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PENYAKIT PARKINSON

UTOYO SUNARYO
DEPARTEMEN NEUROLOGI
FK U.W.K SURABAYA
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
MAKRO ANATOMI SUSUNAN
SARAF PUSAT
 OTAK.
 OTAK BESAR ( SEREBRUM).
 OTAK KECIL ( SEREBELUM).
 DIENSEFALON.
 THALAMUS.
 HIPOTHALAMUS
 BATANG OTAK ( TRUNKUS SEREBRI).
- MESENSEFALON.
- PONS.
- MEDULA OBLONGATA.
 MEDULA SPINALIS
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
GANGLIA BASALIS

1. Striatum (neostriatum & limbic striatum)


Neostriatum : - Putamen &
- Nucleus Caudatus
2. Globus Palidus (GP) : Interna & Externa.
3. Substansia Nigra (SN) :
- SNc : Pars Compacta
- SNr : Pars Reticular
4. Subthalamic Nucleus (STN)
CORTICAL MOTOR AREAS

THALAMUS BASAL
GANGLIA

CEREBELLUM

BRAINSTEM

SPINAL
CORD
Ganglia Basalis dalam
Menciptakan Gerakan :
 Stimulasi Korteks Motorik.

 Melalui pintu masuk putamen

(di Striatum)
 Keluar melalui Globus Palidus

internus
 Menciptakan jalur direk dan indirek.
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
PATOFISIOLOGI PENYAKIT
PARKINSON
“Ketidak seimbangan saraf
dopaminergik dan kolinergik”
DA AK AK = ASETIL KOLIN
DA = DOPAMIN

NORMAL
(AK=DA)

PENYAKIT PARKINSON
(AK>DA)
BRAIN
Ganglia basalis
Dopamin Acetylcholin
Normal
MAO MAO I ( selegiline )
Anticholinergic
Perokside Radical H (Trihexylphenidyl)
Dopamin Tissue
Receptor D2 damage
Decarboxylase
Levodopa Acetylcholin PD
BLOOD BRAIN BARIER
Levodopa 3 OMD
COMT Inhibitor
COMT
Dopamin Agonist (entacapone)
Decarboxylase

Decarboxylase Inhibitor
Ergot Non Ergot
Dopamin
(Benzeraside)
(bromocryptin) (pramipexole)
PERIFER (carbidopa)
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
EPIDEMIOLOGI

Prevalensi dari Penyakit Parkinson belum


diketahui secara pasti diperkirakan 1 %
dari jumlah penduduk antara usia 50 th
s/d 70 th dari pria atau wanita dengan ras
Kaukasian lebih banyak dibanding Afro
Amerika .
Poli saraf RS Dr Soetomo Sby : sekitar 190
pasien parkinson per tahun.
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
Parkinson’s Disease
Definisi by James Parkinson 1817 :

T
R
Penyakit A
Parkinsonisme
Parkinson P
What is Parkinson’s Disease?

 Clinical syndrome manifesting


characteristically with parkinsonism
(bradikinesia, hipo/akinesia. rigidity,
tremors, and postural instability),
caused by significant dopaminergic
striatal denervation. (Wolters, 2007)
PENYAKIT PARKINSON
vs PARKINSONISM

PARKINSONISM

 SINDROMA :
Tremor, Rigidity, Akinesia, Postural
instability (TRAP)

 MACAM-MACAM PENYEBAB
PENYAKIT PARKINSON
Etiologi : Idiopatik
Degenerasi neuron di substansia nigra &
ada lewy bodies
80% Parkinsonism
 Onset :
 >50 TAHUN
 PUNCAK 70 an TAHUN

PRIA : WANITA = 3 :2
Rata-rata 9 tahun meninggal tanpa
terapi
KLASIFIKASI PARKINSONISM
 Parkinsonism Idiopatik / Primer
= Penyakit Parkinson=
 Parkinsonism Simptomatik / Sekunder
Drugs Induced, Infectious, Head Trauma,
Tumor/paraneoplastik, Vascular, Toxins,
Metabolic, Hidrocephalus,Anoxic encephalopathy
 Parkinsonism Plus
Dementia syndroma (Alzheimer’s Disease) ,
Multiple System Atrophy, Normal Pressure
Hydrocephalus, Progressive supranuclear palsy,
Corticobasal ganglionic degeneration, Hereditary
disorders.
Parkinson’s Disease
ETIOLOGY:

