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What is your approach to managing seronegative myasthenia gravis in patients who have not responded to immunotherapy?

When would you consider an alternative diagnosis such as mitochondrial myopathy, and what is your stepwise approach?
1 Answer
Mednet MemberInvited Expert
Neurology · University of Minnesota
Answered on

The quick and easy answer is that the diagnosis must be reconsidered. To facilitate the discussion, I would divide the seronegative patients into two groups: ocular and generalized.

I assume that patients have been tested for AchR, MuSK antibodies (and possibly LRP4?), and the results are negative.

1.) If a patient has been diagnosed with seronegative generalized MG and they are not responding to immunotherapy, something is fundamentally wrong with the diagnosis. Repetition of electrodiagnostic studies is critical in this scenario - is there a decrement on RNS, and/or abnormal single fiber EMG? And if a technically flawless RNS study shows a decrement, what do the needle EMG and nerve conduction studies show?

  • A reminder that abnormal RNS ...

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