Lecture:

“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: -, and-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
Pathways of Extrapyramidal system:
The afferent pathways of The efferent pathways of
Extrapyramidal Extrapyramidal
system: system:

• T – EPNS – T • Tr. olivospinalis

• T – cortex – EPNS – T • Tr. rubrospinalis
• T – cortex - pons – • Tr. vestibulospinalis
cerebellum – • Tr. tectospinalis
thalamus
• Tr. reticulospinalis
 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.
Functions of Extrapyramidal system

• It prepares muscles to smooth economical

movements
• It determines the posture
• It makes automatical involuntary regulation of
active 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 to the modern
investigations- in base of this disease
is inborn dificiency of
tyrosintransferase enzyme which
provides transformashion of tirosin 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)
• 1.The gait and posture are affected
• 2.A lack of mobility
• 3.The head and the shoulders are stooped
forward
• 4.The armes are slightly abducted
• 5.The forearmes are flexed
• 6.Poverty of movements
 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
tonus:
• 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
 Other features of Parkinson’s 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
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;
 Chorea
• 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.
 Chorea
• 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
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
 Functions
• Vermix functions are:
• Equilibrium of the body
• Regulation of muscle tone

• Hemispheres functions 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(unsteadiness)
• Disorders of equlibrium – patient can’t to
stand
• Muscular hypotonia

• Nystagmus: horizontal, vertical, vertical

rotary, or dissociated (different in each eye)
 Cerebellar ataxia
• Intention tremor – is dynamic tremor (it is
more expressed while moving and
disappears while rest)
• 1.Finger-nose test
• 2.Heel to knee test
• 3.Finger to finger tesf
• 4.Index test
 Cerebellar ataxia
• 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.
 Symptoms of lesion
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
 Symptoms of lesion
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
 Thanks
for your attention