Extrapyramidal

system and
Cerebellum
Extrapyramidal system consists of:
 cortical areas 4, 6, 8
 the basal ganglia: n. caudatus, n.lenticularis
(putamen, globus palidus)
 the nuclei of brain stem (black substance, red
nucleus, vestibular nuclei, reticular nuclei,
nucleus of Darkshevych, Lues’ body, lower
olives)
 spinal cord: -motor neurons and -small
motor neurons, which are located in anterior
horns of the spinal cord
 Two parts of Extrapyramidal
system:
Pallidum
globus palidus
black substance
red nuclei
vestibular nuclei
nucleus of Darkshevych
lower olives
Lues body

 Pallidum is phylogenetically older then striatum.

That’s why in newborn babies pallidum dominates
 Two parts of Extrapyramidal
system:
Striatum
cortical areas 4,6,8
n. Caudatus
Putamen

Only at the age of 4 – 5 months old striatum

starts to influence on motor functions
Connections of Extrapyramidal system:

Pallidum and striatum are closely connected

with each other by means of such pathways:
 nigrostriatal (dopaminergic) – black
substance – nucleus caudated – it inhibits the
neurons of striatum
 strionigral (GABA-ergic)- nucleus caudated
(GABA) – black substance – it controls
production of dopamine.
The afferent pathways The efferent pathways
of Extrapyramidal of Extrapyramidal
system: system:

 T – EPNS – T  Tr. olivospinalis

 T – cortex – EPNS – T  Tr. rubrospinalis
 T – cortex - pons –  Tr. vestibulospinalis
cerebellum – thalamus  Tr. tectospinalis
 Tr. reticulospinalis
Functions of Extrapyramidal system
 It prepares muscles to smooth economical
movements
 It determines the posture
 It makes automatical involuntary regulation of
active conscious movements
 It provides automatical stereotyped
movements and reflector protective
movements
 It provides motor manifestation of emotions
Extrapyramidal system’s lesion:

Parkinson’s syndrome
(lesion of pallidum)
Syndrome of involuntary
movements (lesion of
striatum)
 Parkinson’s disease
 In 1817 – James Parkinson described the
major manifestation of this syndrome
 In 1874 – this disease was called after
James Parkinson – Parkinson’s disease
 In 1920 – Tretiakov noticed that the greater
cell loss in substantia nigra, the lower
concentration of dopamine is in striatum
and more severe the degree of clinical
Parkinsonism.
 Etiology
 According the modern investigations in
base of this disease is inborn dificiency
of tyrosintransferase enzyme in
dopamine.
 Parkinsonism may be defined in
biochemical term as an inborn
dopamine deficiency state.
 Basic symptoms of Parkinson’s
syndrome:

 Hypokinesia (Akinesia)

 Rigidity

 Tremor
The main pathogenetic mechanisms:
 Great cell loss in the substantia nigra,
low concentration of dopamine in
striatum, the influence of striatum on
pallidum. As a result akinesia occurs.

 The main cause of rigidity is increasing

of tonic reflex on muscles tension.

 The main source of tremor is thalamus (

its nucleus ventrolateralis ).
 Hypokinesia (Akinesia)
 Bradykinetic and akinetic state
 The gait is shuffling and the steps are slight
 Parallel footprints
 The loss of associated swinging of the arm
or arms when walking - (acheirokynesis)
 A lack of mobility of facial expression
(Bechterev’s symptome)
 Infrequent blinking of the eyelids (Mary’ s
symptome)
 Fixed look
 Hypokinesia (Akinesia)
 Inertia of rest (that means it is very
difficult for patient to start moving)
 Inertia of movement (the so-called
propulsive gait, lateropusion or
retropulsion)
 Micrography – handwriting is too small
 Speach is quite and inexpressive
(bradylalia)
 Paradoxical kynesia is possible after
strong impression or great emotions
 Rigidity
plastic type of increased muscle tone:

 Cogged-wheel symptom (in

carporadial joint)
 Tonus increases in course of
evaluation of nervous system state
 Tonus is expressed in the same
manner in the group of flexors and
extensors
 Tremor:
 Are much more expressed in distal
parts of extremities, sometimes tremor
of lips or lower jaw can occur
 It looks like coins counting
 It is much more expressed while resting.
It disappears or decreases while moving
 Its frequency is 3 – 6 times per second
 Other features of Parkinson’s disease
 Bradyphrenia (thoughts are too slow)
 Bradymnesia (recollection is slow too)
 While speaking such patients are boring (akairia -
Astwatsaturov symptome)
 Usually they are in a bad mood. Depression is very
typical for the patients with Parkinson disease
 Sometimes they have autonomic disorders
 Parasympathetic nervous system dominates in such
patients – they have running saliva (aeriel symptome),
hyperhydrosis, fatty skin and type of hair, bradicardia
and arterial hypotension
 T e s t s:
The symptom of air pillow or
Vartenberg symptom –
 The patient is lying down. One props up his
head a little bit and then quickly takes his
hand out. Normally the head is falling down.
But in patients with Parkinson disease the
head stays in the same position for a while.
 T e s t s:
 Westfahl’s phenomena of paradoxical
muscle constriction – While foot extension it
stays in the same position for a while.
 Hand extension test.
 Test of knee flexion – The patient is lying on
his abdomen; his lower extremities are bended in
knees. In patients with Parkinson disease the legs
are fixed in this position for several minutes.
 Tests:

The symptom of
Noica–Haneva
While evaluations of muscle tone
one asks the patient to raise his
opposite extremity. In patients with
Parkinson disease the tonus
suddenly increases.
Clinical forms of Parkinson disease:

 Rigid (hypokynesia dominates)

 Trembling

 Mixed :
 rigid – trembling or trembling – rigid
 The degree of severity
(according to Petelin):
 The first degree - expressed one or
two main symptoms. The patient
preserves professional and home
activity
 The second degree – The patient is
disabled professionally
 The third degree – The patient cannot
take care of himself
 Symptomatic Parkinsonism
 Craniocerebral trauma ( Muhammad Ally)
 CO, Mn intoxication
 Brain tumor
 Encephalitis (Economo)
 Strokes
 Cerebral atherosclerosis
 Medicinal parkinsonism (reserpinum,
neuroleptics)
 Poisoning of heavy metals
Hyperkynetic–hypotonic syndrome:
The main clinical signs of this syndrome are:
 Muscular hypotonia
 Involuntary movements – hyperkynesis.

Involuntary movements are characterized by:

 the amplitude of the movement
 location of muscle involved
 the rate
 the duration of contraction and the relaxation
 Kinds of hyperkynesis
 Chorea
 Athetosis
 Choreoathetosis
 Ballism and hemiballism
 Myoclonus
 Torsion spasm
 Tics
 Facial cramp
 Tremor
 Chorea
 spontaneous, irregular, purposeless and asymmetric
movements
 they are present at rest and subside during sleep
 eyes and tongue symptom ( or Hersonsky’s symptom ) -
the patients are unable to maintain tongue protrusion for
more than a few seconds;
 Hordon’s II symptom – while checking knee – reflex crus
stays in the position of extension for a while and then
slowly goes down;
 Cherni symptom - pathologic sudden abdomen at breath.
 The most common diseases - Huntington’s chorea
(inherited disease), rheumatic subcortical encephalitis or
chorea Sydenhams (juvenile disease), atherosclerotic
chorea, chorea gravidarum, electric chorea.
 Athetosis
 Movements are slower and more sustained than
choreiform movements
 they affect primarily the distal portion of extremities
 snakelike movement of any combination of flexion,
extension, adduction and abduction in varying
degrees
 They are regularly associated with increased
muscular tone
 It is supposed that athetosis is the result of nucleus
caudated lesion.
Choreoathetosis
 is a term selected to describe movements that are
intermediate between chorea and athetosis.
 Ballism and hemiballism
 Its the more or less continues gross abrupt
contractions of axial and proximal muscles
of the extremities
 In the most cases this movement disorder is
confined to one side of the body
(hemiballism)
 It may be associated with hypotonia
 Torsion spasm

 Twisting or turning movements

 The muscles of trunk and neck are involved
 Sometimes torticollis can occur
 Usually it is the result of putamen lesion
 Myoclonus
 Is a jerking movement of one or more muscle
groups (for example palatine, tongue, pharynx,
larynx, diaphragm and skeletal muscles)
 Usually only one muscle group is involved
 They are synchronous in most of cases and
sometimes they are asynchronous
 Their frequency is about 15 – 18 per minute
 They may be induced by visual, tactile, or auditory
stimuli (stimulus-sensitive myoclonus) or by the
initiation of the voluntary movement (intention
myoclonus).
 Tics
 are an involuntary compulsive stereotyped
movements
 they may be simple or complex
 tics may involve any portion of the body ( they are
most common about the face where they are
manifest as blinking, grinning, smirking, lip licking,
nose wrinkling)
Facial cramp
 is tonic seizure in facial muscles

