone, raise the buttocks, and then climb with their ai ms up their own thighs till they are
upright "climbing up his own trunk" (Fig.
17. Demyelinating diseases18.1).
of tlie nervous system. Multiple sclerosis,... | 136
Weakness of abdominal and paravertebral muscles gives the patient a characteristic lordotic posture.
The tendon reflexes are diminished. Dystrophin is expressed not only in skeletal musde, but also in the
smooth muscle and in the brain. The heart is affected by various types of arrhythmias. Mild degrees of
mental retardation are observed in many cases. Most patients become unable to walk at some time
between the ages of 8 and 15. Death is usually the iesult of pulmonary infections and respiratory failure
and sometimes of cardiac decompensation.
 Diagnosis. The serum creatinekinase (CK) concentration is elevated, especially at the beginning of
disease; the values are 50 to 100 times normal. The EMG reveals a myopathic pattern. The nerve
conduction velocities are normal.

Fig. 18.1. Duchenne pseudo hypertrophic MD. Gower's manoeuvre

Muscle biopsy reveals a pathological variation of muscle fibers. Blood cells or muscle should be
examined for deletions or duplications in the dystrophin gene. These findings are important for prenatal
diagnosis and for the detection of female carriers. Careful examination of the mothers of affected boys
shows in half of cases slight muscle involvement (slight weakness and enlargement of the calves as well
as elevaled CK values and abnormalities of the EMG and muscle biopsy).

Becker muscular dystrophies

This milder dystrophy is closely related to the Duchenne type clinically, genetically, arid
ultrastructurally. Dystrophin is present in a diminished quantity and/or abnormal distribution. The
incidence is in the range of 3 lo б per 100.000 male. Like Duchenne form, it is an.X-linked disorder
limited to males and transmitted by females.
Clinical features. It causes weakness and hypertrophy in the same muscles as Duchenne dystrophy
(proximal weakness and atrophy, with pseudohypertrophy of the calves), but the onset is much later
(mean age: 12 years; range: 5 to 45 years), progression is much slower and relatively benign. On
average, patients become unable to walk in their thirties. Most die in the fifth decade of life. Intellect is
usually normal and cardiac involvement is far less fiequent than in Duchenne dystrophy.
Diagnosis. The serum CK concentrations elevated less than in Duchenne form. The findings of EMG
and muscle biopsy resemble those in Duchenne MD.

Landouzy— Dejerine facioscapulohumeral muscular dystrophies

І РАПТ 2: Special Neurology
The first descriptions of facioscapulohumeral dystrophy were published by L. T. J. Landouzy and J. J.
Dejerine in 1894. This is a slowly progressive dystrophy involving primarily the musculature of the face
and shoulders. The pattern of inheritance is autosomal dominant. There are also occasional sporadic
cases. The underlying genetic defect is a deletion in the 4d35 region of chromosome 4. The incidence of
this disorder is relatively high: 4 per 100.000 births. The age of onset is usually between 6 and 20 years
and progresses veiy slowly.
Clinical features. The disease initially affects the face and the shoulder girdle. Eye closure may be
incomplete. There is an inability to purse the lips, drink through a straw, to whistle, smile looks
"transversal", the lips have a tendency to protrude. The objective findings include a myopathic face
without ptosis.
 There is a difficulty in raising the arms above the head. Atrophic process involves the proximal
muscles of the shoulder gir die. The bones of the shoulders become prominent; the scapulae are winged
and elevated ("angel-wing" appearance), and the clavicles stand out. If the examiner tries to pick up ttie
patient by holding him under the axillae, he tends to slip out of the examiner's hold ("loose shoulder
sign"). The pelvic girdle muscles are involved later and to a milder degree. Many of the patients with
milder degrees of this form of dystrophy are unaware that they have the disease. Cardiac involvement is
rare and mental function is normal.
Diagnosis. The serum CK concentration is normal to mildly elevated. EMfi and muscle biopsy reveal
myopathic changes.

Erh limb-girdle muscular dystrophies

There is a large heterogenous group of patients with muscular dystrophy the only unifying feature
being the presence of limb-girdle weakness without weakness of the facial muscles and hypertrophy.
The inheritance is variable but the autosomal recessive forms are the most common. Now limb-girdle
muscular dystrophies (LGMD) are classified as L.GMD1 for the autosomal dominant types (6 forms)
and LGMD2 for the autosomal recessive types (11 forms). The clinical manifestations are highly
variable.
Clinical features. Weakness and atrophy may become evident during either late childhood or early
adult life and spread from shoulders to hips or from hips to shoulders. Sometimes pseudohypertrophy
may occur. There are no sensory disturbances. The proximal muscles of the pelvic girdle and/or shoul-
der girdle are involved more than distal. Weakness of the pelvic girdle muscles leads to characteristic
waddling gait, lordotic posture, Gower's sign, when the patient try to stand up from a lying position.
Cardiac involvement is infrequent, and mental function is normal. In case of the later onset of these
disorders, the course will be benign.
Diagnosis. Increasing of serum CK concentration, EMG and muscle biopsy findings help to confirm
diagnosis. In case of the later onset of LGMD the EMG of lesser-affected patients is myopathic, the CK
values are only moderately elevated and may be normal. More severe cases can have greatly elevated
CK levels.

