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儿童兰伯特-伊顿肌无力综合征

Lambert-Eaton myasthenic syndrome in childhood.

作者信息

Kostera-Pruszczyk A, Ryniewicz B, Rowinska-Marcinska K, Dutkiewicz M, Kamińska A

机构信息

Department of Neurology, Medical University of Warsaw, Poland.

出版信息

Eur J Paediatr Neurol. 2009 Mar;13(2):194-6. doi: 10.1016/j.ejpn.2008.03.006. Epub 2008 Jun 30.

Abstract

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction. LEMS can be associated with a variety of neoplasms. Patients present with proximal muscle weakness and fatigability, often combined with areflexia. Only 5% of reported cases are children. We report a case of 11-year old boy with non-neoplastic Lambert-Eaton myasthenic syndrome. Repetitive nerve stimulation test showed 83% increment after maximal voluntary contraction, presence of antibodies against voltage-gated calcium channels confirmed the diagnosis. The boy responded well to immunosuppressive treatment with prednisone and azathioprine and remains cancer-free for 4 years.

摘要

兰伯特-伊顿肌无力综合征(LEMS)是一种罕见的神经肌肉接头自身免疫性疾病。LEMS可与多种肿瘤相关。患者表现为近端肌肉无力和易疲劳,常伴有反射消失。报告病例中仅5%为儿童。我们报告一例11岁非肿瘤性兰伯特-伊顿肌无力综合征男孩病例。重复神经电刺激试验显示最大自主收缩后增幅为83%,抗电压门控钙通道抗体阳性确诊了该疾病。该男孩对泼尼松和硫唑嘌呤免疫抑制治疗反应良好,且4年来一直未患癌症。

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