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Practice Current: How do you manage treatment of myasthenia gravis?

While recognizing each case is different, please choose the answer that best reflects your common practice and limit free text to strategies that are not included.

Estimated Time: 5 Minutes

CASE 1
A 21-year-old woman reports 3 weeks of intermittent double vision and eyelid drooping. She reports no other bulbar or focal weakness. On clinical exam she has ptosis on the left with sustained upgaze without ophthalmoparesis. She does not have fatigable weakness in the limbs. As part of her work-up she is found to have positive acetylcholine receptor antibodies.
1. What treatment would you recommend next based on her clinical status? (Check all that apply) *This question is required.
2. One month later she returns for follow-up and reports improvement in her ocular symptoms since initiation of pyridostigmine (60mg three times a day), but she has some difficulty lifting up her head at the end of the day and difficulty walking up the stairs. She reports no shortness of breath or any other bulbar complaints. What treatment would you recommend next based on her clinical status? (Check all that apply.) *This question is required.
  • * This question is required.
3. At this visit you have the results of a CT scan of her chest that reveals no thymic mass. Would you refer her for a thymectomy? *This question is required.
The patient was referred for thymectomy. What procedures are available to you at your institution? *This question is required.
What type of procedure would you like to offer this patient (regardless of whether or not you have access to it)?
4. The patient tolerated pyridostigmine (60mg three times a a day) and when she increases her prednisone to 40mg daily (she weighs 60kg), she begins to develop leg swelling, irritability, and weight gain (now weighs 65kg) after 2 weeks on this dose. She still has fatigue in her legs and a head drop at the end of the day. She is also more fatigued at work. She reports a nasal quality to her voice and some increased oral secretions. She reports no shortness of breath. Would you recommend changing the medication?
What would you do with the prednisone? *This question is required.
What would you do with the pyridostigmine? *This question is required.
Would you start a new treatment? (Check all that apply.) *This question is required.
Which steroid sparing medication would you choose? *This question is required.
CASE 2
A 35-year-old woman with acetylcholine receptor antibody MG s/p thymectomy, is in clinical remission on mycophenolate and pyridostigmine. She has been clinically stable on this regimen for the last 3 years. When she comes to her follow-up examination she discusses that she would like to start a family.
5. Would you recommend changing the medications? *This question is required.
What would you do with the mycophenolate? *This question is required.
What would you do with the pyridostigmine? *This question is required.
6. Based on the decision you made above, is there any other drug you would like to start the patient on? (Check all that apply.) *This question is required.
7. What mode of childbirth delivery would you advise for this patient? *This question is required.
8. The patient becomes pregnant and her MG remains under good control until 37 weeks gestation when she develops pre-eclampsia. What course of action would you recommend? (Check all that apply.) *This question is required.
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9. Which of these treatments would you NOT RECOMMEND in your practice for this patient with myasthenia in pre-eclampsia? (Check all that apply.) *This question is required.
CASE 3
A 27-year-old woman comes to your office for evaluation of progressive difficulty speaking and coughing after she eats over the last 4 months. Examination reveals dysarthria, tongue atrophy, and limitation of tongue movements. She has no ocular findings, no fluctuating limb weakness, and no pathological reflexes. She brought in outside serum studies that revealed negative acetylcholine receptor antibodies and a normal CPK. EMG/NCS reveals normal repetitive nerve stimulation in the extensor indicis proprius, trapezius, and nasalis. Pyridostigmine trial results in worsening dysphagia and muscle twitching, and it's stopped.
10. What would be your next course of action? Check all that apply. *This question is required.
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Her single fiber EMG study reveals jitter in the frontalis muscle and she is found to have positive muscle specific tyrosine kinase antibodies. Chest imaging reveals a normal thymus gland. What treatment would you initiate based on her clinical status? Check all that apply. *This question is required.