Spinal muscular atrophy

Spinal muscular atrophies (SMA) are diseases of the motor neurons of the medulla and spinal cord
that most often present with muscle weakness in infancy and childhood. The inheritance pattern is most
commonly autosomal recessive, but there are torms that are dominant and X linked. All forms of
autosomal recessive SMA have been linked to chromosome 5q11.3-13.1, a region that contains multiple
copies of genes and pseudogenes and is characterized by instability. Deletions, truncations, or point
mutations in the gene for survival of motor neurons (SMN) result in death of neuronal cells in the
anterior horn of the spinal cord and subsequent system-wide muscle wasting (atrophy). Caused by lesion
of the anterior corn, fasciculations are practically always present, generalized symmetrical proximal
weakness and atrophy with the absense of deep reflexes and normal sensation. The autosomal recessive
SMA are the most common heritable cause of deaths in infancy, with incidence estimates of 1 in 10.000
to 25.000 in the Western world.

Infantile SMA (Werdnig - Hoffmann)

It is the most frequent and severe form of spinal muscular atrophies, occurring once in every 20.000
live biiths. Usually onset is in infancy, most commonly between the ages of 6 and 12 months. In rare
cases, the disorder is already apparent prenatally.
Clinical features. In severe cases with early onset the muscle weakness is generalized froin the
beginning. Hypotonia accompanies
138 І РАПТ the weakness. The child is flaccid and weak, lies with flexed arms
2: Special Neurology
("jug-handle posture") and floppy legs in the "frog position". The cry becomes feeble, and sucking and
swallowing are less efficient, breathing becomes paradoxical due to involvement of the bulbar cranial
nerves nuclei. Volume of muscle is diminished but is difficult to detect in the extremities because of the
coverings of adipose tissue. Fasciculations are mainly seen in the tongue. The weakness and hypotonia
progress gradually and spread to all of the skeletal muscles except for the ocular ones. The tendon
reflexes are absent. Death comes early, usually within the first year due to respiratory failure related to
diaphragmatic weakness.
 Infants in whom the disease becomes apparent only after several mouths or after first year of life have
a less-rapid course of disease. In this case, muscles of the trunk and proximal parts of the extremities are
at first affected, while the fingers and hands, toes and feet, and cranial muscles retain mobility. Some of
them become able to sit and creep and even to walk with suppoit and may survive for several years and
even into adolescence or early adult life.
Diagnosis. Muscle enzymes in the serum aie usually normal. The EMG displays fibrillations, motor
unit potentials are diminished in number. In the more slowly evolving cases, some are larger than
normal — giant or polyphasy potentials reflecting ^'innervation. Motor nerve conduction velocities are
normal.

Kugelberg - Welandar SMA

Kugelberg — Welander SMA (also known as Wohlfart — Kugelberg — Welander syndrome or mild
SMA) is a milder form of SMA, with symptoms typically presenting after the age of 18 months.
Clinical features. The disease usually becomes evident between the 2 and 10 year with weakness and
atrophy of the pelvic girdle and proximal leg muscles, followed by involvement of the shoulder girdle
and upper arm muscles. Atrophy is bilaterally symmetrical from the beginning. The absence of the knee
reflex is an early sign. Pseudohypertrophy of the calf may be seen, and fasciculations are practically
always present. Corticospinal tract signs and bulbar signs are usually absent. The disease progresses
very slowly, and some patients survive to old age without serious disability. In general, the earlier the
onset, the less favorable the prognosis; however, even the most severely affected patients retain the
ability to walk for at least 10 years after the onset.
Diagnosis. Patient will present hypotonia associated with absent reflexes and the EMG will show
fibrillation and muscle denervation are presumedly by SMA. Serum creatine kinase may be normal or
increased. Genetic testing will show bi-allelic deletion of exon 7 of the SMI\I1 gene — this is conclusive
of the disease.

Hereditary neuropathies
In general, the hereditary sensory and motor neuropathies are peripheral neuropathies that affect
autonomic nerves, sensory nerves, motor fibers, or a combination thereof. The various types can be
diagnosed on the basis of their clinical manifestations, electrophysiologic findings, histologic
characteristics on nerve biopsy, and genetics. The sign of polyneuropathies are genetic transmission,
complete symmetry, very slow progression, and a loss of myelin disproportionate to axons.

Peroneal muscular atrophy.

