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6/28/091
MYASTHENIA GRAVIS
Pakamas Pasogpakdee,MD
6/28/09
!
 
Introduction
•
 
The most common primary disorder of neuromusculartransmission•
 
Usual cause is an acquired immunological abnormality , somecases result from genetic abnormality at the NMJ•
 
Women : 2
nd
and 3
rd
decades , Men : 6
th
decades•
 
USA : M>F (5
th
decades)
Neurology in Clinical Practice , 5
th
Edition , 2008
 
6/28/09
!
Immunopathology of MG
LEMS
!
6/28/09
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•
 
80% of MG pts. , weakness result from the effects of circulating anti-AChR Ab (T cell dependent)•
 
destruction of the folds , accelerated internalization & destruction of AChR , block Ach-AchR binding•
 
10% of MG pts. have circulating Ab to MuSK •
 
Remaining = seronegative
Neurology in Clinical Practice , 5
th
Edition , 2008
 
Immunopathology of MG
6/28/09
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Immunopathology of MG
 AChR Ab +ve
 AChR Ab neg AntiMUSK 
 Antititin Ab to ryanodine receptor
 AntiRapsyn
6/28/09
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Clinical Presentation
•
 
65%
ptosis & diplopia•
 
15%
difficult chewing , swallowing , talking•
 
10%
limb weakness•
 
rare
single muscle group weakness•
 
Typically fluctuates during the day , usually being least in themorning and worse as the day progresses , esp. after prolongeduse of affected muscles
Neurology in Clinical Practice , 5
th
Edition , 2008
 
6/28/09
!
 
6/28/092
Clinical Presentation
•
 
Careful questioning often reveals evidence of earlier myasthenicmanifestration•
 
- frequent purchases of new eyeglasses•
 
- avoidance of foods that became difficult to chew or swallow•
 
- cessation of activities that require prolonged use of specificm.•
 
- friends may have noted a sleepy or sad facial appearance•
 
caused by ptosis or facial weakness
Neurology in Clinical Practice , 5
th
Edition , 2008
 
6/28/09
!
Physical Findings
•
 
pattern of muscle involvement•
 
Ocular muscle•
 
- eye movements•
 
- Eye lid•
 
Oropharyngeal muscle•
 
- "myasthenic snarl“•
 
- nasal speech , difficulty chewing , difficulty swallowing•
 
choking on liquids
 
!
•
 
Limb muscle•
 
- Limb muscle weakness , Fatiguability
Neurology in Clinical Practice , 5
th
Edition , 2008
 
6/28/09
!
 
EYE & MG
•
 
Ptosis•
 
- usually asymmetrically•
 
- allow soap or water in the eyes during bathing•
 
Diplopia•
 
- Asymmetric weakness of several muscle in both eyes•
 
- Pattern of weakness is not characteristic of lesions of one or more nerves•
 
- Pupillary responses are normal•
 
- Weakness is most frequent & usually most severe inMR •
 
should raise suspicion of MG in the combination of ptosis ,ophthalmoparesis , weak eye closure
Neurology in Clinical Practice , 5
th
Edition , 2008
 
6/28/09
!
Eyelid manifestation
•
 
Levator palpebrae•
 
- Ptosis - Upper eyelid retraction•
 
- Lid fatigue test - Enhancing ptosis•
 
- Cogan lid twitch sign - Ice test•
 
- Sleep test - Rest test•
 
Orbicularis oculi muscle•
 
- Forced eye closure: buried eyelashes•
 
- Open the eyes against forced eyelid closure
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6/28/09
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Upper eyelid retraction
Left upper eyelid retraction contralateral to a ptotic rightupper eyelid
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Lid fatigue test
(A)
 
On initial upgaze, minimal left upper eyelid ptosis is evident(C)
 
 After 2 minutes of sustained upgaze, the degree of leftupper eyelid ptosis is significantly increased owing to levatormuscle fatigue
6/28/09
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Cogan lid twitch sign
•
 
looks down for at least 10 to 20 seconds
makesan upward saccade back to primary gaze•
 
transient overshoot of the upper eyelid , which maybe followed by nystagmoid twitches of the uppereyelid and then downward drifting of the eyelid to anormal or ptotic position
6/28/09
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Ice test
Preceding the test An ice pack is applied to theclosed left eye for 2 minutesRight upper eyelid ptosis issignificantly improved
6/28/09
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Sleep test
(A) before the test(B) After 30 minutes of sleep(lying down in a quiet and dimlylit room), the ptosis isnoticeably improved in botheyes
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Rest test
(A) Before rest (B) After only 5 minutes of gentle eye closure, the ptosisis much improved in both eyes
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bilateral lagophthalmosNot “bury” the eyelashes duringforced eye closure
Orbicularis oculi muscle
severe bilateral orbicularis weaknesssecondary lower eyelid retractionweak orbicularis muscle in the right eye duringforced eye closure
6/28/09
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