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
Sindrom neurologis di mana gerakan
abnormal terjadi karena gangguan
kelancaran dan kecepatan gerakan
sukarela atau adanya gerakan ekstra
yang tidak diinginkan
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Ektrapyramidal System
A GROUP STRUCTURAL OF Gray matter
wich located in hemispher cerebri and
mainly functional was motor activity

TERDIRI DARI :

Caudate nucleus

1.Basal Ganglia

Putamen

2.Brain stem

Globus palidus

3.Cortek serebri
KELOMPOK STRUKTURAL DARI
"materi Gray" yang terletak di
"hemispher cerebri" dan terutama
fungsional aktivitas motorik
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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. Cortex Striatum Globus pallidus
Thalamus Cortex
2. Cortex Striatum Substantia nigra
Striatum Cortex
3. Cortex Striatum Substantia nigra
Thalamus Cortex
4. Cortex Globus pallidus Sub
thalamic nuclei Thalamus Cortex
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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

Hasil dari disfungsi sistem

ekstrapiramidal
Basal ganglion: kaudatus,
putamen, globus pallidus, inti
subthalamic, dan substantia
nigra
daerah korteks motorik
ganglion basal
(pengorganisasian perintah
gerakan) korteks motorik
area
mempengaruhi ukuran dan
kecepatan gerakan
pemilihan komponen gerakan
atau urutan gerakan multilangkah
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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
10

THE MAIN FUNGTION OF

EPS ASCOCIATED WITH
FUNGSI UTAMA DARI EPS TERKAIT
DENGAN

Ascociative movement
Regulation of posture
Autonomic integration
asosiatif gerakan
Peraturan postur
integrasi otonom
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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

Positive symptom

1. Bradikinesia 1.Involuntary
2. Disturbances movement tremor,
posture
athetosis, ballismus,
chorea, dystonia
2. Rigiditas

13

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
Gerakan berirama 3 sampai 5/Sec,
beristirahat, terutama di jari, lengan
dan dag

15

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
Chorea:
Arrythmic gerakan jenis, paksa cepat,
dendeng, mempengaruhi jari, tangan,
dan tungkai seluruh atau beberapa
bagian lain dari tubuh.
Chorea mungkin terbatas pada satu
tubuh (hemichorea). Ketika gerakan
melibatkan otot-otot ekstremitas
proksimal dan unusualy kekerasan
dan melemparkan Hemiballismus

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Athetosis :
Generally denotes abnormal
movements that are slow,
sinuous, irregular both in arm
and fingers
Athetosis:
Umumnya menunjukkan gerakan
abnormal yang lambat, berliku-liku,
tidak teratur baik di lengan dan jarijari

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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.

distonia:
Sebuah postur yang abnormal
berkerut, klasik dalam satu atau lain
dari gerakan ekstrem athetoid,
dengan kecenderungan untuk otot
korset batang dan tungkai atau
tangan atau kaki. Postur distonia juga
occure tanpa athetosis menyertainya.

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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 druginduced 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

EPS sekunder untuk agen

farmakologi adalah yang paling
umum.
Risiko mengembangkan obatinduced EPS dimulai pada awal
pengobatan dengan agen
menyinggung.
Akut: dalam beberapa jam atau
beberapa hari
Subacutely: selama beberapa
minggu
Terlambat atau tertunda onset:
enam bulan atau lebih setelah
paparan (dyskinesia)
terapi jangka pendek dari dosis
terapi yang minimal harus strategi
yang digunakan
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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

Definisi
tahan lama kontraksi atau
spasme otot
umumnya kurang umum daripada
kebanyakan gejala
ekstrapiramidal lainnya
muda usia dan jenis kelamin lakilaki dominan
Mekanisme patofisiologi saat ini
tidak diketahui
Diagnosis: Kelompok-kelompok
otot yang paling umum terkena
adalah mata, rahang, lidah, dan
leher
trismus, blefarospasme, krisis
oculogyric, tortikolis,
opisthotonus, kejang laring
<most dangerous>
Pengobatan
antikolinergik obat. 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
pengobatan
Turunkan dosis agen
Beralih ke agen potensi rendah
obat antikolinergik

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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

Definisi
rasa gelisah motor di mana pasien
merasa perlu terus-menerus untuk
bergerak.
Jenis yang paling umum dari EPS
Patofisiologi: tidak diketahui
Diagnosa
cenderung memiliki keluhan subjektif
'gelisah batin' yang paling sering di
kaki
Mereka merasa bahwa mereka harus
bergerak, dan ini bermanifestasi
sebagai perubahan pada postur
tubuh, menyilang dan uncrossing kaki
... ..
Sering dikaitkan dengan dysphoria
berat, kecemasan, dan lekas marah

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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

definisi
sindrom gerakan abnormal
berikut setidaknya enam bulan
dan sering bertahun-tahun
terapi obat
patofisiologi
denervasi-fenomena
hipersensitivitas
Tampaknya dengan blokade
reseptor berkepanjangan,
rebound reseptor, menjadi
supersensitized
diagnosa
Ditandai oleh gerakan tak
terkendali dari bibir, lidah,
rahang, dan ekstremitas
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Lipsmacking, wajah dan bahasa

gerakan pengunyahan, goyang
batang dan gerakan kaki gelisah
Dikurangi dengan gerakan sukarela
dari daerah yang terkena
Peningkatan oleh gerakan sukarela
dari daerah tidak terpengaruh
Meningkat dengan gairah emosional
Absen ketika individu tertidur

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

30

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
Penyakit Parkinson - Hypokinetic
Didefinisikan sebagai sindrom yang terdiri dari kombinasi variabel
tremor, rigiditas, bradykinesia, dan gangguan karakteristik gaya
berjalan dan postur
Onset: pertengahan akhir kehidupan; usia rata-rata adalah 57 thn

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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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