Extrapyramidal syndrome

(movement disorders)

Dr. Zuraini, Sp.S

Definition
Neurologic syndromes in which
abnormal movement occur due
to a disturbance of fluency and
speed of voluntary movement
or the presence of unintended
extra movements

Ektrapyramidal System

A GROUP STRUCTURAL OF Gray matter

wich located in hemispher cerebri and
mainly functional was motor activity

CONSIST OF:

Caudate nucleus

1.Basal Ganglia

Putamen

2.Brain stem

Globus palidus

3.Cortek serebri

Nucleus caudatus + Putamen

Corpus striatum (neostriatum)
Putamen + Globus palidus
(nucleus lenticularis)

BRAINSTEM

Subthalmicus nuclei
Substantia Nigra
Parts of formatio reticularis

CORTEKS CEREBRI

Area 4 S
Area 6
Area 8
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Ekstra Pyramidal Circuit

1.
2.
3.
4.

Cortex Striatum Globus pallidus

Thalamus Cortex
Cortex Striatum Substantia nigra
Striatum Cortex
Cortex Striatum Substantia nigra
Thalamus Cortex
Cortex Globus pallidus Sub
thalamic nuclei Thalamus Cortex

Pathogenesis 1

Results from dysfunction of the

extrapyramidal system
Basal ganglion caudate, putamen, globus
pallidus, subthalamic nucleus, and
substantia nigra
motor area of cortex basal
ganglion(organizing movement commands)
motor area of cortex
affects the size and speed of movements
selection of components of movements or the
sequencing of multi-step movements
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Pathogenesis 2

Movement disorder
Motor network
Upper motor neurons
Lower motor neurons
Cerebellar circuitry
Basal ganglia
circuitry
Motor association
cortex
Sensory systems

Subtthalamicus
nuclei

Serebellum
Thalamus

Cortex

Globus pal

Substantia
nigra

Caudatus+put

Pons

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striatum
Nigro reticulo spinal tract

Diagram of ekstrapyramidal circuit

Piramidal tract
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THE MAIN FUNGTION OF

EPS ASCOCIATED WITH
Ascociative movement
Regulation of posture
Autonomic integration

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EKSTRAPYRAMIDAL
SISTEM DISTURBANCES

Ektrapyramidal syndroma = Basal

Ganglia syndroma
1.Primary functional deficit
negative symptom
2.Secundary efect/ release pheno
mena positive symptom

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EPS SYNDROMA

Negative symptom
1. Bradikinesia
2. Disturbances
posture

Positive symptom

1.Involuntary
movement tremor,
athetosis, ballismus,
chorea, dystonia
2. Rigiditas
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Types of hyperkinetic movement

disorders

dancelike

Slow,distal

The differential diagnosis of chorea

Practical Neurology 2007;7;360-373

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TREMOR
A Rythmic Movement 3 to 5/Sec,
resting, mainly in fingers, arms
and chin

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Chorea:
Arrythmic movement of a forcible, rapid,
jerky type, affecting the fingers, hand, and
entire limb,or some other part of the body.
Chorea may be limited to one of the body
(hemichorea). When the movements involve
the proximal limb muscles and are unusualy
violent and flinging Hemiballismus

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Athetosis :
Generally denotes abnormal
movements that are slow,
sinuous, irregular both in arm
and fingers

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Dystonia:
An abnormal contorted posture,
classically in one or other of the
extremes of athetoid movement, with
a predilection for muscles of the trunk
and limb girdle or a hand or a foot.
Dystonia posture also occure without
an accompanying athetosis.

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Clinicopathologic correlation of Extrapyramidal

Symptom

Location of lesion

Unilateral plastic rigidity with static tremor

contralat subs nigra

Unilateral hemiballismus and hemichorea

contralat subthal nucleus

of luysial

Chronic chorea of huntington type

caudatus nuc & putamen

Athetosis and dystonia

contralateral striatum

Cerebellar incord,Intention tremor and

homolat cerebel hemisfher

Hypotonia

or midle and inf cerebellar

peduncles

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Drug-induced EPS

EPS secondary to pharmacologic agents are the

most common.
The risk of developing a drug-induced EPS
begins at the onset of treatment with an
offending agent.
Acutely: within hours or a few days
Subacutely: over several weeks
Late or delayed onset: six months or longer
after exposure(tardive)
short-term therapy of minimal therapeutic
dosages should be the strategy employed
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Five classes of drugs are known to

affect central dopaminergic systems

Central stimulants act as indirect dopamine

agonist ex. Amphetamine
Levodopa a precursor of dopamine
Direct dopamine agonist ex. Bromocriptine
Presynaptic dopamine antagonists ex.
Reserpine
Antagonize or block central dopamine
receptors
neuroleptics, metoclopramide primperam
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Manifestation
Acute dystonia
Parkinsonism
Akathisia
Tardive dyskinesia

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Acute dystonia
Definition
long-lasting contraction or spasm of musculature
generally less common than most other
extrapyramidal symptoms
young age and male sex predominant
The pathophysiological mechanism is presently
unknown
Diagnosis: The most common muscle groups
affected are the eyes, jaw, tongue, and neck
trismus, blepharospasm, oculogyric crisis, torticollis,
opisthotonus, laryngeal spasm most dangerous
Treatment
anticholinergic drug. Ex. Benztropine,
diphenhydramine
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Parkinsonism
Pathophysiology: blockade of postsynaptic
dopamine(D2) receptors in the corpus
striatum
Diagnosis three cardinal symptoms

Tremor
Muscle rigidity
Bradikinesia

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Treatment
Lower dose of agent
Switching to a low potency agent
Anticholinergic drugs

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Akathisia
Definition
a sense of motor restlessness in which the patient
feels a constant need to move about.

