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

TERDIRI DARI :

Caudate nucleus

1.Basal Ganglia 2.Brain stem 3.Cortek serebri

Putamen
Globus palidus

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

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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Results from dysfunction of the extrapyramidal system Basal ganglioncaudate, 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

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mempengaruhi ukuran dan

kecepatan gerakan pemilihan komponen gerakan atau urutan gerakan multi-langkah

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

Subtthalamicus nuclei Thalamus

Serebellum

4 Globus pal

Cortex

Substantia nigra

Caudatus+put

Pons

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striatum
Nigro reticulo spinal tract Piramidal tract
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Diagram of ekstrapyramidal circuit

FUNGSI UTAMA DARI EPS TERKAIT DENGAN

Ascociative

movement Regulation of posture Autonomic integration


asosiatif gerakan Peraturan postur integrasi otonom

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

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Negative symptom 1. Bradikinesia 2. Disturbances posture

Positive symptom

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

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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

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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
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 Unilateral plastic rigidity with static tremor Unilateral hemiballismus and hemichorea

Location of lesion contralat subs nigra contralat subthal nucleus of luysial

Chronic chorea of huntington type


Athetosis and dystonia Cerebellar incord,Intention tremor and Hypotonia

caudatus nuc & putamen


contralateral striatum homolat cerebel hemisfher or midle and inf cerebellar peduncles

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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 stimulantsact as indirect dopamine agonist ex. Amphetamine Levodopaa precursor of dopamine Direct dopamine agonistex. Bromocriptine Presynaptic dopamine antagonists ex. Reserpine Antagonize or block central dopamine receptors neuroleptics, metoclopramideprimperam

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Acute

dystonia Parkinsonism Akathisia Tardive dyskinesia

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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. 23 Benztropine, diphenhydramine

Pathophysiology: blockade of postsynaptic dopamine(D2) receptors in the corpus striatum Diagnosisthree 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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a sense of motor restlessness in which the patient feels a constant need to move about.

Definition

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

Definition

Pathophysiology

Diagnosis

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

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

definisi sindrom gerakan abnormal berikut setidaknya enam bulan dan sering bertahuntahun 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, 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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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

Treatment
The best treatment is prevention gradual reduction Low dose of benzodiazepine Dopamine antagonist Dopamine depleting agents

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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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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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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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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
spinocerebellaris tract gait maintenance

Paleocerebellum receive aferents fibre from

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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Regulation and control of muscle tone Coordination of movement, mainly skill movement Control posture and gait

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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
dismetria disdiadokinesia intention tremor Disarthria Dyssynergy
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of coordination Ataxia

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