penyakit parkinson - · pdf fileprevalensi dari penyakit parkinson belum ... dementia...
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PENYAKIT PARKINSON
UTOYO SUNARYORSUD Dr.MOHAMAD SALEH
KOTA PROBOLINGGO
PROBOLINGGO , 23 NOVEMBER 2005
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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MAKRO ANATOMI SUSUNANSARAF PUSAT
OTAK. OTAK BESAR ( SEREBRUM). OTAK KECIL ( SEREBELUM). DIENSEFALON.
THALAMUS. HIPOTHALAMUS
BATANG OTAK ( TRUNKUS SEREBRI).- MESENSEFALON.- PONS.- MEDULA OBLONGATA.
MEDULA SPINALIS
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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GANGLIA BASALISGANGLIA BASALIS
1. Striatum (neostriatum & limbic striatum)Neostriatum : - Putamen &
- Nucleus Caudatus2. Globus Palidus (GP) : Interna & Externa.3. Substansia Nigra (SN) :
- SNc : Pars Compacta- SNr : Pars Reticular
4. Subthalamic Nucleus (STN)
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CORTICAL MOTOR AREASCORTICAL MOTOR AREAS
THALAMUS BASALGANGLIA
CEREBELLUM
BRAINSTEM
SPINALCORD
THALAMUS BASALGANGLIA
CEREBELLUM
BRAINSTEM
SPINALCORD
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Ganglia Basalis dalamMenciptakan Gerakan :Ganglia Basalis dalamMenciptakan Gerakan :
Stimulasi Korteks Motorik.
Melalui pintu masuk putamen
(di Striatum)
Keluar melalui Globus Palidus
internus
Menciptakan jalur direk dan indirek.
Stimulasi Korteks Motorik.
Melalui pintu masuk putamen
(di Striatum)
Keluar melalui Globus Palidus
internus
Menciptakan jalur direk dan indirek.
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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PATOFISIOLOGI PENYAKITPARKINSON
““KetidakKetidak seimbanganseimbangan sarafsarafdopaminergikdopaminergik dandan kolinergikkolinergik””
DA AK
NORMAL(AK=DA)
PENYAKIT PARKINSON(AK>DA)
AK = ASETIL KOLINDA = DOPAMIN
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B R A I NB R A I NGanglia basalis
Acetylcholin Normal
Dopamin
Acetylcholin PD
Perokside Radical HTissueTissue
damagedamage
Anticholinergic
(Trihexylphenidyl)
MAO MAO I ( selegiline )
D2
Dopamin
Receptor
DopaminDopamin AgonistAgonist
Ergot(bromocryptin)
Non ErgotNon Ergot((pramipexolepramipexole))
Levodopa
Levodopa
Dopamin
Decarboxylase
Decarboxylase Inhibitor(Benzeraside)(carbidopa)
3 OMD
COMTCOMT Inhibitor
(entacapone)
BLOOD BRAIN BARIER
PERIFER
Decarboxylase
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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EPIDEMIOLOGI
Prevalensi dari Penyakit Parkinson belumdiketahui secara pasti diperkirakan 1 %dari jumlah penduduk antara usia 50 ths/d 70 th dari pria atau wanita dengan rasKaukasian lebih banyak dibanding AfroAmerika .
Poli saraf RS Dr Soetomo Sby : sekitar 190pasien parkinson per tahun.
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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Parkinson’s Disease
Definisi by James Parkinson 1817 :
PenyakitParkinson
Parkinsonisme
TRAP
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1919
What is Parkinson’s Disease?
Clinical syndrome manifestingcharacteristically with parkinsonism(bradikinesia, hipo/akinesia. rigidity,tremors, and postural instability),caused by significant dopaminergicstriatal denervation. (Wolters, 2007)
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PARKINSONISM
SINDROMA :Tremor, Rigidity, Akinesia, Posturalinstability (TRAP)
MACAM-MACAM PENYEBAB
PENYAKIT PARKINSONPENYAKIT PARKINSONvsvs PARKINSONISMPARKINSONISM
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Etiologi : IdiopatikDegenerasi neuron di substansia nigra &
ada lewy bodies80% Parkinsonism Onset :
>50 TAHUN PUNCAK 70 an TAHUN
PRIA : WANITA = 3 :2Rata-rata 9 tahun meninggal tanpa
terapi
PENYAKIT PARKINSONPENYAKIT PARKINSON
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KLASIFIKASI PARKINSONISM Parkinsonism Idiopatik / Primer
= Penyakit Parkinson= Parkinsonism Simptomatik / Sekunder
Drugs Induced, Infectious, Head Trauma,Tumor/paraneoplastik, Vascular, Toxins,Metabolic, Hidrocephalus,Anoxic encephalopathy
Parkinsonism PlusDementia syndroma (Alzheimer’s Disease) ,Multiple System Atrophy, Normal PressureHydrocephalus, Progressive supranuclear palsy,Corticobasal ganglionic degeneration, Hereditarydisorders.
