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GANGGUAN GERAK DAN PENYAKIT

PARKINSON
Tinjauan 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


Lumpuh
1. Sistem piramidal
Kejang
2. Sistem ekstrapiramidal Gangguan
gerak
3. Serebelum Gangguan koordinasi
(ataksia)

Extrapyramidal System
Facilitation
Physiology: function by
Suppression
Facilitate
Pathophysiology: failure to
Extrapyramidal dysfunction
Suppress

MOVEMENT DISORDER

MOVEMENT DISORDER
Definition
Movement disorder Is a neurological syndrome in
which there is
either an excess of movement, or a paucity of
voluntary and
Automatic 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
Myoclonus
- Diminished postural response
- Tics

- Dyskinesia
- Tremor

Diagnosing Movement Disorders


Requires:
1. Indentify the pattern and type of movement
2. Isolated or accompanied by other Neurologic signs
3. Diagnosing movement disorder syndrome
4. Determine probable etiology
Through:
1. History
2. Physical / Neurologic examination
3. 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 (contd)
Tics
-abnormal movement (motor tics) or abnormal sounds (phonic tics), or
both (Tourettes 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
Parkinsons 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,
Huntingtons disease, Wilsondisease

PENYAKIT PARKINSON

1. APA ITU 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.

EPIDEMIOLOGI 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

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.

Parkinsons 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

Apa penyebab penyakit


Parkinson ?
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

2. GEJALA-GEJALA KLINIS 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

Bagaimana Perjalanan 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.

Parkinsonism Types
Primary Parkinsonism
Parkinsonism
Idiopathic Parkinsons disease
Dementia with Lewy bodies
Gentic Parkinsonism
Frontotemporal dementia
With Parkinsonism
Alzheimers disease
MSAp and MSAc
PSP, CBD
Neuroacantocythosis
syphilis
Huntingtons disease
Creutzfeld-Jacob disease
Spinocerebellar degeneration
infarction

Secondary
Iatrogenic:
Phenothiazines, butyrophenones,
lithium,
Na valproate, Ca-blockers, reserpine
tetrabenazine, -m-dopa
Toxic:
MPTP, CO, Mg,Cyanide,methanol,
organophospates
Infectious:
Encephalitis lethargica, CJD, AIDS,
Metabolic:
Hypoparathyroidism, Wilsons diseae
Structural:
NPH, CNS trauma, tumor,

PARKINSONS 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.

PARKINSONS DISEASE:
NEW CLINICAL DIAGNOSTIC CRITERIA
(UK Parkinsons Disease Society Brain Bank /
UKPDSBB)
Step 1. Diagnosis of Parkinsonism
Step 2. Exclusion criteria for Parkinsons
disease
Step 3. Supportive prospective positive criteria
for
Parkinsons 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

UKPDSBB Clinical Diagnostic Criteria


Step 2 Exclusion criteria for Parkinsons
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 Parkinsons disease (contd)


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 Parkinsons
disease
Three or more required for diagnosis of definite Parkinsons
disease in
combination 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