单独维生素E缺乏导致脱髓鞘性神经病  

Isolated vitamin E deficiency with demyelinating neuropathy

作　　者：Puri V. Chaudhry N. Tatke M. Prakash V. 周永 

机构地区：[1]Dr. Department of Neurology, G. B. Pant Hospital, New Delhi 110002, India

出　　处：《世界核心医学期刊文摘（神经病学分册）》2005年第11期54-55,共2页Digest of the World Core Medical Journals:Clinical Neurology

摘　　要：A 22-year-old man, with a past history of generalized tonicclonic seizures treated with phenobarbital, presented with spinocerebellar ataxia. The electrophysiological studies revealed a demyelinating motor-sensory neuropathy. The serum vitamin E level was low. Sural nerve biopsy revealed loss of large myelinated fibers with evidence of remyelination. Vitamin E supplementation led to clinical and electrophysiological recovery of sensory conduction and evoked potentials. Motor nerve conduction, however, showed only partial recovery. Vitamin E deficiency leading to a demyelinating neuropathy, as in the present case, suggests that the full spectrum of the disease entity is not fully defined.A 22-year-old man, with a past history of generalized tonicclonic seizures treated with phenobarbital, presented with spinocerebellar ataxia. The electrophysiological studies revealed a demyelinating motor-sensory neuropathy. The serum vitamin E level was low. Sural nerve biopsy revealed loss of large myelinated fibers with evidence of remyelination. Vitamin E supplementation led to clinical and electrophysiological recovery of sensory conduction and evoked potentials. Motor nerve conduction, however, showed only partial recovery. Vitamin E deficiency leading to a demyelinating neuropathy, as in the present case, suggests that the full spectrum of the disease entity is not fully defined.

关 键 词：脱髓鞘性 神经传导 腓肠神经活检 阵挛 电生理研究 电生理学 诱发电位 临床症状 

分 类 号：R744[医药卫生—神经病学与精神病学]