GANGGUAN GERAK DAN PENYAKIT PARKINSONTinjauan Umum

SISTEM MOTORIK

1. Sistem piramidal 2. Sistem ekstrapiramidal

3. Serebelum

Interaksi ketiganya menghasilkan gerak

Types of Movements

● Automatic movement Learned motor behaviors performed without conscious effort

(walk, speak)● Voluntary movement Intentional planned or self initiated, or externally triggered● Involuntary movement Not suppressible (tremor, myoclonus)● Semi-voluntary (un-voluntary) movement induced by inner sensory stimulus, move to suppress

unpleasant sensation, suppressible for short time (tic, akathisia, RLS)

GANGGUAN SISTEM MOTORIK

Lumpuh1. Sistem piramidal Kejang2. Sistem ekstrapiramidal Gangguan gerak

3. Serebelum Gangguan koordinasi (ataksia)

Extrapyramidal System

FacilitationPhysiology: function by Suppression

FacilitatePathophysiology: failure to Extrapyramidal dysfunction Suppress

MOVEMENT DISORDER

MOVEMENT DISORDER

DefinitionMovement disorder Is a neurological syndrome in which there iseither an excess of movement, or a paucity of voluntary andAutomatic movement. Unrelated to weakness or spasticity

It is a term for: 1. A physical sign 2. Describing a specific syndrome / condition

The Origin of Movement Disorders

1. Basal ganglia - Cerebral Globus pallidus, Caudate nucleus, Putamen - Diencephalon Subthalamic nucleus - Mesencephalon Substantia nigra

2. Non-basal ganglia a. Cerebellum b. Cerebral cortex c. Brainstem

3. Peripheral ? (Hemifacial spasm)

Specific Site for Specific MD

1. Basal Ganglia - Substantia nigra Bradykinesia, rest tremor - Subthalamic nucleus Ballism - Caudate nucleus Chorea - Putamen Dystonia

2. Non-ganglia basal - Cerebellum ataxia, dysmetria, intention tremor, progressive myoclonic ataxia - Brainstem reticular reflex myoclonus, hyperekplexia, palatal myoclonus, ocular myoclonus - Cerebral cortex cortical reflex myoclonus - Limbic structure + basal ganglia (?) tics

Classification of Movement disorders

Extrapyramial Dysfunction

Failure to Facilitate Failure to Suppress

HYPOKINESIA HYPERKINESIA (“Involuntary movement”)- Akinesia/Bradykinesia - Rigidity - Dyskinesia – Myoclonus- Diminished postural response - Tremor - Tics- Freezing - Chorea - Akathesia - Athetose - Hyperekplexia

No Weakness ! ! - Dystonia - Stereotypy

Diagnosing Movement Disorders

Requires:1. Indentify the pattern and type of movement2. Isolated or accompanied by other Neurologic signs3. Diagnosing movement disorder syndrome4. Determine probable etiology

Through:1. History2. Physical / Neurologic examination3. Investigations: blood test, spinal tap, neuroimaging, neurophysiologic exam

Pattern and Type of Movement HYPERKINESIA

● Tremor rhythmic, alternating agonist and antagonist , sinusoidal, regular Type: essential, rest, action trremor ● Chorea (“dance”) rapid, forceful, semipurposeful● Ballism large amplitude choreic movements of proximal parts of limbs ● Athetosis slow, writhing, mostly distally● Dystonia involuntary, sustained muscle contraction, causing repetitive twisting movement and abnormal

posture - focal, segmental, generalized● Myoclonus sudden brief shock-like involuntary movement from: muscle contraction (positive myoclonus) or muscle inhibition (negative myoclonus)

Pattern and Type of Movement

HYPERKINESIA (cont’d)● Tics -abnormal movement (motor tics) or abnormal sounds (phonic tics), or both (Tourette’s syndrome) - abrupy, brief moments - preceded by urge ● Akathesia feeling of inner restlesness leading to complex stereotyped movements, which may

reduce the sensation● Stereotypy - coordinated movements that repeat themselves continually and identically - not preceded by urge - In tardive dyskinesia● Restless leg syndrome - urge to move the limb with uncomfortable sensations

