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早发型重症肌无力

Early-onset myasthenia gravis.

作者信息

Roach E S, Buono G, McLean W T, Weaver R G

出版信息

J Pediatr. 1986 Feb;108(2):193-7. doi: 10.1016/s0022-3476(86)80981-7.

Abstract

Signs of myasthenia gravis developed by age 3 years in 11 children. Six of these patients had persistent neonatal myasthenia gravis, a familial abnormality of neuromuscular transmission that is not immunologically mediated. Five patients had juvenile onset myasthenia gravis, an autoimmune disorder similar to myasthenia gravis in adults. Autoimmune myasthenia has rarely been recognized by age 3 years, but the presence of five cases in our series suggests that the disorder may be more common in young children than once believed. The development of anti-acetylcholine receptor antibody assays makes it easier to distinguish autoimmune myasthenia gravis from the congenital forms. This distinction is important, because the prognosis, treatment, and risk of recurrence in family members is different for each type of myasthenia.

摘要

11名儿童在3岁前出现重症肌无力症状。其中6名患者患有持续性新生儿重症肌无力,这是一种非免疫介导的神经肌肉传递家族性异常。5名患者患有青少年型重症肌无力,这是一种与成人重症肌无力相似的自身免疫性疾病。自身免疫性重症肌无力在3岁前很少被认识到,但我们系列中有5例病例表明,这种疾病在幼儿中可能比以前认为的更常见。抗乙酰胆碱受体抗体检测方法的发展使得区分自身免疫性重症肌无力和先天性重症肌无力变得更容易。这种区分很重要,因为每种类型的重症肌无力在预后、治疗和家庭成员复发风险方面都有所不同。

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