Spinocerebellar degeneration secondary to chronic intestinal malabsorption: A vitamin E deficiency syndrome
Spinocerebellar degeneration secondary to chronic intestinal malabsorption: A vitamin E deficiency syndrome
复制标题
慢性肠道吸收不良继发的脊髓小脑变性：维生素 E 缺乏综合征
作者：
MD A. E. Harding;PhD D. P. R. Muller;DScJ P. K. Thomas;MB H. J. Willison;D. Harding
两个成年人描述谁开发了一个渐进的神经系统疾病超过20年后，慢性脂肪吸收不良的发病。临床特征包括构音障碍、小脑性共济失调和明显的本体感觉丧失伴腱反射减弱或消失。血清维生素E在这两种情况下都检测不到。1例患者在口服维生素E治疗后临床和电生理改善。这些患者的结果与最近报道的其他维生素E缺乏伴胆道闭锁患者的结果相似。电生理学观察表明，人类缺乏状态与维生素E缺乏动物的神经病理学发现相似。
Two adults are described who developed a progressive neurological disorder more than 20 years after the onset of chronic fat malabsorption. The clinical features included dysarthria, cerebellar ataxia, and prominent proprioceptive loss with depressed or absent tendon reflexes. Serum vitamin E was undetectable in both cases. One patient improved clinically and electrophysiologically after oral therapy with vitamin E. The findings in these patients were similar to those in others recently reported with vitamin E deficiency associated with biliary atresia. Electrophysiological observations suggested that the human deficiency state parallels that found neuropathologically in vitamin E‐deficient animals.