PENYAKIT PARKINSON
UTOYO SUNARYORSUD Dr.MOHAMAD SALEH
KOTA PROBOLINGGO
PROBOLINGGO , 23 NOVEMBER 2005
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
MAKRO ANATOMI SUSUNANSARAF 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 PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
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)
CORTICAL MOTOR AREASCORTICAL MOTOR AREAS
THALAMUS BASALGANGLIA
CEREBELLUM
BRAINSTEM
SPINALCORD
THALAMUS BASALGANGLIA
CEREBELLUM
BRAINSTEM
SPINALCORD
Ganglia Basalis dalamMenciptakan Gerakan :Ganglia Basalis dalamMenciptakan Gerakan :
Stimulasi Korteks Motorik. Melalui pintu masuk putamen
(di Striatum)
Keluar melalui Globus Palidusinternus
Menciptakan jalur direk dan indirek.
Stimulasi Korteks Motorik. Melalui pintu masuk putamen
(di Striatum)
Keluar melalui Globus Palidusinternus
Menciptakan jalur direk dan indirek.
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
PATOFISIOLOGI PENYAKITPARKINSON
KetidakKetidak seimbanganseimbangan sarafsarafdopaminergikdopaminergik dandan kolinergikkolinergik
DA AK
NORMAL(AK=DA)
PENYAKIT PARKINSON(AK>DA)
AK = ASETIL KOLINDA = DOPAMIN
B R A I NB R A I NGanglia basalis
Acetylcholin Normal
Dopamin
Acetylcholin PD
Perokside Radical H TissueTissuedamagedamage
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
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
EPIDEMIOLOGIPrevalensi dari Penyakit Parkinson belum
diketahui 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.
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
Parkinsons DiseaseDefinisi by James Parkinson 1817 :
PenyakitParkinson Parkinsonisme
TRAP
1919
What is Parkinsons Disease?
Clinical syndrome manifestingcharacteristically with parkinsonism(bradikinesia, hipo/akinesia. rigidity,tremors, and postural instability),caused by significant dopaminergicstriatal denervation. (Wolters, 2007)
PARKINSONISM
SINDROMA :Tremor, Rigidity, Akinesia, Posturalinstability (TRAP)
MACAM-MACAM PENYEBAB
PENYAKIT PARKINSONPENYAKIT PARKINSONvsvs PARKINSONISMPARKINSONISM
Etiologi : IdiopatikDegenerasi neuron di substansia nigra &ada lewy bodies
80% Parkinsonism Onset :
>50 TAHUN PUNCAK 70 an TAHUN
PRIA : WANITA = 3 :2Rata-rata 9 tahun meninggal tanpaterapi
PENYAKIT PARKINSONPENYAKIT PARKINSON
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 (Alzheimers Disease) ,Multiple System Atrophy, Normal PressureHydrocephalus, Progressive supranuclear palsy,Corticobasal ganglionic degeneration, Hereditarydisorders.
Parkinsons DiseaseETIOLOGY:
Etiology dari Penyakit Parkinson belumdiketahui secara pasti , namun ada beberapafaktor yang dapat meningkatkan Resikodari Penyakit Parkinson :
Parkinsons DiseaseFaktor yang dapat menyebabkanmeningkatnya Resiko Penyakit Parkinson :
Aging , Gender ( Men) , and Ras ( White )Riwayat Keluarga :TraumaStress EmosionalPersonality ( Shyness and Depressiveness)Environmental ExposuresMetals ( Manganese , Iron )Drinking unwell waterFarmingHerbicide and Pepticide exposureMPTP ( methyl phenyl tetrahydropiridine)
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
TREMOR RIGIDITY AKINESIA POSTURAL INSTABILITY
GEJALA UTAMA (TRAP)GEJALA UTAMA (TRAP)PENYAKIT PARKINSONPENYAKIT PARKINSON
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
Dua dari 3 gejala kardinal (TRA)
Tiga dari 4 gejala utama (TRAP)
Responsif terhadap L-dopa
KRITERIA KLINISKRITERIA KLINIS
KRITERIA HUGHES
POSSIBLETdpt salah satu gejala utama:Tremor istirahat.RigiditasBradikinesia.Kegagalan reflesk postural
KRITERIA HUGHES
PROBABLEBila terdapat kombinasi dua gejala utama
( termasuk kegagalan refleks postural) DEFINITEBila terdapat kombinasi tiga dari empat
gejala.
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
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.
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.
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
Progresifitas ParkinsonDisease
AAN 2012
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.
Stadium 2 : terdapat gejala bilateral,terdapat kecacatan minimal, sikap/caraberjalan terganggu.
Stadium 3 : gerak tubuh nyatamelambat, keseimbangan mulaiterganggu saat berjalan/berdiri,disfungsi umum sedang.
Stadium 4 : terdapat gejala yang lebihberat, masih dpt berjalan hanya utkjarak tertentu, rigiditas danbradikinesia, tdk mampu berdiri sendiri,tremor dpt berkurang dibandingstadium sebelumnya.
Stadium 5 : stadium kakhetik ( cacheticstage ) , kecacatan total, tidak mampuberdiri dan berjalan, memerlukanperawatan tetap.
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
Brook D.J: Neuroimaging in Parkinsons Disease,Neuro RX 1: 243-254,2004
Marek K et al: (123 I )B CIT Spect Imaging Assesment of the rate of Parkinson DiseaseProgression, Neurology 2001, 57:2089 -2054
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
NEUROPATOLOGI
DEGENERASI NEURON SUBSTANSIANIGRA.
o TERDAPAT LEWI BODY.
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
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.
PENGOBATANFARMAKOLOGIS DIBAGI: (2)
1.3 Faktor tropik.GDNF.GMI ganglioside.
1.4 Immunomodulator.NSAID.Immunophyllins.
1.5 Cyclosporine A, calpain inhibitor, caspase inhibitor.
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.
PENGOBATANFARMAKOLOGIS DIBAGI: (4)
2.2 Agonis dopamin.Derivat ergot: bromocriptin, pergolide, lisuride,cabergoline.Derivat non ergot: PRAMIPEXOLE, ropinirole,talipexole, apomorfin, selegiline.
PENGOBATANFARMAKOLOGIS DIBAGI: (5)
NON DOPAMINERGIK.ANTIKOLINERGIK: Trihexyfenidyl, benstropin.GLUTAMAT ANTAGONIS: Amantadine, memantine.ADENOSINE A2A ANTAGONIS.GM 1 GANGLIOSIDE.
ALGORITMAPENATALAKSANAAN
PENYAKIT PARKINSON
Konsensus POKDIGangguan GerakPERDOSSI 2003
OUTLINE NEUROANATOMI. NEUROFISIOLOGI. NEUROPATOFISIOLOGI. NEUROEPIDEMIOLOGI BATASAN. GEJALA KLINIS. NEURORADIOLOGI. NEUROPATOLOGI. NEUROFARMAKOLOGI. UNIFIED PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
UNIFIED PARKINSONS 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
UNIFIED PARKINSONS DISABILITYRATING SCALE ( UPDRS ) cont
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 PARKINSONS DISABILITYRATING SCALE ( UPDRS )
TESTING FUNCTIONAL PERFORMANCEIN PEOPLE WITH PARKINSONDISEASE
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 PARKINSONS DISABILITY RATING SCALE
( UPDRS ) LIVING WITH PARKINSONS DISEASE
UTOYO SUNARYO, NEUROLOGIST OF MOHAMAD SALEH MD HOSPITAL , PROBOL INGGO