Extrapyramidal system and

cerebellum

Matej Skorvanek MD
Dept. of Neurology
Safarik University in Kosice
Extrapyramidal system - anatomy
 Ncl. caudatus
 Ncl. lentiformis
(putamen + gl.
pallidus)
 Corpus striatum -
ncl. caudatus a
putamen
 Ncl. Accumbens
 Ncl. Basalis
Meynerti
 Ncl. Subthalamicus
 Substantia nigra –
pars compacta /
pars reticularis
 Ncl. ruber
 Pedunculopontine
nucleus
Extrapyramidal syndromes

• Hypokinetic syndrome

• Hyperkinetic syndrome
 Hypokinetic syndrome
• Bradykinesia – pathological slowness of movements

• Hypokinesia – lower amplitude of movements

• Akinesia – problem with movement initiation

• Rigidity – increased muscle toneýšenie svalového

tonusu (compared to spasticity resistance is the same
during whole range of passive movement – „lead
pipe phenomenon / cogwheel phenomenon“)
Parkinsonian syndrome
• Bradykinesia (hypokinesia, akinesia) + at least
one of the following:
▫ Rigidity
▫ Resting tremor
Parkinsonian syndrome - aetiology
 Parkinson‘s disease 80%
 Neurodegenerative disorders 10%
 Atypical parkinsonism
 Progressive supranuclear palsy
 Corticobasal degeneration
 Multiple system atrophy
 Dementia with Lewy Bodies
 Secondary 10%
 Drug-induced, vascular, toxic, posttraumatic,
postencephalitic
Hyperkinetic syndrome

• Tremor
• Chorea
• Balismus
• Dystonia
• Myoclonus
• Tics
Tremor
• Rytmical oscillation of a body part around a joint

• Alternating contractions of agonist and

antagonist

• Dif.dg. – rytmical myoclonus, dystonic tremor

 Classification
• According to body part affected – most frequently arms
• According to position where tremor is observed
▫ Resting (parkinsonian)
▫ Postural (essential, physiological, drug-induced)
▫ Kinetic/intentional (cerebellar)

• According to frequency
• According to amplitude
 History
1. Anatomical localization?
2. Task-specific?
3. Age of onset?
4. Disease course? (progressive – PD, static – ET,
sudden onset and/or sudden remission – functional?)
5. Factors that improve or worsen tremor? – alcohol,
fatigue, anxiety, stress, mental tasks, ...
6. Drug history
7. Bradykinesia/rigidity in history
8. Metabolic diseases? – hyperthyroidism,...
9. Family history
Clinical examination
• Anatomical location – hands, arms, head, chin, legs,
trunk
• Symetrical/asymetrical
• Position – resting, postural, kinetic, intentional
(finger-nose-finger)
• Frequency (low/high), amplitude (big/small)
• Activation maneuvers
• Other extrapyramidal signs – rigidity, dystonia, ...
• Oculomotor abnormalities
• Signs of neuropathy
• Gait abnormalities
Activation maneuvers
• Cognitive task – naming months backwards,
counting (increase of supressed organic tremor/
decrease or character change of functional)
• Archimedes spiral
• Drinking a cup of water
• Writing
Patophysiology of tremor

Helmich 2013
Chorea
• Disorder characterised by involuntary brief jerky or
twisting movements, which appear at random in the
affected body part

• Important is the random nature and unpredictability

of the following movement.

• Movements are brief and brisk, but can be also longer

and more twisting (choreoathetosis), they are most
pronounced at distal body segments
Chorea
• Worsening during speech, movements, emotions
• E.g. „milking maid handshake“, can‘t keep the
tounge protruded, dancing gait (st. Vitus dance)
• Distribution
▫ Focal
▫ Segmental
▫ Hemichorea
▫ Generalized
Chorea - aetiology
• Neurodegenerative – Huntington‘s disease
• Metabolic, toxic and drug-induced (hepatal
encephalopathy, CO poisoning, Mn, tardive
dyskinesias after neuroleptics,...)
• Secondary due to BG lessions (trauma,
stroke, tumors)
• Autoimunne (assoc. with antibodies against
streptococcus – chorea minor assoc. with
rheumatic fever, chorea gravidarum)
Ballism
• Variant of chorea with large amplitude
involuntary movements of proximal parts of the
limbs
• Usually due to lesions of ncl.subthalamicus Luysi
or putamen
• Most frequently hemiballism after contralateral
lesion of the subthalamic nucleus
 Dystonia
• Dystonia is defined as a movement disorder
characterized by sustained or intermittent muscle
contractions causing abnormal, often repetitive,
movements, postures, or both.

• Dystonic movements are typically patterned and

twisting, and may be tremulous.