The most common type of EPS

Pathophysiology: unknown
Diagnosis

tend to have subjective complaints of inner

restlessness most often in legs
They feel that they must move, and this manifests as
frequent changes in posture, crossing and uncrossing
of the legs..
Often associated with severe dysphoria, anxiety, and
irritability
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Treatment
Lower

dose of agent
Switching to a low potency agent
Anticholinergic drugs
beta-adrenergic blockers
benzodiazepine

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Tardive dyskinesia
Definition
a syndrome of abnormal movements following at
least six months and often many years of drug
therapy

Pathophysiology
denervation-hypersensitivity phenomenon
It appears with prolonged receptor blockade, the
receptors rebound, becoming supersensitized

Diagnosis
Characterized by involuntary movement of the
lips, tongue, jaw, and extremities
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Lipsmacking, facial and lingual

masticatory movements, trunk
rocking and restless foot movements
Reduced by voluntary movements of
the affected areas
Increased by voluntary movements
of unaffected areas
Increased with emotional arousal
Absent when the individual is asleep
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Treatment
The best treatment is prevention
gradual reduction
Low dose of benzodiazepine
Dopamine antagonist
Dopamine depleting agents

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Movement Disorders

Parkinsons Disease - Hypokinetic

Defined as a syndrome consisting of

variable combination of tremor, rigidity,

bradykinesia, and characteristic
disturbance of gait and posture
Onset: mid-late life; mean age is 57 yrs

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 Epidemiology:
Affects all ethnicities
has equal M/F distribution
occurs 1-2 per 1,000 people in
general population
occurs 1 per 100 people that are over
65 yrs
4th most common disease in the
elderly
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Cause: unknown
Pathophysiology:
1. Loss of dopaminergic cells in the substantia
nigra

Dopamines normal function

2. Over excitation of the caudate & putamen

3. Over excitation of the corticospinal tracts
4. Decrease in thalamic excitation of the
motor cortex

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 Four Hallmark Signs

Resting Tremor (Pill-Rolling)

Rigidity
Bradykinesia
Flexed Posture with shuffling gait
(Festinating)

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Movement Disorders

Huntingtons Disease Hyperkinetic

A neurodegenerative disorder which

predominately has behavioral, cognitive,
or movement disorders signs
Onset: Usually begins during adult life

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 Cause: Autosomal Dominant Disorder

Pathophysiology:

Mutation on chromosome 4
Uncertainty?

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 Pathological Changes
Atrophy & neuronal
degeneration of cortex
Hallmark: caudate
atrophy

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 Examination:
Physical Findings
Initial Findings
Gradual onset
Slowed saccadic movements 1st
sign
In 85% chorea is predominate
movement disorder

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 Adult Onset
Prominent chorea
Bradykinesia
Postural reflex compromise
Terminal Phase
Dysarthria, dysphagia, &
respiratory difficulties
General
Cognitive impairment
Depression
Psychiatric disorders

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Movement Disorders

Restless Legs Syndrome Hyperkinetic

Common movement disorder

Diagnostic Criteria
Desire to move limbs which is associated
with unpleasant sensations
Restlessness
Worsening of symptoms @ rest w/
temporary relief w/ movement
Worsening of symptoms @ night

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 Common Descriptions

Always unpleasant, but not

necessarily painful
Need to move
Crawling
Tingling
Itching
Restless

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CEREBELLUM
Located in posterior fossa behind
pons/med oblongata
Consists of vermis on medial part and 2
hemispher, with 3 anatomical component
1.Flocculonodularis lob = archicerebellum
2.Anterior lob = paleocerebelum
3.Posterior lob = neocerebellum

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Archicerebellum receive afferents fibre from

vestibuer system maintain of equilibrium

Paleocerebellum receive aferents fibre from

spinocerebellaris tract gait maintenance

Neocerebellum receive aferents fibre and send

eferents fibre from/to motor cortek/vestibuler
nuclei, basal ganglia and pons maintain postural
tonus and modulation motor skill.

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Pedunculus serebelli
Tree pairs, located on top and around
forth ventricle, make the cerebellum
attach to brain stem. Contain of tracts
to and from brain stem

Pedunculus cerebelli inferior

contain the fibre from med spin,
low part of brain stem, nuclei/
vestibuler nerve
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Pedunculus cerebelli medialis

(fibre from pontin nuclei contra lateral)

Pedunculus cerebelli superior

(eferents fibre thalamus / med
spinalis )

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CEREBELLUM FUNCTION

Regulation and control of muscle

tone
Coordination of movement, mainly
skill movement
Control posture and gait

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CEREBELLUM
DYSFUNGTION

Reduction muscle tone, mainly in

acute lesion
Disturb of coordination voluntary
movement ataxia
Disturb of equilibrium and gait
Tremor that derives from ataxia and
hipotonia

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Because the efferent cerebellar pathway

To the cerebral hemispheres are crossed
And the corticospinal system is again
Crossed, a unilateral lesion of the
Cerebellum causes an ipsilateral
Disorder of movement

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Hipotonia
Decreased of muscle resisten on

palpation or fasive movement

(abnormality of activity gamma and alfa
motor neuron)

Disorders of coordination Ataxia

dismetria
disdiadokinesia
intention tremor
Disarthria
Dyssynergy
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THANK YOU !
Dopamine
GABA

Serotonin
Cholinergic

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