Charcot - Marie - Tooth disease
The disease was described in 1886 almost simultaneously by H. H. Tooth in England and by J. M.
Charcot and P. Marie in France. This is the most common form of inherited peripheral neuropathy.
Although more than .50 genetic types have been described, only a few occur regularly. The frequency of
the disease is about 1 in 2500 of the population. The inheritance of Charcot — Marie — Tooth disease
(CMTD) is most often that of an autosomal dominant trait, less often it is autosomal recessive and rarely
X-linked. Probably a small number arise as the new mutations.
Clinical and electrophysiologic features. CMTD was subdivided into two broad types: CMTD 1
and CMTD 2. This subdivision was-made oil the basis of conduction velocity in the median or ulnar
nerve and EMG date — slow conduction in type 1 (demyelinating type), and normal or near normal
conduction in type 2 (axonal type).
Type 1 has its onset during the first decade while the peak age of onset of type 2 is in the second
decade or even later. Both motor and sensory signs more severe in the first type. The chronic
degeneration 18.
ofHereditary
peripheraldiseasesnerves results
of neuromuscular in distal
apparatus. muscle
Myasthenia atrophy
arid myasthenic beginning
syndromes... | 139 in the feet and legs and later
involving the hands. The initial finding in 70 % of cases is a deformity of the foot that first appears in
childhood, Fridreieh's foot (pes cavus) with high arches and hammertoes. Progressive atrophy of the calf
muscles innervated by the peroneal nerve, weakness of dorsiflexion with relatively preserved plantar
flexion is manifested as a steppage gait. The atrophy and weakness of the calf muscles may progress
over time, the powerful thigh muscles contrast with the wasted calf muscles ("stork legs," "inverted
bottle sign").
 After a period of many years, atrophy of the hand and forearm muscles develops, the hands become
clawed. Only about one-quarter lo half of all patients develop mild deficit of deep and superficial
sensation in the feet and hands, paresthesias and cramps usually only later in the course of the disease.
Walking difficulty is because of a combination of sensory ataxia and weakness. The only distinguishing
clinical feature between types 1 and 2, in only a minority of cases, is enlargement of the nerves in type 1,
most easily appreciated by palpation of the greater auricular and peroneal nerves. The tendon reflexes
are absent in the involved limbs. The illness progresses very slowly over decades, giving the impression
of stabilization for long periods and patients often can work even at the old age.
Diagnosis. EMG is of basic importance. The nerve conduction velocity is markedly diminished in all
cases, slowing of nerve conduction in CMT 1 in contrast to near-normal velocities with evidence of
denervation in type 2. Sural nerve biopsy reveals signs of chronic segmental denervation and
regeneration, axonal degeneration. Muscle biopsy reveals muscle changes which are typical of
neurogenic denervation.
Treatment. There is no specific treatment for myopathies, spinal and neural muscular atrophies.
Traditionally, drug therapy of all these diseases is aimed at normalization of muscular trophism
(ryboxin, rhetabolil, cerebrolisin), improvement of conduction through nerves (prozerin, galantamin, В
group vitamins), improvement of microcirculation (nicotinic acid, pentoxyphillin). The administration
of prednisone appears to slightly retard the time of progression of Duchenne dystrophy for the period of
up to 3 years. An optimal dose is 0.75 mg/kg given daily.
Therapy includes psychological counseling of the parents, physical therapy and orthopedic aids.
Important measures to be taken in early childhood to prevent contractures include regular exercise,
swimming, passive stretching of the limbs, night splints. Surgical fixation of the scapula to the chest
wall occasionally improves function. Stabilizing the ankles by arthrodeses is indicated in cases of CMT
— disease if foot-drop is severe, in mild and early cases — fitting the legs with light braces and the
shoes with springs to overcome foot- drop can be helpful.

Myotonia congenital. Thomsen's Disease

This is an uncommon disease of skeletal muscle that begins in early life and is characterized by
myotonia, muscular hypertrophy, nonprogressive course, and dominant inheritance. Thomsen's disease
is caused by one of several inherited molecular defects in the voltage-dependent chloride channel gene.
The disorder was first brought to the attention ot the medical profession in 1876 by Julius Thomsen, a
Danish physician who himself suffered from the disease, as 20 members of his family over 4
generations did. Later W. H. Erb provided the first description of its pathology and called attention to
two additional unique features: muscular hyperexcitability and hypertrophy.
Myotonia congenita becomes manifested at a very early age, sometimes "in the cradle." In other
cases, the myotonia becomes evident only later in the first or second decade.
Clinical features. Myotonia, a tonic spasm of muscle after forceful voluntary contraction, stands as
the cardinal feature and is best represented in this disease.
This phenomenon reflects electrical hyperexcitability of the muscle membrane. It is most pronounced
after a period of inactivity. At first, the patient cannot cany out any rapid movement, but this becomes
possible after repeated attempts. The disease is usually inherited as a dominant trait so that most often,
other members of the family have been affected. In many cases muscles are more prominent than
normal, so that patients may actually have an athletic habitus. One of the characteristic features is grip
myotonia in which the patient is unable to release a handshake and must slowly open the fingers one at a
time. After a period of rest, the patient may have difficulty in arising from a chair or climbing stairs.
The hypertrophic masseter muscles lend a characteristic appearance to the face. Smooth and cardiac
muscles are not affected and intelligence is normal. Myotonia can also be induced in most cases by
tapping a muscle belly with a percussion hammer (percussion myotonia) — contraction of a fascicle in
response to striking140the
І РАПТmuscle, it persists for several seconds. If tapped, the tongue shows a similar
2: Special Neurology
reaction. In Thomsen's disease, the stiffness is somewhat exaggerated in cold. On a cold day, affected
individuals may have a prolonged grimace with closed eyes after a sneeze. Myotonia tends to become
less severe over time, and patients are generally fully able to work. The life expectancy is normal,
Diagnosis. The EMG reveals low-amplitude, high-frequency potentials continue to be seen for
several seconds after the end of voluntaiy contraction and are heard in the audio channel as a
characteristic "motorcycle" or "dive-bomber" sound. The same occurs when the EMG needle is
mechanically displaced. When a nerve is electrically stimulated, even at low intensity, results is not a
motor unit contraction, but tetany. Muscle biopsy reveals no abnormality other than enlargement of
muscle fibers, and this change occurs only in hypertrophied muscles.
Treatment. Myotonic rigidity can be ameliorated by various types of medications that lower
membrane excitability, among them local anesthetics, antiarrhythmics, and anticonvulsants such as
mexiletine and phenytoin. Of these, mexiletine is the most effective. Acetazolamide has a palliative
effect in the Thomsen's form of myotonia congenita.