• Dystonia is often initiated or worsened by voluntary

action and associated with overflow muscle
activation.

Albanese et al. 2013

Dystonia
• Fixed dystonia
• Mobile dystonia
• Dystonic myoclonus
• Dystonic tremor

• Dif.dg. Of dystonie
▫ Chorea
▫ Myoclonus
▫ Tics
▫ Stereotypies
Axis I: clinical characteristics Axis II: Etiology
I. Age at onset I. Nervous system pathology
I. Infancy (birth to 2 years) I. Evidence of degeneration
II. Childhood (3–12 years)
III. Adolescence (13–20 years) II. Evidence of structural (often
IV. Early adulthood (21–40 years) static) lesion
V. Late adulthood (>40 years) III. No evidence of degeneration or
II. Body distribution structural lesion
I. Focal II. Inherited or acquired
II. Segmental
III. Multifocal I. Inherited
IV. Generalized (with or without leg I. AD, AR, X-linked, mitochondrial
involvement) II. Acquired
V. Hemidystonia
I. Perinatal brain injury
III. Temporal pattern
I. Disease course II. Infection
I. Static III. Drug-induced
II. Progressive IV. Toxic
II. Variability V. Vascular
I. Persistent VI. Neoplastic
II. Action-specific
VII. Posttraumatic
III. Diurnal
IV. Paroxysmal VIII.Psychogenic
IV. Associated features III. Idiopathic
I. Isolated dystonia or combined with I. Sporadic
another movement disorder II. Familial
II. Occurence of other neurological or
systemic manifestations Albanese et al. 2013
 Clinical characteristics
• Body distribution
▫ Focal – only 1 muscle group or body segment
▫ Segmental – 2 neighbouring muscle groups or body
segments (e.g. head + neck)
▫ Multifocal – 2 non-neighbouring muscle groups or body
segments (e.g. right arm + left leg)
▫ Hemidystonia
▫ Generalized – affection of the trunk + at least 2 other
body segments – with or without leg involvement
Age of onset
I. Infancy (<2 years)

II. Childhood (3-12 years)

III.Adolescence (13-20 years)

IV.Early adulthood (21-40 years)

V. Late adulthood (>40 years)

Temporal pattern
I. Disease course
I. Static
II. Progressive
II. Variability
I. Persistent symptoms
II. Action-specific
III.Diurnal fluctuations
IV. Paroxysmal dystonia
Associated features

I. Dystonia isolated or combined with other

extrapyramidal symptom

I. Isolated dystonia

II. Combined dystonia

II. Other neurological or systemic symptoms

Myoclonus
• Very brief involuntary jerky movements caused by
intermitent muscle contractions or relaxations

• Regular / irregular

• Positive / negative (asterixis)

• Focal, segmental, multifocal, generalized

• Stimulus sensitive to sound, touch?

• Cortical / subcortical / spinal

• Dif.dg. Tremor, chorea, epilepsy, fasciculations, psychogenic

 Tics
 Fast (clonic) or slow (tonic) irregular repetitive stereotyped
movements or vocalizations
 Preceded by growing inner tension and urge to perform the
tic and followed be relief after performing the tic
 Partially voluntarily supressible
 Simple motor tics
 Complex motor tics
 Simple vocal tics
 Complex motor tics
Cerebellar anatomy
• Archicerebellum
▫ Oldest part
▫ Flocculonodular lobe
▫ Balance, eye movements
• Palleocerebellum
▫ Ant. a post. part of vermis
▫ Modulates sequential movements
• Neocerebellum
▫ Youngest part
▫ Hemispheres and central part of vermis
▫ Fine motor and speech control
Clinical features of cerebellar dysfunction
• Ataxia (appendicular/axial)
▫ Dysmetria (hyper) – disturbance of aiming
▫ Dysdiadochokinesis – disturbance of fast
alternating movements
▫ Dyssynergy – loss of coordination between
involved muscle groups
• Rebound
• Failure/disturbance of inhibition
Clinical features of cerebellar dysfunction
• Tremor (kinetic/intentional)
• Titubations and oscillations around the axis on standing
• Hypotonia
• Dysarthria, Ataxic (scanning) speech
• Dysphagia
• Ocular movement abnormalities (nystagmus, slow saccades, saccadic
dysmetria, occular flutter, opsoclonus, skew deviation)
• Cognition – slow thinking, problems in motor learning
• Palleocerebellar syndrome
▫ Ataxia of stance and gait (archicerebellum)
▫ Trunk assynergz (axial muscles)
▫ Falls (frequent, usually backwards)
• Neocerebellar syndrome
▫ Hypermetria
▫ Adiadochokinesis
▫ Assynergy of fine movements
▫ Intentional tremor
▫ Pasivity (lower muscle tone)