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8/19/2019 Disorder of Neuromuscular Junction
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Disorder ofNeuromuscular Junction
Pembimbing :dr. Agus Permadi, Sp.S
Hanifah Khalid61110027
FAKU !AS K"#$K!"%A& U&'("%S'!AS)A!A*
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A. *+as enia -ra is -angguan Au / imun +ang
disebab an /leh adan+a
au /an ib/dies spesifi un uresep /r ase il /lin manusia
A h%3, +ang er /nsen rasi di
4ila+ah p/s sinapsis darineur/mus uler 5un i/n
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8ani a 9 pria)isa er5adi pada semua usia
ETIOLOGI
Idiopatik
D penicillamine
Terapi Alfa-Interferon
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Gejala Klinis
2 ! diplopia2 ! ptosis"#! Gejala $ul%ar &disartria ataudisfa'ia("#! 'an''uan %erjalan"# ! 'enerali)ed *eakness" ! ke'a'alan pernafasan
Kondisi +an' memper%erat,tres fisikstres emosionalInfeksipaparan o%at +an' men''an''u transmisi neuromuskuler
&perioperatice suksinilkolin anti%iotik amino'l+c.osidekina /uinidine %otulinum to0in(1
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DIAGNO,I,
Anamnesa
Gejala TemuanKlinis
3emeriksaan
Edrop.oniumtest
Acet.+lcolinreceptorsanti%odies
4u,Kanti%odies
E4G
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Pr/gn/sisSe i ar 1 ;20< pasien a an e ap
mengalami ge5ala / ular. *- $ ular3
%emisi sp/n an 10;1 <Sisan+a mengembang an
elemahan umum generali=ed *-3
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TE5A3I
6irst Line
T.+mectom+
kortikosteroidIn.i%itorKolinesterase
3en'o%atan den'ano%at
immunosuppressi7e
4+cop.enolate 4ofetil &2 #-## m' 20 se.ari(
A)at.ioprine & # m'8.ari" # m'8.ari(9+closporin &dosis a*al :-
m'8k'8.ari(
Immuno'lo%ulinintra7ena dosis
tin''i
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;nit Dose A7era'e dose &adult(3+ridosti'mine%romide ta%let
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Ped/man *ana5emen *-
3astikan dia'nosis
Edukasi 3asien
Kapan Barus Dira*at
4+ast.enic crisis
9orrect an+ underl+in'medical pro%lems
O%at mence'a. 4G
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4+ast.enic crisis 9.oliner'ic crisis
5espirator+ distress A%normal cramps
5espirator+ arest Diarr.ea
9+anosis Nausea and 7omitin'
Increased pulse and %lood pressure E0cessi7e secretion
Diap.oresis 4iosis
3oor cou'. 6asciculations
Ina%ilit+ to .andle oral secretion Diap.oresis
D+sp.a'ia Ceakness
Ceakness Corse *it. edrop.onium
Impro7e *it. edrop.onium
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Ped/man erapi spesifiKelema.an rin'an lokal atau
umum 9EI p+ridosti'mine
Kelola d'n 9EI
Immunosuppression
3lasma e0c.an'e atau I IG
4+c.op.enolatea)at.ioprine c+closporin
9.est 9T scan
5ujukan ke neurolo'ist
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!ransien &e/na al *+as henia!er5adi se i ar 10<'bu dengan *- au /imun ba+i :
angisan > menghisap lemah, fl/pp+-e5ala berangsur menghilang dalam
beberapa minggu
Parah P+rid/s igmin /ral 1;2mg? g?@ 5am
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/ngeni al *+as enia-angguan neur/mus uler 5un i/n
+g Heredi er
&/nimun/l/gi /mbinasi .@. diamin/p+ridine >
p+rid/s igmin
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). amber ;ea /n S+ndr/mePen+a i presinap i , dapa di ai an
eganasan
)erbeda dg *- 5umlah esi elase il /lin +ang dilepas an membranepresinaps ser a resp/n membranp/s sinaps n/rmal, e api di emu anpenurunan pelepasan esi el saa er5adip/ ensial a si.
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,+mptoms
3ro0imal lim% *eaknessLe's arms
6ati'ue or fluctuatin' s+mptoms
Difficult+ risin' from a sittin' position clim%in' stairs
4etallic taste in mout.
Autonomic d+sfunction Dr+ mout. 9onstipation $lurred 7ision Impared s*eatin'
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,i'ns
3ro0imal lim% *eaknessLe's arms
Ceakness on e0am is less compared to patient s le7el ofdisa%ilit+
B+poacti7e or a%sent muscle strec. refle0es
Lam%ert s si'n &'rip %ecomes more po*erful o7er se7eralseconds(
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3emeriksaan
3ro0imal lo*er e0tremit+ *eakness3emeriksaan le%i. rin'an dari .istoripasien14uscle strec. refle0 a%sentLam%ert s si'n
Dia'nosis Konfirmasi E4G
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3en'o%atan
Ke%er.asilan pen'o%atan tumor 3er%aikan 'ejalap+ridosti'mine: = diaminodip+ridine &DA3( "#m' tiap =-< jam "## m'8.ariImmunosupressi7e kortikosteroid sesuai tin'katkepara.an 'ejala
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. )$!U 'S* ma anan +ang er /n aminasi
b/ ulinum
A, ) dan " B seaf//der /n aminasi
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Klinis
"2-=> jam setela. makanmakanan terkontaminasidiplopia ptosis pen'li.atanka%ur d+sart.ria dan disfa'iakelema.an pada tun'kai atas dankemudian di tun'kai %a*a.Ke'a'alan napasDisfun'si otonom constipasipupil dilatasi mulut kerin'
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Dia'nosis
compound motor action
potential&94A3(395ELI,A
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3enatalaksanaan
4onitorin' 6un'si 3aruIntu%asi jika perlutri7alent %otulinum antito0in
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TE5I4A KA,IB