Tremor
 Is rhythmical jerking of arms, legs or head
 Its frequency is about 4 – 6 per second
 Cerebellum

is a reflexional organ of
coordination of movements,
equilibrium and muscular
tone
 Cerebellum
 Contains :

Two large lateral hemispheres

Flocculo-nodular lobe (vermix)
Three pairs of peduncles.
 Grey matter of cerebellum
 Is presented by cortex of
hemispheres and nuclei of
cerebellum:
 Nucleus fastigii
 Nucleus globosus
 Nucleus emboliformis
 Nucleus dentatus
 Cerebellum peduncles:
The lower peduncles (corpora restiformia)
provide connection with oblong brain and
spinal cord:
 Tr. spinocerebellaris dorsalis (Flexig’s)
 Tr. vestibulocohlearis (from nuclei
vestibularis to nucleus fastigii)
 Tr. olivocerebelaris (from lower olives to
nucleus dentatus)
 Fibre arcuate externe (from nuclei Holl
and Burdach to hemispheres and vermis)
 Cerebellum peduncles:
The middle peduncles (pedunculum
cerebellaris medii) provide
connection with pons
 Tr. pontocerebellaris. They connect
nuclei of pons with the opposite
hemisphere of cerebellum
 Cerebellum peduncles:
The upper peduncles of cerebellum
(pedunculi cerebellaris superior) connect
cerebellum with middle brain. They include
two systems:
 Afferent one – from spinal cord to cerebellum
– tr. spinocerebellaris ventralis (Hover’s)
 Efferent one – from cerebellum to the
structures of extrapyramidal nervous system
– tr. cerebellotegmentalis et tr. dentorubralis.
The way of cerebellum correction
 The first neuron – tr. fronto-temporo –
occipito- pontinus
 The second neuron – tr. pontocerebellaris
(pontino- cerebellaris decussation)
 The third neuron – tr. cerebello – dentatus
 The forth neuron – tr. dentorubralis
(Vernekink’s decussation)
 The fifth neuron – tr. rubrospinalis (Forel’s
decussation)
 The sixth neuron – tr. spinomuscularis
The main functions of cerebellum:

 body equilibrium
 regulation of muscle tone
 coordination of movements
 synergy
  Vermix function are:
 Equilibrium of the body
 Regulation of muscle tone

 Hemispheres function are:

 Coordination of the movements of extremities
 Synergy of the muscle
 Ataxia
 means disturbances of equilibrium of
the body and coordination of
movements.
 Cerebellum lesion produces
cerebellar ataxia:
 Static (lesion of vermix)
 Dynamic (lesion of hemispheres)
 Kinds of ataxias:
 Cerebellar (cerebellum lesion)
 Sensitive (loss of deep joint sense)
 Frontal (cortical) (lesion of Frontal lobe)
 Vestibular (lesion of vestibular apparatus)
 Hysterical (in case of Hysteria)
 Mixed
 Cerebellar ataxia
 Attactic gait – patient can’t to walk
 Disorders of equlibrium – patient can’t to stand
 Muscular hypotonia
 Intention tremor – is dynamic tremor (it is more
expressed while moving and disappears while rest)
 Nystagmus: horizontal, vertical, vertical rotary, or
dissociated (different in each eye)
 Scanning speech –patient can’t to speak slowly
 Macrographia – handwriting is too large
 Dysmetria (disturbed ability to gauge distances)
 Adiadochokinesia (disturbed ability to perform rapid
alternating movements)
 Asynergia – disturbed ability to controls function of
antagonists muscles
 Symptoms of lesion
Cerebellar lower peduncles lesion can cause:
 Cerebellar ataxia
 Bulbar syndrome
 Sometimes pathology of Holl’s and Burdach’s nuclei
is associated with this lesion. Then ataxia is complex
and is called cerebellar – sensitive ataxia.
Cerebellar upper peduncles lesion can cause:
 Cerebellar ataxia at the side of lesion
 Trochlear Nerve lesion
 Midbrain lesion symptoms
 Symptoms of lesion
Nucleus ruber lesion can cause:
 Cerebellar ataxia in the opposite extremities
 Resting tremor
 Webber’s syndrome or paresis of
convergence may occur
Pontino – cerebellar angle lesion manifests as:
 Cerebellar disorders and pathology of VII,
VIII, V, VI pairs of CNs at the side of lesion
 Pyramidal and sensory hemisyndrome on the
opposite side