Etiology dari Penyakit Parkinson belum


diketahui secara pasti , namun ada beberapa
faktor yang dapat meningkatkan Resiko
dari Penyakit Parkinson :
Parkinson’s Disease
Faktor yang dapat menyebabkan
meningkatnya Resiko Penyakit Parkinson :

Aging , Gender ( Men) , and Ras ( White )


Riwayat Keluarga :
•Trauma
•Stress Emosional
•Personality ( Shyness and Depressiveness)
Environmental Exposures
•Metals ( Manganese , Iron )
•Drinking unwell water
•Farming
•Herbicide and Pepticide exposure
•MPTP ( methyl phenyl tetrahydropiridine)
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
PENYAKIT PARKINSON

GEJALA UTAMA (TRAP)


 TREMOR

 RIGIDITY

 AKINESIA

 POSTURAL INSTABILITY
Tremor

 Resting tremor, postural tremor


 Kasar, 3-7 getaran / detik
 Tangan, kaki, badan, bibir
Rigidity

 Cogwheel phenomenon

 Leher, Lengan, Tungkai, Badan


Akinesia

 Lama tidak bergerak atau lambat

(Bradikinesia)

 Muka topeng, kurang berkedip


Postural Instability

 Mudah terjatuh, propulsi

 Pada stadium lanjut


Diagnosis
KRITERIA KLINIS
 Dua dari 3 gejala kardinal (TRA)

 Tiga dari 4 gejala utama (TRAP)

 Responsif terhadap L-dopa


KRITERIA HUGHES
 POSSIBLE
Tdpt salah satu gejala utama:
Tremor istirahat.
Rigiditas
Bradikinesia.
Kegagalan reflesk postural
KRITERIA HUGHES
 PROBABLE
Bila terdapat kombinasi dua gejala utama
( termasuk kegagalan refleks postural)
 DEFINITE

Bila terdapat kombinasi tiga dari empat


gejala.
TIGA FASE PARKINSON
DISEASE
1. Preclinical stage – at this PD-spesific
pathology is present with no clinical
signs at all and potential imaging and
biomarker abnormalities may be able
to detect these changes
2. Pre-motor – extranigral pathology
causing symptoms
3. Motor PD – classical nigral pathology
AAN 2012
GNM/Pre-motor
Penyakit Parkinson (PP)
Yang disepakati sebagai gejala dini PP
berdasar HistoPA dari Braak dkk:

Tolosa, E, et al. Neurology 2009 ( Suppl 2 ): S12-S20.


GNM lainnya

Gejala Non-Motor sebelum dan sesudah


dx. PP ditegakkan, sbb.:
Neuropsikiatri:
* gg. kognisi dan demensia
* apatis, ansietas, panik
* ilusi, delusi, halusinasi, dilirium
* ICDs (impulse control disorders)
* depresi.
GNM lainnya
Gangguan tidur:
* RBD (REM behavior disorders)
* EDS (excessive daytime sleepiness)
* RLS (restless legs syndrome)
* PLMS (periodic limb movement in sleep)

Gangguan sistem saraf otonom:


* hipotensi ortostatik * OAB (overactive bladder)
* disfungsi ereksi * hiperseks
* konstipasi * drooling saliva 37
Progresifitas Parkinson
Disease

AAN 2012
PERJALANAN PENYAKIT
( Hoehn and Yahr )
 Stadium 1: gejala dan tanda pada satu
sisi, terdapat gejala yg ringan, terdpt
gejala mengganggu ttp tdk
menimbulkan kecacatan, biasanya
tremor pd satu anggota gerak, gejala
yg timbul dapat dikenali orang terdekat.
 Stadium 2 : terdapat gejala bilateral,
terdapat kecacatan minimal, sikap/cara
berjalan terganggu.
 Stadium 3 : gerak tubuh nyata
melambat, keseimbangan mulai
terganggu saat berjalan/berdiri,
disfungsi umum sedang.
 Stadium 4 : terdapat gejala yang lebih
berat, masih dpt berjalan hanya utk
jarak tertentu, rigiditas dan
bradikinesia, tdk mampu berdiri sendiri,
tremor dpt berkurang dibanding
stadium sebelumnya.
 Stadium 5 : stadium kakhetik ( cachetic
stage ) , kecacatan total, tidak mampu
berdiri dan berjalan, memerlukan
perawatan tetap.
DIFFERENTIAL DIAGNOSIS OF
PARKINSON DISEASE
 ESSENTIAL TREMOR – OCCASIONALLY
CONFUSED WITH PARKINSON DISEASE.
HOWEVER, 20% OF ET PATIENTS DEVELOP
PD
 SECONDARY PARKINSONISM, i.e. DRUGS,
NPH, INFECTIONS, etc.
 “PARKINSON – PLUS” SYNDROMES, i.e. CBD,
LBD, AD, MSA, PSP
 HEREDODEGENERATIVE – HD, WILSON,
HALLERVORDEN-SPATZ
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
Brook D.J: Neuroimaging in Parkinson’s Disease,Neuro RX 1: 243-254,2004
Marek K et al: (123 I )B CIT Spect Imaging Assesment of the rate of Parkinson Disease
Progression, Neurology 2001, 57:2089-2054
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
NEUROPATOLOGI