Myasthenia
Myasthenia gravis is a chronic autoimmune neuromuscular disease, characterized by weakness and
abnormal fatigue of striated muscle. It occurs in people aged 20-30 years. Women suffer 2 times more
often than men.
Pathogenesis. Myasthenia is considered as a classical organospecific antibody induced
T-cell-dependent autoimmune disease. The antibodies enter the competitive interaction ot acetylcholine
at postsynaptic plate, blocking neuromuscular transmission. In addition, antibodies block the
postsynaptic receptors, causing accelerated degradation ot receptors. A disorder ot acetylcholine
synthesis due to enzyme activity deficiency occurrs.
Clinical manifestations. A specific feature of myasthenia is abnormal fatigue of muscles that occurs
after physical exertion. Muscle weakness is different from usual paresis with repeating stereotyped
movements; it sharply increases and can reach a degree of paresis or paralysis. After having a rest the
quantity of movements increases.
According to clinical symptoms there are distinguished localized myasthenia with the defeat of
oculomotor muscles (ocular form), the tongue muscles, laryngeal (bulbar form) and generalized one
—accompanied by lesions of muscles face, neck, trunk, and extremities. Approximately in 70 % of
patients with myasthenia disease begins with oculomotor disorders and in 20 % — with bulbar.
In typical cases the first disturbances are oculomotor disorders, patients complains of drooping
eyelids, doubling of visual objects. During the neurological examination ptosis is revealed, often
asymmetrical one and increasing during gaze fixation. Later weakness and fatigue of masticatory and facial
muscles appear. A typical complaint of patients is fatigue of masticatory muscles during chewing food.
In severe cases patients should take a break while eating.
Disorder of bulbar muscle causes dysfunction of the soft palate and epiglottis; patients complain of
difficulty swallowing, "nasal" tone of their voice. If severe illness patients cannot swallow the saliva,
chew solid food. Disturbance of the act of swallowing can develop aspiration pneumonia or alimentary
exhaustion. If there is a generalized form of myasthenia one of the most serious symptoms is weakness
of respiratory muscles.
The course of the disease is progressive, often with remission. The patient's condition worsens after
influenza and other infections or intoxications, although decline can develop for no apparent reason.
Sudden sharp deterioration of ttie patient is called myasthenic crisis. In that case generalized muscle
weakness, pronounced bulbar disorders, respiratory failure and cardiac activity develop. Muscle
weakness that sometimes comes to the state of quadriplegia accompanied by pronounced autonomic
disorders: tachycardia, weak pulse, mydriasis. In such cases for tens of minutes death may occur.
Diagnosis. Myasthenia is diagnosed on the basis of the following clinical signs:
> appearance of muscle weakness without pain and sensitivity disorders;
> early asymmetric involvement of oculomotor muscles while maintaining the pupillary reactions;
> combination of muscle weakness with normal or sometimes increased tendon and periosteal
reflexes;
> recovering or decreasing of weakness under the influence of anticholinesterase medications.

Besides clinical symptoms pharmacological tests with prozerin, electromyography, blood test for
antibodies to the acetylcholine receptor, CT or MRl of the mediastinum to detect a pathology of the
18. Hereditary diseases of neuromuscular apparatus. Myasthenia arid myasthenic syndromes... | 141
retrosternal gland are carried out.
Treatment can be conservative and surgical. In order to compensate disorders of neuromuscular
transmission anticholinesterase agents are used: calimin, ubretyd, neuromidin. Pulse therapy by
glucocorticoids is successfully used — methylprednisolone with a dose of 250-500 mg daily for 3-5
days. In some cases the transfer of patients with an average dose of prednisolone orally every two days
(40-60 mg) is needed. Sometimes cytostatic immunosuppressants (azathioprine, cyclophosphamide) or
plasmapheresis are used. The main indications for plasmapheresis using: myasthenic crisis, acute
myasthenia course, the initial steroid therapy linked with possible disease exacerbation.
Surgical treatment (thymectomy) can be applied in thymoma presence in the case of generalized
myasthenia with symptoms of bulbar palsy and if there is no effect of conservative therapy.
Congenital myasthenic syndromes are characterized by fatigable weakness of skeletal muscle
(e.g., ocular, bulbar, limb muscles) with onset at or shortly after birth or in early childhood. Cardiac and
smooth muscle are not involved. The severity and course of the disease are highly variable, ranging
from minor symptoms to progressive disabling weakness. Later childhood onset subtypes show
abnormal muscle fatigability with difficulty in activities such as running or climbing stairs; fluctuating
eyelid ptosis and fixed or fluctuating extraocular muscle weakness a r e common presentations.
Hereditary diseases with lesion of the pyramidal system. Hereditary spastic paraplegias