Pattern and Type of Movement

HYPOKINESIA● Rigidity - increased tone throughout all directions of movement. - Flexor > extensor - “lead pipe”/”plastic”, “cogwheel” phenomenon● Bradykinesia slowness of movement● Freezing - motor act halted transiently (several seconds) - Includes: start hesitation, turning hesitation, destination hesitation● Apraxia - inability to perform complex learned voluntary movement - not due to weakness, spasticity, rigidity, sensory loss

HIPOKINESIA

PARKINSONISM

Parkinsonism adalah suatu sindroma yang ditandai oleh gejala-gejala klinis yang terdiri dari rest tremor , rigiditas, akinesia, dan instabilitas postural.

Jenis-jenis Parkinsonism

I. Idiopathic/Primary parkinsonism Parkinson’s disease, juvenile parkinsonism

II. Symptomatic /secondary parkinsonism Infectious, drugs, toxin, vascular, trauma, others (hypothyroidism, tumor, NPH,

obstructive hydrocephalus)

III. Parkinson plus syndrome / Multiple system degenerations PSP, MSA (SNA, OPCA, SDS), Parkinsonism-dementia- ALS complex of Guam (PDACG), progressive pallidal atrophy.

IV. Hereditary parkinsonism Hereditary juvenile dystonia parkinsonism, Lewy body disease, Huntington’s

disease, Wilson’disease

PENYAKIT PARKINSON

PENYAKIT PARKINSON adalah penyakit degeneratif susunan saraf pusat yang idiopatik, bersifat kronis dan progresif, ditandai oleh gejala-gejala kelainan motorik berupa gemetar , kaku , gerakan lamban, dan gangguan stabilitas tubuh.

Penyakit Parkinson pertama kali dikemukakan oleh James Parkinson tahun 1817. Dinamakan juga paralysis agitans oleh Marshall Hall tahun 1841.

1. APA ITU PENYAKIT PARKINSON ?

● Penyakit Parkinson merupakan jenis Parkinsonism yang terbanyak (± 80%)

● Prevalensi (USA): 150-200/100.000 penduduk

● Usia timbul: 40-70 tahun, puncaknya 60 tahun, jarang sebelum 30 tahun

● Prevalensi meningkat seiring dengan usia: > 50 tahun: 1000/100.000 > 65 tahun: 1100/100.000● Distribusi sex: pria : wanita 3:2

● Keturunan : 5 % bersifat familial

EPIDEMIOLOGI PENYAKIT PARKINSON

Pathology and PD Stages

New Insight into Pathological Process of PD

Braak and colleagues study on the course of PD pathological process.

PD is now considered multisystem degenerative disease, involving: Dopaminergic, Noradrenergic, Serotoninergic, Cholinergic, and other central neurotransmitter systems.

Parkinson’s disease is now considered to be a Neuropsychiatric disorder, not a pure motor disorder.● Manifesting with Motor and Non-motor symptoms● Non-motor symptom may precede Motor symptom : Depression , Anxiety, REM Sleep Behaviour Disorder (RBD) , Anosmia or hyposmia

◘ Sejauh ini penyebab Penyakit Parkinson belum diketahui.

◘ Beberapa faktor risiko diketahui dapat meningkatkan kemungkinan mendapat penyakit Parkinson, a.l.: 1. Usia Risiko meningkat seiring dengan meningkatnya usia 2. Jenis kelamin Laki-laki lebih mudah terkena daripada wanita. 3. Genetik 4. Lingkungan Toksin MPTP, gas CO, pestisida, herbisida, minum air sumur tertentu, infeksi 5. Ras Orang kulit putih lebih sering terkena daripada orang kulit hitam dan Asia

Apa penyebab penyakit Parkinson ?

