Severe Elevation of Creatine Phosphokinase and the Development of a Honeycomb Structure in a Patient with Progressive Spinal Muscular Atrophy

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A 22-years-old man was admitted to our hospital for evaluation of muscle weakness. At the age of 14, he noticed difficulty in running and climbing stairs due to muscle weakness; it progressed gradually with age. On admission, he displayed waddling gait, showed moderarely severe muscular atrophy, especially in proximal portion of the extremities, and had decreased tendon reflexes. Serum examination showed severe elevation of creatine phosphokinase. Muscle biopsy specimen revealed neuropathic and myopathic changes, and there were many necrotic fibers. Moreover, honeycomb structures were observed electronmicroscopically. It is extremely rare to observe a severely increased serum creatine phosohokinase activity more than ten time the normal level in progressive spinal muscular atrophy, and this is the first report of honeycomb structure in progressive spinal muscular atrophy. We suggested that elevation of creatine phosphokinase and honeycomb structures might be due to long administration of phentyoin sodium.
Yamaguchi University Graduate School of Medicineの論文