Hereditary spastic paraplegias or Strumpell — Lorrain syndrome, are a group ot genetically and
clinically diverse disorders characterized by lower extremity spasticity and weakness. Generally, they
are classified according to the mode of inheritance and symptoms.
Pathogenesis. It has been well established that uncomplicated autosomal dominant, autosomal
recessive, and X-linked hereditary spastic paraplegias, are heterogeneous disorders. Because families
are linked to different genetic loci, there may be various points of disturbance in a common biochemical
pathway that leads to degeneration of the longest ascending and descending CNS pathways, particularly
the corticospinal tracts from the motor cortex to the legs, the fasciculus gracilis fibers, and the
spinocerebellar fibers. Genetic penetrance is age-dependent and nearly complete.
Clinical features. The patient generally presents with leg stiffness, weakness in the hip flexors, and
impaired foot dorsiflexion in the second through fourth decades. The gait disturbance progresses
continuously. On neurological examination, generally there are no abnormalities of the corticobulbar
tracts or upper extremities, except for possibly brisk deep tendon reflexes. In the lower extremities, deep
tendon reflexes are pathologically increased and there is decreased hip flexion and ankle dorsiflexion.
Crossed adductor reflexes, ankle clonus, and extensor pathological plantar responses are present.
Patients may also have paresthesia and mildly decreased vibratory sense below the knees, slight
dysmetria, and urinary urgency and incontinence late in the disease. Pes cavus may be present
Diagnosis. Particular care is necessary if the family history is not revealing or if there are numerous
complications or atypical features. Because hereditary spastic paraplegias can mimic treatable
disorders, such as vitamin B12 deficiency, dopa-responsive dystonia, cervical spondylosis, or multiple
sclerosis, exhaustive evaluation is required. Electrophysiological studies are the most revealing.
Somatosensory evoked potentials of the lower extremities show conduction delay while evoked
potentials of the arms are either normal or mildly slow. Magnetic resonance imaging of the brain and
spinal cord should be performed. Molecular diagnosis is available clinically only for pro- teolipoprotein,
while testing for L1 cell adhesion molecule, atlastin, spastin, and paraplegin is available at some
research facilities.
There is no treatment available to address the underlying process in hereditary spastic paraplegias.
Symptomatic treatment tor the lower extremity spasticity by oral or intrathecal baclofen or less
commonly oral dantrolene can be helpful.

Hereditary diseases with lesion on the extrapyramidal system. Parkinson s disease

Parkinson disease (PD) was described by James Parkinson (1817) in "An essay on the shaking palsy'.
Prevalence rates of PD amounts 190 per 100.000, male to female ratio — 3:2. Age of onset: 50 years
upwards. Incidence peaks in mid-70s then declines. More than 15 genes of hereditary forms of primary
parkinsonism are identified. 6 genetic variants are the most studied and the most important in studying
of PD, among them PARK1 (a-synuclein), PARK2 (parkin), PARK6 (pinki) and others.
Pathology. The142substantia nigra contains pigmented cells (neuromelanin) which give it a
І РАПТ 2: Special Neurology
characteristic "black" appearance (macroscopic). These cells are lost in PD and the substantia nigra
becomes pale. Remaining cells contain atypical eosinophilic inclusions in the cytoplasm — Lewy
bodies.
Clinical features. Clinic is characterized by availability of 4 main symptoms: tremor, muscle
rigidity, bradykinesia, and postural instability (see Chapter 3).
A coarse tremor at a rate of 4-7 Hz usually develops early in the disease. It is often "pill rolling",
begins unilaterally in the upper limbs and eventually spreads to all limbs. It occurs at rest, improves with
movement and disappears during sleep. Rigidity is detected by examination. It predominates in flexor
muscles of the neck, trunk and results in typical "mannequin's posture". The gait becomes shuffling and
posture more flexed.
Diagnostics. There are criteria for clinical diagnostics of the PD: presence of hypokinesia and
additionally one of the following symptoms: tremor at rest, muscle rigidity and postural instability, a
stable positive effect to the peroral use of L-DOPA a of 250 mg, asymmetrical disease onset,
progressive course, the absence of possible etiological factors of secondary parkinsonism in the case
history.
Treatment. There are 5 directions of therapeutic correction dopamine insufficiency for patients with
PD:
► an increased dopamine synthesis in the black substance using products containing L-DOPA.
L-DOPA prescribed dose is 100 mg 2-3 times a day, and then, if necessary, the dose is increased;
► direct stimulation of dopamine receptors of a striped body. For this purpose dopamine agonists
are used — derivatives from alkaloids (bromocriptine, parlodel) and non-ergolinic synthetic drugs:
pramiksol (mi- rapex), ropinirol;
► stimulation of dopamine discharge from presynaptic neurons (neomi- dantane, amantadine).
► using of central cholinolytics that reduce high cholinergic activity of the caudate nucleus
(cyclodol, parcopan).
► MAO inhibitors type В (selegelin) which reduces the catabolism of dopamine and increases its
amount and the duration of the effect tor dopamine receptors.

