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一例儿童重症肌无力合并慢性炎症性脱髓鞘性多发性神经根神经病。

A case of myasthenia gravis in childhood associated with chronic inflammatory demyelinating polyradiculoneuropathy.

作者信息

Kimura K, Nezu A, Kimura S, Otsuki N, Kobayashi T, Nomura Y, Segawa M

机构信息

Yokohama City University School of Medicine, Japan.

出版信息

Neuropediatrics. 1998 Apr;29(2):108-12. doi: 10.1055/s-2007-973544.

Abstract

We report a 14-year-old girl who developed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) during the course of myasthenia gravis. Myasthenia gravis, which was clinically of ocular type, but with waning phenomenon of the extremities, occurred at 2 years and 4 months of age. Muscle weakness of the lower extremities gradually developed over the next 6 years. The electrophysiological and pathological findings fulfilled the criteria of "possible CIDP" with severe axonal involvement. The signs of myasthenia gravis and CIDP fluctuated synchronously. A common immunological abnormality was suggested to underlie this rare association of myasthenia gravis and CIDP in childhood.

摘要

我们报告一名14岁女孩,她在重症肌无力病程中出现了慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)。重症肌无力于2岁4个月时发病,临床为眼肌型,但伴有肢体无力减退现象。在接下来的6年里,下肢肌肉无力逐渐发展。电生理和病理检查结果符合“可能的CIDP”标准,且有严重的轴突受累。重症肌无力和CIDP的体征同步波动。提示一种共同的免疫异常是儿童期重症肌无力与CIDP这种罕见关联的基础。

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