Extrapyramidal syndrome(movement disorders)

Dr. Zuraini, Sp.S

2

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 movementsSindrom neurologis di mana gerakan abnormal terjadi karena gangguan kelancaran dan kecepatan gerakan sukarela atau adanya gerakan ekstra yang tidak diinginkan

3

Ektrapyramidal System

A GROUP STRUCTURAL OF “ Gray matter” wich located in “ hemispher cerebri” and mainly functional was motor activity

TERDIRI DARI : Caudate nucleus

Putamen

Globus palidus

1.Basal Ganglia

2.Brain stem

3.Cortek serebri

KELOMPOK STRUKTURAL DARI "materi Gray" yang terletak di "hemispher cerebri" dan terutama fungsional aktivitas motorik

4

Nucleus caudatus + Putamen

Corpus striatum (neostriatum)

Putamen + Globus palidus (nucleus lenticularis)

5

Subthalmicus nuclei Substantia Nigra Parts of formatio reticularis

Area 4 S Area 6 Area 8

BRAINSTEM

CORTEKS CEREBRI

6

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

7

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 multi-langkah

8

Pathogenesis 〈 2 〉

9

Movement disorder

Motor network ： Upper motor

neurons Lower motor

neurons Cerebellar circuitry Basal ganglia

circuitry Motor association

cortex Sensory systems

10

Thalamus

CortexGlobus pal

Caudatus+put

Subtthalamicusnuclei

PonsSubstantia

nigra

Serebellum

Nigro reticulo spinal tract Piramidal tract

1

3

2

Diagram of ekstrapyramidal circuit

striatum

4

11

THE MAIN FUNGTION OF EPS ASCOCIATED WITH

Ascociative movement Regulation of posture Autonomic integration

FUNGSI UTAMA DARI EPS TERKAIT DENGAN

asosiatif gerakanPeraturan posturintegrasi otonom

12

EKSTRAPYRAMIDAL SISTEM DISTURBANCES

Ektrapyramidal syndroma = Basal Ganglia syndroma

1.Primary functional deficit negative symptom

2.Secundary efect/ “release pheno mena positive symptom

13

EPS SYNDROMA

Negative symptom

1. Bradikinesia 2. Disturbances posture

Positive symptom

1.Involuntary movement tremor, athetosis, ballismus, chorea, dystonia

2. Rigiditas

14

Types of hyperkinetic movement disorders

The differential diagnosis of chorea

Practical Neurology 2007;7;360-373

Slow,distal

dancelike

15

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

16

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

17

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 jari-jari

18

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.

19

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

20

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

EPS sekunder untuk agen farmakologi adalah yang paling umum.

Risiko mengembangkan obat-induced 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

21

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 〉

22

Manifestation

Acute dystonia Parkinsonism Akathisia Tardive dyskinesia

23

Acute dystonia Definition

long-lasting contraction or spasm of musculaturegenerally less common than most other extrapyramidal symptomsyoung age and male sex predominantThe 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 laki-laki 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

24

ParkinsonismPathophysiology: blockade of postsynaptic dopamine(D2) receptors in the corpus striatumDiagnosis 〈 three cardinal symptoms 〉

TremorMuscle rigidityBradikinesia

25

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

pengobatanTurunkan dosis agenBeralih ke agen potensi rendahobat antikolinergik

26

Akathisia

Definition a sense of motor restlessness in

which the patient feels a constant need to move about.The most common type of EPSPathophysiology: unknownDiagnosis

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

27

TreatmentLower dose of agentSwitching to a low potency agentAnticholinergic drugsbeta-adrenergic blockersbenzodiazepine

28

Tardive dyskinesia

Definition a syndrome of abnormal

movements following at least six months and often many years of drug therapy

Pathophysiologydenervation-hypersensitivity phenomenonIt appears with prolonged receptor blockade, the receptors rebound, becoming supersensitized

DiagnosisCharacterized by involuntary movement of the lips, tongue, jaw, and extremities

definisi     sindrom gerakan abnormal berikut setidaknya enam bulan dan sering bertahun-tahun terapi obatpatofisiologidenervasi-fenomena hipersensitivitasTampaknya dengan blokade reseptor berkepanjangan, rebound reseptor, menjadi supersensitizeddiagnosaDitandai oleh gerakan tak terkendali dari bibir, lidah, rahang, dan ekstremitas

29

Lipsmacking, facial and lingual masticatory movements, trunk rocking and restless foot movementsReduced by voluntary movements of the affected areasIncreased by voluntary movements of unaffected areasIncreased with emotional arousalAbsent when the individual is asleep

Lipsmacking, wajah dan bahasa gerakan pengunyahan, goyang batang dan gerakan kaki gelisahDikurangi dengan gerakan sukarela dari daerah yang terkenaPeningkatan oleh gerakan sukarela dari daerah tidak terpengaruhMeningkat dengan gairah emosionalAbsen ketika individu tertidur

30

TreatmentThe best treatment is preventiongradual reductionLow dose of benzodiazepineDopamine antagonistDopamine depleting agents

31

Movement Disorders

Parkinson’s 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 - HypokineticDidefinisikan sebagai sindrom yang terdiri dari kombinasi variabel tremor, rigiditas, bradykinesia, dan gangguan karakteristik gaya berjalan dan posturOnset: pertengahan akhir kehidupan; usia rata-rata adalah 57 thn

32

› 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

33

› Cause: unknown› Pathophysiology:

1. Loss of dopaminergic cells in the substantia nigra • Dopamine’s normal function

2. Over excitation of the caudate & putamen3. Over excitation of the corticospinal tracts4. Decrease in thalamic excitation of the

motor cortex

34

› Four Hallmark Signs# Resting Tremor (Pill-Rolling)# Rigidity# Bradykinesia# Flexed Posture with shuffling gait

(Festinating)

35

Movement Disorders

Huntington’s Disease – Hyperkinetic› A neurodegenerative disorder which

predominately has behavioral, cognitive, or movement disorders signs

› Onset: Usually begins during adult life

36

› Cause: Autosomal Dominant Disorder› Pathophysiology:

# Mutation on chromosome 4# Uncertainty?

37

# Pathological Changes# Atrophy & neuronal

degeneration of cortex# Hallmark: caudate

atrophy

38

› Examination:# Physical Findings

# Initial Findings# Gradual onset# Slowed saccadic movements 1st

sign# In 85% chorea is predominate

movement disorder

39

# Adult Onset# Prominent chorea# Bradykinesia# Postural reflex compromise

# Terminal Phase# Dysarthria, dysphagia, &

respiratory difficulties# General

# Cognitive impairment# Depression# Psychiatric disorders

40

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

41

› Common Descriptions# Always unpleasant, but not

necessarily painful# Need to move# Crawling# Tingling# Itching# Restless

42

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 = archicerebellum2.Anterior lob = paleocerebelum3.Posterior lob = neocerebellum

43

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.

44

Pedunculus serebelliTree 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

45

Pedunculus cerebelli medialis(fibre from pontin nuclei contra lateral)

Pedunculus cerebelli superior

(eferents fibre thalamus / med spinalis )

46

CEREBELLUM FUNCTION

Regulation and control of muscle tone

Coordination of movement, mainly skill movement

Control posture and gait

47

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

48

Because the efferent cerebellar pathwayTo the cerebral hemispheres are crossed

And the corticospinal system is againCrossed, a unilateral lesion of the Cerebellum causes an ipsilateral

Disorder of movement

49

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

Dopamine Serotonin GABA

Cholinergic

50

THANK YOU !