Muscle-specific tyrosine kinase-antibody-positive myasthenic crisis with detailed electrophysiologic studies.

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A 69-year-old male who presented in a coma due to sudden respiratory arrest was transferred to our hospital. After endotracheal intubation with manual ventilation, he became alert and his neurologic findings were within the normal range, except for palsy of the respiratory muscles. Biochemical analyses of the blood and brain computed tomography failed to indicate the cause of the respiratory arrest. An edrophonium test did not improve the respiratory arrest. An urgent electromyogram at the dorsal interossei, biceps, and sternocleidomastoideus muscle and a repetitive nerve stimulation test at the trapezius and deltoid muscle were also negative on the first hospital day. However, on the 16th day in the hospital, a repetitive nerve stimulation test at the levator labii superioris alaeque nasi showed a waning phenomenon. This result indicated a diagnosis of myasthenia gravis. Anti.muscle-specific tyrosine kinase antibody was found to be positive. After treatment with plasmapheresis and prednisolone, he regained normal respiratory function. Anti.muscle-specific tyrosine kinase (MuSK)-antibodypositive myasthenia gravis (MG) (MuSK-MG) tends to be associated with a lower incidence of a positive edrophonium test, a lower incidence of a positive electrophysiologic study excluding the face, and a higher incidence of respiratory failure in comparison to anti.acetylcholine receptors (AchR)-antibody-positive MG (AchR-MG). Respiratory failure is curable with treatment. Accordingly, in addition to obtaining a precise diagnosis, an emergency physician should recommend an electrophysiologic study including the face to make a differential diagnosis for respiratory arrest when biochemical and radiologic studies fail to indicate the cause of the respiratory arrest.

Muscle-specific tyrosine kinase-antibody-positive myasthenic crisis with detailed electrophysiologic studies.

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