ppt myasthenia winda
TRANSCRIPT
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MYASTHENIA GRAVIS
By: Winda Diah Nugraheni
Lecturer Advier: Dr! D"nny H! Ha#id S$S
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Definition
Myasthenia gravis is a disorder of neuromuscular
transmission, characterised by weakness and fatiguing of
some or all muscle groups. Weakness worsening onsustained repeated exercise and relieved by rest. This
condition is a consequence of an autoimmune destruction
of the post synaptic reseptor for acetylcholine.
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Anat"#y "%
Neur"#ucu&ar Tran#ii"n
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'hyi"&"gy "% Neur"#ucu&ar
Tran#ii"n
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E$ide#i"&"gy
The prevalence of autoimmune M! is estimated at "
case in "#.###$%#.### people
Women are affected more often in the second and third
decades of life, and men more often in the fifth and sixthdecades.
The peak age of onset is between %# and years in
women and between '# and (# years in men.
)ssociated autoimmune diseases are present inapproximately '* of patients, and comorbid thyroid
disease occurs in more than "#*.
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'ath"genei
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'ath"genei
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+linical Manifestations
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+lassifications
. -cular myasthenia "' to %# percent/
.A. Mild generali0ed myasthenia with slow progression, no crises, drug1
responsive percent/
. B. Moderately severe generali0ed myasthenia2 severe skeletal and bulbar
involvement but no crises, drug response less than satisfactory %'percent/
. )cute fulminant myasthenia2 rapid progression of severe symptoms
with respiratory crises and poor drug response, high incidence of
thymoma, high mortality "' percent/.
3. 4ate severe myasthenia2 symptoms same as , but resulting from
steady progression over % years from class to class "# percent/.
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Diagn"i
'hyica& E(a#inati"n". +ranial nerve signs and symptoms5
-cular involvement produces ptosis
and muscle paresis.
Weakness of 6aw muscles allows themouth to hang open
Weakness of facial muscles results in
expressionless appearance
-n smiling, buccinator weakness
produces a characteristics smilemyasthenia snarl/
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%. 7ulbar involvement may
result in5
Dysarthric, dysphonic
speech and dysphagia 8asal regurgitation of fluids
or nasal quality to speech
&. The demonstration of
fatiguing 5
1 9impson test
1 +ogan:s lid twitch sign1 7lowing out cheeks
against pressure
1 ) counting test "##5
+ounting as far aspossible in one breathe
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9impson test
; ;
+ogan:s lid twitch sign
; ;
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atigue may be demonstrated bymovement against a constant resistance.
4imb reflexes are often hyperactive and fatigue on
repeated testing.
Muscle wasting occurs in "'* of cases.
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Diagnostic 9tudies
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Di%%erentia& Diagn"i
>or generali0ed M! ; the differential diagnosis includes
4ambert1?aton myasthenic syndrome, botulism, and
myopathy
>or ocular myasthenia ; alternative diagnoses include
progressive external ophthalmoplegia, thyroid disease,
and oculopharyngeal muscular dystrophy
>or bulbar predominant myasthenia gravis ; Motor
neuron disease, brainstem stroke, diphtheria, and
botulism
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Treat#ent
The treatment of this disease involves the careful use
of two groups of drug1anticholinesterases andimmunosuppresants including corticosteroids and in
special acute circumstances, plasma exchange and
intravenous immunoglobulin, an elective thymectomy is
appropriate in many patients as discussed below
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". )nticholinesterase Drugs
The two drugs that give the best results in
ameliorating myasthenia weakness are
neostigmine and pyridostigmine The usual dose of pyridostigmine is to @#
mg given every ( h typically a (# mg pill is
tried first/
The oral dose of neostigmine ranges from A.'
to
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%. +orticosteroids
The usual form of corticosteroid therapy is
prednisone or corresponding doses of
prednisone/, beginning with "' to %# mgBd andincreasing the dose gradually until a statisfactory
clinical response is obtained or until a daily dose
of '# to (# mg is reached.
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)! '&a#a E(change and Interven"u I##une G&"*u&ine
9triking temporary remissins % to C weeks/ may be
obtained by the use of plasma exchange. This formof treatment may be life saving during a myasthenic
crisis.
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