ppt myasthenia winda

7/26/2019 Ppt Myasthenia Winda

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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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