Monday, June 1, 2009

Pediatric neuromuscular junction disorders

1. How does juvenile myasthenia present?
2. How is junenile myasthenia diagnosed?
3. What does repetitive nerve stim show in autoimmune myasthenia gravis?

Answers:
1. Often in adolescent girls, often severe, with ptosis and ophthalmoplegia, weakness in face, jaw, swallowing, speech, respiration, proximal muscles.
2. Fatigability of muscle after stim of nerve at 4-10 Hz, response to Tensilon, single fiber EMG.
3. Decrement in CMAP with slow stimulation greater than 12-15%.

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