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Parkinson’s Disease
ETIOLOGY:
Etiology dari Penyakit Parkinson belumdiketahui secara pasti , namun ada beberapafaktor yang dapat meningkatkan Resikodari Penyakit Parkinson :
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Parkinson’s DiseaseFaktor yang dapat menyebabkanmeningkatnya 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)
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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TREMOR
RIGIDITY
AKINESIA
POSTURAL INSTABILITY
GEJALA UTAMA (TRAP)GEJALA UTAMA (TRAP)
PENYAKIT PARKINSONPENYAKIT PARKINSON
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Tremor
Resting tremor, postural tremor Kasar, 3-7 getaran / detik Tangan, kaki, badan, bibir
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Rigidity
Cogwheel phenomenon
Leher, Lengan, Tungkai, Badan
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Akinesia
Lama tidak bergerak atau lambat
(Bradikinesia)
Muka topeng, kurang berkedip
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Postural Instability
Mudah terjatuh, propulsi
Pada stadium lanjut
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Diagnosis
Dua dari 3 gejala kardinal (TRA)
Tiga dari 4 gejala utama (TRAP)
Responsif terhadap L-dopa
KRITERIA KLINISKRITERIA KLINIS
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KRITERIA HUGHES
POSSIBLETdpt salah satu gejala utama:Tremor istirahat.RigiditasBradikinesia.Kegagalan reflesk postural
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KRITERIA HUGHES
PROBABLEBila terdapat kombinasi dua gejala utama
( termasuk kegagalan refleks postural) DEFINITEBila terdapat kombinasi tiga dari empat
gejala.
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1. Preclinical stage – at this PD-spesificpathology is present with no clinicalsigns at all and potential imaging andbiomarker abnormalities may be ableto detect these changes
2. Pre-motor – extranigral pathologycausing symptoms
3. Motor PD – classical nigral pathology
TIGA FASE PARKINSONDISEASE
AAN 2012
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3535
GNM/Pre-motorPenyakit Parkinson (PP)
Yang disepakati sebagai gejala dini PPberdasar HistoPA dari Braak dkk:
Tolosa, E, et al. Neurology 2009 ( Suppl 2 ): S12-S20.
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3636
GNM lainnya
Gejala Non-Motor sebelum dan sesudahdx. PP ditegakkan, sbb.:Neuropsikiatri:
* gg. kognisi dan demensia* apatis, ansietas, panik* ilusi, delusi, halusinasi, dilirium* ICDs (impulse control disorders)* depresi.
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3737
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
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Progresifitas ParkinsonDisease
AAN 2012
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PERJALANAN PENYAKIT( Hoehn and Yahr )
Stadium 1: gejala dan tanda pada satusisi, terdapat gejala yg ringan, terdptgejala mengganggu ttp tdkmenimbulkan kecacatan, biasanyatremor pd satu anggota gerak, gejalayg timbul dapat dikenali orang terdekat.
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Stadium 2 : terdapat gejala bilateral,terdapat kecacatan minimal, sikap/caraberjalan terganggu.
Stadium 3 : gerak tubuh nyatamelambat, keseimbangan mulaiterganggu saat berjalan/berdiri,disfungsi umum sedang.
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Stadium 4 : terdapat gejala yang lebihberat, masih dpt berjalan hanya utkjarak tertentu, rigiditas danbradikinesia, tdk mampu berdiri sendiri,tremor dpt berkurang dibandingstadium sebelumnya.
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Stadium 5 : stadium kakhetik ( cacheticstage ) , kecacatan total, tidak mampuberdiri dan berjalan, memerlukanperawatan tetap.
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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Brook D.J: Neuroimaging in Parkinson’s Disease,Neuro RX 1: 243-254,2004
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Marek K et al: (123 I )B CIT Spect Imaging Assesment of the rate of Parkinson DiseaseProgression, Neurology 2001, 57:2089 -2054
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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NEUROPATOLOGI
DEGENERASI NEURON SUBSTANSIANIGRA.
o TERDAPAT LEWI BODY.
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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PENGOBATANFARMAKOLOGIS 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.
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PENGOBATANFARMAKOLOGIS DIBAGI: (2)
1.3 Faktor tropik.GDNF.GMI ganglioside.
1.4 Immunomodulator.NSAID.Immunophyllins.
1.5 Cyclosporine A, calpain inhibitor, caspase inhibitor.
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PENGOBATANFARMAKOLOGIS DIBAGI: (3)
DOPAMINERGIK.2.1 Prekursor dopamin.
Levodopa.Levodopa CRLevodopa cair.Obat obat menaikkan kadar levodopa.
o Levodopa + decarboxylase inhibitor.o Levodopa + COMT inhibitor.o Levodopa + selegilene.
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PENGOBATANFARMAKOLOGIS DIBAGI: (4)
2.2 Agonis dopamin.Derivat ergot: bromocriptin, pergolide, lisuride,cabergoline.Derivat non ergot: PRAMIPEXOLE, ropinirole,talipexole, apomorfin, selegiline.
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PENGOBATANFARMAKOLOGIS DIBAGI: (5)
NON DOPAMINERGIK.ANTIKOLINERGIK: Trihexyfenidyl, benstropin.GLUTAMAT ANTAGONIS: Amantadine, memantine.ADENOSINE A2A ANTAGONIS.GM 1 GANGLIOSIDE.
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ALGORITMAPENATALAKSANAAN
PENYAKIT PARKINSON
Konsensus POKDIGangguan GerakPERDOSSI 2003
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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UNIFIED PARKINSON’S DISABILITYRATING 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
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UNIFIED PARKINSON’S DISABILITYRATING 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.
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UNIFIED PARKINSON’S DISABILITYRATING SCALE ( UPDRS )
TESTING FUNCTIONAL PERFORMANCEIN PEOPLE WITH PARKINSONDISEASE
Definitif skor setiap item : 0 - 5 Total skor : 0 - 199 Skor 199 : Parkinson Berat.
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OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSON’S DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSON’S DISEASE
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UTOYO SUNARYO, NEUROLOGIST OF MOHAMAD SALEH MD HOSPITAL , PROBOL INGGO