 DEGENERASI NEURON SUBSTANSIA


NIGRA.
o TERDAPAT LEWI BODY.
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
PENGOBATAN
FARMAKOLOGIS DIBAGI: (1)
 NEUROPROTEKTIF.
1.1 MAOIs (Monoamin oxidase inhibitory )
Selegine.
Rasagiline.
Lazabernide.
1.2 Obat antiexcitatory.
NMDA Antagonis: remacemide,amantadine,riluzole.
Coenzyme Q10 dan nicotinamide.
PENGOBATAN
FARMAKOLOGIS DIBAGI: (2)
1.3 Faktor tropik.
GDNF.
GMI ganglioside.
1.4 Immunomodulator.
NSAID.
Immunophyllins.
1.5 Cyclosporine A, calpain inhibitor, caspase inhibitor.
PENGOBATAN
FARMAKOLOGIS DIBAGI: (3)
 DOPAMINERGIK.
2.1 Prekursor dopamin.
Levodopa.
Levodopa CR
Levodopa cair.
Obat obat menaikkan kadar levodopa.
o Levodopa + decarboxylase inhibitor.
o Levodopa + COMT inhibitor.
o Levodopa + selegilene.
PENGOBATAN
FARMAKOLOGIS DIBAGI: (4)
2.2 Agonis dopamin.
Derivat ergot: bromocriptin, pergolide, lisuride,
cabergoline.
Derivat non ergot: PRAMIPEXOLE, ropinirole,
talipexole, apomorfin, selegiline.
PENGOBATAN
FARMAKOLOGIS DIBAGI: (5)
 NON DOPAMINERGIK.
ANTIKOLINERGIK: Trihexyfenidyl, benstropin.
GLUTAMAT ANTAGONIS: Amantadine, memantine.
ADENOSINE A2A ANTAGONIS.
GM 1 GANGLIOSIDE.
ALGORITMA
PENATALAKSANAAN
PENYAKIT PARKINSON

Konsensus POKDI
Gangguan Gerak
PERDOSSI 2003
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
UNIFIED PARKINSON’S DISABILITY
RATING SCALE ( UPDRS )
 Mentation, Behaviour and Mood.
 Intelectual impairment.
 Thought disorder.
 Depresion.
 Motivation – initiative.
 Activities of Daily Living.
 Speech.
 Salivation.
 Swallowing.
 Handwriting.
 Cutting food-handling utensils.
 Dressing.
 Hygiene.
 Turning in Beds-Adjusting Bed Clothes.
 Falling-unrelated to freezing.
 Walking.
 Tremor.
 Sensory Complaints related to Parkinsonism
UNIFIED PARKINSON’S DISABILITY
RATING SCALE ( UPDRS ) con’t
 Motor Examination.
 Speech.
 Facial Expression.
 Tremor at Rest.
 Action or Postural Tremor.
 Rigidity.
 Finger Tab.
 Hand Movement.
 Rapid Alternating Movement.
 Leg Agility.
 Arising from Chair.
 Posture.
 Gait.
 Postural Stability.
 Body Bradykinesia.
UNIFIED PARKINSON’S DISABILITY
RATING SCALE ( UPDRS )

 TESTING FUNCTIONAL PERFORMANCE


IN PEOPLE WITH PARKINSON
DISEASE
 Definitif skor setiap item : 0 - 5
 Total skor : 0 - 199
 Skor 199 : Parkinson Berat.
OUTLINE
 NEUROANATOMI.
 NEUROFISIOLOGI.
 NEUROPATOFISIOLOGI.
 NEUROEPIDEMIOLOGI
 BATASAN.
 GEJALA KLINIS.
 NEURORADIOLOGI.
 NEUROPATOLOGI.
 NEUROFARMAKOLOGI.
 UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS )
 LIVING WITH PARKINSON’S DISEASE
UTOYO SUNARYO, NEUROLOGIST OF MOHAMAD SALEH MD HOSPITAL , PROBOLINGGO

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