Huntington's disease
Huntington's disease (HD) is inherited as an autosomal dominant disease that is developed choreic
movements and dementia. It has a frequency of 4 to 7 per 100.000 persons and typically becomes
noticeable in mid-adult life. It is much more common in people of Western European descent than in
those of Asian or African ancestry. The disease is caused by an autosomal dominant mutation in either
of an individual's two copies of a gene called Huntingtin. HD is associated with increases in the length
of a cytosine-adenine-guanine (CA6) coding for the amino acid glutamine triplet repeat, presented in
this gene located on chromosome 4pl6.3.
Pathology: A neuronal loss in the striatum is associated with a reduction in projections to their basal
ganglia structures. In addition, cells of the deep layers of the frontal and parietal cortex are lost. The
neurochemical basis of this disorder involves deficiency of CAG and acetylcholine with a reduced
activity of some enzymes.
Clinical features: Physical symptoms of HD usually begin between 35 and 44 years of age. The
earliest symptoms are often subtle problems with mood or cognition. A general lack of coordination and
an unsteady gait often follows. As the disease advances, uncoordinated, jerky body movements become
more apparent, along with a decline in mental abilities and behavioral and psychiatric problems.
Physical abilities are gradually impeded until coordinated movement becomes very difficult. Mental
abilities generally decline into dementia. HD, as a rule, terminates fatally 10-20 years after clinical
onset.
Diagnosis: Medical diagnosis of the onset of HD can be made following the appearance of physical
symptoms specific to the disease. Genetic testing can be used to confirm a physical diagnosis if there is
no family history of HD. MRl can show atrophy of the caudate nuclei early in the disease, but these
changes are not diagnostic of HD. Cerebral atrophy can be seen in the advanced stages of the disease.
Treatment. There is no cure for Huntington's disease, but there are treatments available to reduce the
severity of some of its symptoms. The drugs that help to reduce chorea include neuroleptics and
benzodiazepines. Selective serotonin reuptake inhibitors have been recommended for depression, while
atypical antipsychotic drugs are recommended for psychosis and behavioial problems.
18. Hereditary diseases of neuromuscular apparatus. Myasthenia arid myasthenic syndromes... | 143
Hepatolenticular degeneration (Wilson - Konovalov disease)
An autosomal recessive disorder, which is characterized by the accumulation of intracellular copper
with hepatic and neurological consequences.
Pathogenesis. This disease is connected with disorders of ceruloplasmin (copper-bearing protein)
metabolism. It is produced in the liver. Pathologically there is accumulation of copper in the basal
ganglions, brain cortex, cerebellum, liver, kidney, iris.
Clinical features: The first sings of the disease are observed in childhood. There are behavioral
changes, involuntary movements (different hyperkinesis), liver involvement common. Sometimes
seizures can be observed. One of the most specific changes is Kayser — Fleischer ring. It is a dark ring
that appears to encircle the iris of the eye due to copper deposition.
According to the Konovalov classification there are 4 clinical forms of the disease:
► abclomninal — the main signs are impairments of the liver and kidneys;
► arrhythmo-hyperkineticiorm is manifested at the age of 5-15. Arhythmi- cal hyperkinesias are
typical, usually of a torsal-distonic character or atethosis in extremities, body, muscles of the throat
and larynx, which leads to dysarthria and dysphagia;
► trembling-rigid — both rigidity and trembling are typical, dysphagia, dysarthria are frequent;
► extrapyramidal-cortical—the main symptoms are cognitive dysfunction and hyperkinesis.
Diagnosis. The disease may be suspected on the basis of any of the symptoms mentioned above, or
when a close relative has been found to have hepatolenticular degeneration. Most patients have slightly
abnormal liver function tests such as a raised aspartate transaminase, alanine transaminase and bilirubin
level. If the liver damage is significant, albumin may be decreased due to an inability of damaged liver
cells to produce this protein and the prothrombin time may be prolonged.
MRI shows hyperintensities in the basal ganglia in the T2 imaging and may also demonstrate the
characteristic "face of the giant panda" pattern. This one is apparently caused by a high signal in the
tegmentum, normal signals in the red nuclei and lateral portion of the pars reticulata of the substantia
nigra, and hypointensity of the superior colliculus. It is most associated with Wilson's disease, but
obviously can appear in other disorders as well.
There is no totally reliable test for Wilson's disease, but levels of cerulo- plasmin and copper in the
blood, as well of the amount of copper excreted in urine during a 24-hour period, are together used to
form an impression of the amount of copper in the body. The most ideal test is a liver biopsy. Mutation
analysis of the ATP7Bgene, as well as other genes linked to copper accumulation in the liver, may be
performed.
Treatment. Low copper diet and a chelating agent, penicillamine (cuprenil) 1-1.5 g daily, is the first
treatment used. This binds copper and leads to excretion of copper in the urine. Once all results have
returned to normal, zinc acetate may be used instead of chelators to maintain stable copper levels in the
body. Zinc stimulates metallothionein, a protein in gut cells that binds copper and prevents their
absorption and transport to the liver. Zinc therapy is continued unless symptoms recur, or if the urinary
excretion of copper increases.

Hereditary diseases with lesion of the coorriinative system. Hereditary Ataxias

The degenerative ataxias comprise a wide spectrum of disorders with ataxia as the prominent
symptom. The neuropathological hallmark of these diseases is degeneration of the cerebellar cortex and
of the spinal pathways. Hereditary ataxias are divided into ataxias with autosomal recessive inheritance
and ataxias witli autosomal dominant inheritance, which are now usually named spinocerebellar ataxias
(SCA). In recent years, the causative mutations of most hereditary ataxias have been identified, which
allows to classificate them. The two most prevalent types of hereditary ataxia are Friedreich's ataxia
(recessive) and Marie's ataxia (dominant).

