Meier C, Kauer B, Müller U, Ludin H P
J Neurol. 1979 Jan 5;220(4):231-9. doi: 10.1007/BF00314147.
Clinical, electrophysiological, and the nerve and muscle biopsy findings from a case treated with amiodarone are reported. Marked distal motor and sensory impairment and distal muscular atrophy were observed clinically. The electrophysiological examination revealed normal motor and sensory conduction velocities in the median nerve; the sensory action potentials were polyphasic and reduced in amplitude. Electromyography revealed denervation potentials and severe loss of motor units in the M. extensor digitorum brevis and in the M. tibialis anterior. The light and electronmicroscopical study of a N. suralis biopsy displayed total loss of large myelinated fibers and an almost total reduction of small myelinated fibers. The number of unmyelinated axons was markedly reduced. Fibrocytes and degenerative axons polymorphous inclusion bodies were present in Schwann cells. The muscle biopsy revealed both neurogenic and myopathic changes. Lipid storage was also present in the muscle fibers. Physical and chemical analysis of the nerve and muscle biopsy revealed the content of iodine to be more than 40 times increased. The findings indicate damage of axons, schwann cells and muscle fibers. It is suggested that the lipid storage in nerve and muscle tissue might be related to the accumulation of the drug or its metabolites.
报告了一例接受胺碘酮治疗患者的临床、电生理以及神经和肌肉活检结果。临床观察到明显的远端运动和感觉障碍以及远端肌肉萎缩。电生理检查显示正中神经运动和感觉传导速度正常;感觉动作电位多相且波幅降低。肌电图显示趾短伸肌和胫前肌有失神经电位和运动单位严重丧失。腓肠神经活检的光镜和电镜研究显示大的有髓纤维完全丧失,小的有髓纤维几乎完全减少。无髓轴突数量明显减少。施万细胞内有纤维细胞和变性轴突多形性包涵体。肌肉活检显示有神经源性和肌病性改变。肌纤维中也存在脂质蓄积。对神经和肌肉活检的理化分析显示碘含量增加了40多倍。这些结果表明轴突、施万细胞和肌纤维受损。提示神经和肌肉组织中的脂质蓄积可能与药物或其代谢产物的蓄积有关。