1. GEJALA MOTORIK (Gejala Utama), 4 jenis: ● Tremor saat istirahat ● Rigiditas (Kekakuan) ● Akinesia / Bradikinesia (gerakan melambat) ● Postural Instability (gangguan stabilitas tubuh) (T R A P )

2. GEJALA NON-MOTORIK Bisa timbul beberapa tahun sebelum diagnosis Parkinson ditegakkan. a. Disfungsi saraf otonom Orthostatic hypotension, kulit berminyak , keringat berlebihan, inkontinensia urin, disfungsi seksual, konstipasi, gangguan motilitas lambung. b. Gejala neuropsikiatrik Mood, kognisi, perilaku, pikiran. c. Gejala sensori nyeri otot, kesemutan d. Gangguan tidur

2. GEJALA-GEJALA KLINIS PENYAKIT PARKINSON

Stadium Klinis (HOEHN and YAHR)Stage 1 = Gejala unilateral.Stage 2 = Bilateral. Stage 3 = Instabilitas postural; jarang jatuh.Stage 4 = Instabilitas postural jelas; cenderung jatuh.Stage 5 = Berbaring atau duduk di kursi roda saja.

Bagaimana Perjalanan Klinis Penyakit Parkinson ?

Parkinsonism TypesPrimary Parkinsonism Secondary ParkinsonismIdiopathic Parkinson’s disease Iatrogenic:Dementia with Lewy bodies Phenothiazines, butyrophenones, lithium,Gentic Parkinsonism Na valproate, Ca-blockers, reserpineFrontotemporal dementia tetrabenazine, α-m-dopaWith Parkinsonism Toxic:Alzheimer’s disease MPTP, CO, Mg,Cyanide,methanol,MSAp and MSAc organophospatesPSP, CBD Infectious:Neuroacantocythosis Encephalitis lethargica, CJD, AIDS,

syphilisHuntington’s disease Metabolic:Creutzfeld-Jacob disease Hypoparathyroidism, Wilson’s diseaeSpinocerebellar degeneration Structural: NPH, CNS trauma, tumor, infarction

PARKINSON’S DISEASE: CLINICAL DIAGNOSTIC CRITERIA

1. Historical Diagnostic Criteria Symptoms: Tremor, Rigidity, Akinesia, Postural Instability (TRAP)

• Two of three cardinal symptoms (2/3 TRA) - Tremor, Rigidity, A kinesia / bradykinesia OR

• Three of four symptoms (3/4 TRAP) Tremor, Rigidity, Akinesia, Postural instability OR • Responsive to L-DOPA.

PARKINSON’S DISEASE:NEW CLINICAL DIAGNOSTIC CRITERIA

(UK Parkinson’s Disease Society Brain Bank / UKPDSBB)

Step 1. Diagnosis of Parkinsonism Step 2. Exclusion criteria for Parkinson’s disease Step 3. Supportive prospective positive criteria for Parkinson’s disease

UKPDSBB CLINICAL DIAGNOSTIC CRITERIA

Step 1. Diagnosis of PARKINSONISM

√ BRADYKINESIA + At least ONE of the following: √ RIGIDITY √ REST TREMOR (4-6 Hz) √ POSTURAL INSTABILITY not caused by primary visual, vestibular, cerebellar, or proprioceptive dysfunction

UKPDSBB Clinical Diagnostic Criteria

Step 2 Exclusion criteria for Parkinson’s disease• History of repeated strokes with stepwise progression of

parkinsonian features• History of repeated head injury• History of definite encephalitis• Oculogyric crises• Neuroleptic treatment at onset of symptoms• More than one affected relative• Sustained remission

Step 2 Exclusion criteria for Parkinson’s disease (cont’d)

• Strictly unilateral features after 3 years• Supranuclear gaze palsy• Cerebellar signs• Early severe autonomic involvement• Early severe dementia with disturbances of memory, language, and praxis• Babinski sign• Presence of cerebral tumor or communication hydrocephalus on imaging

study• Negative response to large doses of levodopa in absence of malabsorption• MPTP exposure

UK PDSBB CLINICAL DIAGNOSTIC CRITERIA

Step 3. Supportive prospective positive criteria for Parkinson’s disease

Three or more required for diagnosis of definite Parkinson’s disease incombination with step one• Unilateral onset• Rest tremor present• Progressive disorder• Persistent asymmetry affecting side of onset most• Excellent response (70-100%) to levodopa• Severe levodopa-induced chorea• Levodopa response for 5 years or more• Clinical course of ten years or more