Friedreich's ataxia
144 (FA)
І РАПТ 2: Special Neurology

Prevalence rates of FA range from 0.4 to 4.7 per 100.000. As in other recessive disorders, birth
from a consanguineous marriage is a major risk factor for FA. In families with one affected child,
each of the remaining children carries a risk of 25 percent to develop FA.
It is caused by an unstable GAA trinucleotide repeat expansion (> 120 repeats) in the first intern of
the frataxin gene on chromosome 9 (9q13) in both alleles. Patients have low mitochondrial protein
frataxin rriRNA levels suggesting that it is the primary cause of neurodegeneration and cardiomy-
opathy in FA.
The neuropathological abnormalities include degeneration of spinal tracts (the spinocerebellar
tracts, posterior columns, pyramid tract), axonal sensory and motor neuropathy, and hypertrophic
cardiomyopathy. There is only occasional involvement of the cerebellum with a loss of Purkinje cells
and moderate cerebellar atrophy.
Clinical features. FA usually starts before the age of 25 years, most often between ages 10 and 15
years. In most patients, ataxia of gait and posture is the first manifestation of the disease. Clinical
symptoms include progressive ataxia and impaired vibration or position sense, areflexia of lower
extremities extensor plantar responses. There is progressive weakness of the extremities, which is due
to pyramidal tract dysfunction and to distal muscle atrophy. In the course of the disease, all FA
patients develop dysarthria characterized by slow, hesitating, and scanning speech. FA is associated
with a number of non-neurological symptoms that include skeletal deformities, such as scoliosis and
pes cavus, and hypertrophic obstructive cardiomyopathy. Ten percent of the patients have diabetes
mellitus. In a few cases, spontaneous remissions may occur which can last for five to ten years or
sometimes longer;

Diagnosis. In most cases FA can be clearly distinguished from other disorders by its highly
characteristic clinical phenotype. There are typical diagnostic criteria:

a) progressive ataxia with an onset before the age of 25 years,

b) lower limb areflexia,
c) decrease of proprioceptive or vibration sense in lower limbs,
d) dysarthria within 5 years after onset of ataxia.

Electrocardiography and echocardiography are useful to study cardiac involvement in FA. A

molecular diagnosis of FA can be made by demonstration of homozygosity for the GAA repeat
expansion by polymerase chain reaction.
Treatment. Although no specific treatment can stop the progression of this disorder, some
symptoms can be alleviated with proper treatment. Antiataxic drugs such as 5-hydroxytryptophan,
buspirone, and amantadine may be partly effective in FA. Physiotherapy and speech therapy are
generally recommended. Patients with clinically relevant cardiomyopathy and diabetes mellitus should
receive standard medical treatment.

Spinocerebellar Ataxias
The SCA are a genetically heterogeneous group of autosomal dominant progressive ataxia disorders.
Up to now, 15 different gene loci have been found in association with SCA, which count 31 types. The
most frequent ataxia type I is developed with motor impairment (pyramidal), optic atrophy, oculomotor
paralysis, intelligence disorders.

Pierre Marie's disease

In 1893, Pierre Marie described a clinical condition that he termed "hereditary cerebellar ataxia," in
which cerebellar signs prevailed along with spastic signs. This was later referred to as Pierre Marie's
disease or hereditary cerebellar ataxia. The syndrome usually begins between thirty and forty years of
age and may not be as disabling as Friedreich's Ataxia.
Initially, those affected may walk unsteadily and tend to fall frequently. A loss of coordination in the
arms and speech disturbances
18. Hereditary is developed.
diseases of neuromuscular In later
apparatus. Myasthenia aridstages
myasthenicasyndromes...
slight loss| 145 of vision, and a loss of pain or
touch sensations, may also occur. Cognitive decline, depression, brainstem oculomotor problems,
deafness, and urinary complaints may be present. The nonataxic symptoms include pyramidal signs
such as spasticity, hyperreflexia, and the extensor plantar reflex. At the same time, Pierre Marie's
disease is characterized by the absence of .deep sensibility and cardiac disorders, as well as deformities
(scoliosis, pes cavus).
 Diagnosis. In addition to typical clinical picture, there are genetic tests available for some types of
SCA. The diagnosis of SCA types I, II, and III can be confirmed with a standard blood test. Genetic
testing may be developed for other types of SCA.

Tests and typical tasks:

1. What is the feature of Duchenne primary muscle dystrophy?
a) central paresis of the extremities
b) sural muscle pseudohypertrophy
c) pelvic dysfunction
d) muscle fibrillation
e) the dissociated type of sensitive disorders

2. Indicate additional diagnostic methods for patients with myopathy?

a) brain CT and MRl scanning
b) electroencephalography
c) electromyography
d) analysis of copper concentration in the blood
e) myelography

3. What are the features of Huntington disease?

a) muscle hypertone, aphasia
b) peripheral paresis of the extremities, bulbar syndrom
c) ataxia, central limb paresis
d) hyperkinetic syndrome, muscle hypotonus
e) bulbar syndrome, sensitive disorders

4. Choose symptoms of hepatolenticular disease

a) hyperkinesias, Kayser - Fleischer ring
Iі) muscle hypertone, aphasia
c) bulbar syndrome, sensitive disorders
d) hemiplegia-sensitive disorders
e) disk edema of optic nerves, monoparesis

