Neuromuscular transmission defect in inherited polyneuropathy.

Bulletin of clinical neurosciences Pub Date : 1985-01-01
J J Kelly, G A Baquis, L S Adelman, T L Munsat
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Abstract

A 51-year-old woman with an inherited polyneuropathy had fatiguability and a dramatic historical response to prostigmine. Repetitive motor nerve stimulation produced a prominent decrement of the compound muscle action potential in distal muscles with marked facilitation after brief exercise. Defective neuromuscular transmission paralleled the polyneuropathy in distribution and severity. We hypothesize that deficient release of acetylcholine by regenerating or degenerating nerve terminals likely caused the defect of neuromuscular transmission in this patient.

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遗传性多发性神经病的神经肌肉传递缺陷。
51岁女性,遗传性多神经病变,易疲劳,对前体素有明显的历史反应。重复性运动神经刺激使远端肌肉复合动作电位明显下降,且在短暂运动后易化。神经肌肉传导缺陷在分布和严重程度上与多发性神经病相似。我们推测,再生或退行性神经末梢导致乙酰胆碱释放不足可能导致该患者神经肌肉传递缺陷。
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