disorder of neuromuscular junction

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*


2/25

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


3/25

8ani a 9 pria)isa er5adi pada semua usia

ETIOLOGI

Idiopatik

D penicillamine

Terapi Alfa-Interferon


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6/25

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


7/25

DIAGNO,I,

Anamnesa

Gejala TemuanKlinis

3emeriksaan

Edrop.oniumtest

Acet.+lcolinreceptorsanti%odies

4u,Kanti%odies

E4G


8/25

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


10/25

;nit Dose A7era'e dose &adult(3+ridosti'mine%romide ta%let


11/25

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


12/25

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


13/25

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


14/25

!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


15/25

/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


16/25

). 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.


17/25

,+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'


18/25

,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


21/25

. )$!U 'S* ma anan +ang er /n aminasi

b/ ulinum

A, ) dan " B seaf//der /n aminasi


22/25

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

disorder of neuromuscular junction

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