5. What are the myastenia signs?

a) peripheral paresis of the extremities, ataxia
b) muscle fibrillation, sensitive disorders
c) muscle fatigue, weakness
d) hemiplegia, sensitive disorders •
e) pelvic dysfunction, peripheral limb paresis
6.
A 4-year-old boy presents with progressive difficulty walking and climbing stairs. Progressive
muscular dystrophy of Duchenne type was diagnosed What additional tests can prove the diagnosis?
7 An ophthalmologist found Kayser - Fleischer ring of the green-brownish colour in a patient with
hyperkinesias, muscle hypotone and cognitive impairments. What
is the previous diagnosis?
8. A40-years-old man has swinging hyperkinesias together with a decreased muscle tonus and
| List of answers
dementia. He146
fell ill at the age of 30. This brother has the same clinical symptoms. What is the clinical
diagnosis?
9.A young woman of the asthenic type suffers from fast muscle fatigue, disability to walk long
distances. She has problems with swallowing food. The state of the patient gets better after proserin
injection .What is the previous diagnosis?
10. A 18-years-old patient has lower spastic paraparesis with Babinski's and Rosso- limo's signs, foot
clonuses, spastic gait, tendon contractions. Abdominal reflexes are preserved. The function of the
 pelvic organs is not Impaired. Mental power is preserved. What is the clinical diagnosis? What is the
course of the disease?
LIST OF ANSWERS

PART II. Special Neurology

Chapter 10. Infectious diseases of the central nervous system.
Meningitis. Encephalitis. Myelitis. Acute poliomyelitis. Neurosyphilis
1. d. 2. b. 3. a. 4. e. 5. d. 6. Secondary purulent meningitis. 7. Meningococcal meningitis. 8. Tick-borne
encephalitis. Poliomyelitic form. 9. IMeuro-AIDS, Toxo- pasmosis encephalitis. MRl of the brain. 10.
Chorea minor.

Chapter 11. Cerebral vascular diseases. Slowly progressing and transient disturbances of cerebral
blood circulation. Brain stroke
1. a. 2. d. 3. c. 4. e. 5. a, 6. Subarachnoid hemorrhage. Spinal pucture.
7. Transient ischemic attacks in the left middle cerebral artery. 8. Slowly progressing disturbances of
cerebral blood circulation. 9. Ischemic stroke in the vertebro-basilar basin with Wallenberg —
Zakharchenko syndrome. 10. Hemorrhagic stroke.

Chapter 12. Headache. Kinds and nosological forms of a headache. Migraine

1. d. 2. a. 3. b. 4. c. 5. a. 6. Ophthalmic migraine. 7. Liquor-dynamic headache.
8. Ophthalmoplegic migraine. 9. Cluster headache. 10. Tension headache.

Chapter 13. Closed craniocerebral and spinal trauma

1. Сі 2. a. 3. e. 4. b. 5. e. 6. Brain concussion. 7. Epidural hematoma. 8. Brain contusion. 9. Lumbar
punction. 10. Subarachnoid hemorrhage.

Chapter 14. Brain and spinal cord tumors. Syringomyelia

1. а. 2. с. 3. d. 4. b. 5. е. 6. Tumor of the right cerebellum hemisphere. Disk edema of optic nerves. 7.

Extramedullar tumor of the spinal cord at right Th7-Th8 segments. 8. Tumor of the upper pait of the left
precentral gyrus. Brain CT or MRI. 9. Extramedullary spinal cord tumor or radix neurinoma. 10.
Syringomyelia. MRI of the spinal cord. •

Chapter 15. Epilepsy. Nonepileptic paroxysmal states

1. c. 2. b. 3. e. 4. a. 5. d. 6. Occipital zone: the medial surface cortex. Partial epileptic seizure with
secondary generalization. 7. The upper part of the right precentral sulcus. Simple partial motor seizure
with Jackson's marsh. 8. Partial epileptic seizure with psychic symptoms. 9. Status epilepticus. 10. The
absence form of epilepsy.

Chapter 16. Diseases of the peripheral nervous system.

Vertebrogenic affections of the peripheral nervous system. Mononeuropathies and
polyneuropathies
1. a. 2. c. 3. d. 4. e. 5. b. 6. Traumatic neuropathy of the right radial nerve. 7. Neuralgia of the II branch
of the left trigeminal nerve.of neuromuscular
18. Hereditary diseases 8. Left Dejerine-Klumpke paralysis.
apparatus. Myasthenia arid myasthenic 9.| 147
syndromes... Acute herpetic ganglionitis of the
right trigeminal ganglion. 10. Cer- vicalgia.

Chapter 17. Demyelinating diseases of the nervous system.

Multiple sclerosis. Acute disseminated encephalomyelitis. Amyotrophic lateral sclerosis
1. a. 2. c. 3. a. 4. b. 5. d. 6. The pyramidal tracts and posterior funiculi are affected. Multiple sclerosis.
7. Acute disseminated encephalomyelitis. 8. Pyramidal tracts and the anterior horns of the spinal cord.
Amyotrophic lateral sclerosis. 9. The pyramidal tracts, cerebellum, optic, abducens nerves, posterior
funiculi of the spinal cord are affected. Relapsing-remitting multiple sclerosis. 10. Acute disseminated
encephalomyelitis. Mfil of the brain, examination of the cerebrospinal fluid.
Chapter 18. Hereditary diseases of neuromuscular apparatus. Myasthenia and myasthenic
syndromes. Hereditary diseases with lesion of the pyramidal, extrapyramidal
and coordinative systems
1. b. 2. c. 3. d. 4. a. 5. c. 6. The serum creatine kinase concentration is elevated. Electromyography
and muscle biopsy. 7. Hepatolenticular disease. 8. Huntington's chorea. 9. Myastenia. 10. Striimpell's
familial spastic paraplegias. Slowly progressing.

148 | List of answers

149 I Index