Myastenia Grafis
Transcript of Myastenia Grafis
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Myastenia Grafisdr. Neimy Novitasari Sp.S
RSU Haji Surabaya
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Myastenia GrafisPenyakit autoimun yang mengakibatkan gg
imunologis pada reseptor Ach pascasinap (NMJ)
Terutama mengenai nukleus motorik batang otak ( mata, pengecapan, wajah, nelan dan lidah )
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Myastenis GravisPrevalensi :
USA : 14/100.000 pendudukUsia 15 – 30 wanita, laki laki usia >40 tahunDekade 2 - 3
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ImunopatogenesisPengaruh genetik ( HLA B6 & B12 )Mekanisme Ab menyebabkan penurunan
jumlah reseptor Ach1. ↑degradasi reseptor Ach2. Blokade reseptor Ach3. Kerusakan sambungan saraf – otot
Komplek komplemen rusak Pedangkalan & ↓ lipatan post sinap
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Manifestasi klinik50 – 60 % :
lemah otot extraoculi & levator palpebra ( diplopia & ptosis ) memberat saat sore dan membaik dengan istirahat
Kesulitan bicara : gg artikulasi Sulit menelan, mengunyah, sulit bersiul
regurgitasi lewat hidung, Lemah otot leher sulit mempertahankan posisi
kepalaLemah umum otot ekstrimitas
atas,respirasi,badan
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Manifestasi klinik : kriteria Osserman
I : Ocular myastenia : ptosis & diplopiaIIA : Mild generalized myasteniaIIB : Moderate generalized myasteniaIII : Severe generalized + kompl bulbar & respirator
Akut fulminating : permulaan cepat, lemah hebat otot nafas, progresifitas komplit dlm 6 bulan
Late severe : 2 tahun setehan kel I & II, progresifitas pelan , respon obat & prognosis jelek
IV :Myastenic crisis : o.k
Kerja fisik, infeksi, melahirkan,obat blok Nm ( streptomisin, neomisin,kurare, kinin, morfin, enema, muscle relaxan )
Cholinergic crisis : o.k tx antikholinesterase Diare, Miosis, Bronkospasme, Emesis, Lacrimasi, Hipersalivasi
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Dx Kelumpuhan berulang yang membaik dengan
istirahat, melemah saat aktivitasGx……………………………
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Pemeriksaan FisikTujuan : membuktikan kecepatan kelelahan otot 1. Fisik :
MotorikTes pita suara : hitung / baca keras 3 menitTes keping es : pada mata ptosis 2 menit perbaikan ptosis
2. Pemeriksaan tambahan EMNG : Repetitive Nerve StimulationSingle fiber EMGTes farmakologi :
Tes prostigmin Titer Ab Ach reseptor ( N < 0,1 nmoles/ml )
CT Scan Thorax : Timoma
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PenyulitKrisis MyastenicKrisis KolinergikPneumonia
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Diagnosa bandingGBSNeuropati PeriferPolimiositisBotulism ( pre sinap )LEMS (ca paru, kelainan pre sinaps NMJ)Periodic paralisaSindroma myastenik ( kel kongenital, def
sambungan NMJ, - AbAcher, -Ig G )
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Therapy1. Perawatan umum :
Hindari lelah, tidur, menutup mata beberapa menit, hidari stress, suhu terlalu dingin / panas
2. Anti AcheProstigmin inj s.cPyridostigmin bromide tablet : 3x60 mg ↑
3. Imunosupresan : azatriopin, cyclosporin4. IVIG5. Plasmafaresis6. Timektomi : indikasi ( timoma, General MG tak
terkontrol dengan obat )
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TerapyKrisis Myastenia
ABCAnti AchePlasmafaresis IVIG
Krisis KolinergikABCHentikan Anti AcheAtropinisasi 0,4
mg/0,6 mg tiap 15 menit sampai pupil dilatasi – bronkospasme berkurang
PlasmafaresisIVIG