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鉴别家族性神经病与吉兰-巴雷综合征。

Differentiating Familial Neuropathies from Guillain-Barré Syndrome.

作者信息

Bordini Brett J, Monrad Priya

机构信息

Department of Pediatrics, Section of Hospital Medicine, Nelson Service for Undiagnosed and Rare Diseases, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, USA.

Department of Child and Adolescent Neurology, Medical College of Wisconsin, Milwaukee, WI, USA.

出版信息

Pediatr Clin North Am. 2017 Feb;64(1):231-252. doi: 10.1016/j.pcl.2016.08.015.

Abstract

Differentiating Guillain-Barré syndrome (GBS) from inherited neuropathies and other acquired peripheral neuropathies requires understanding the atypical presentations of GBS and its variant forms, as well as historical and physical features suggestive of inherited neuropathies. GBS is typically characterized by the acute onset of ascending flaccid paralysis, areflexia, and dysesthesia secondary to peripheral nerve fiber demyelination. The disorder usually arises following a benign gastrointestinal or respiratory illness, is monophasic, reaches a nadir with several weeks, and responds to immunomodulatory therapy. Inherited neuropathies with onset before adulthood, whose presentation may mimic Guillain-Barré syndrome, are reviewed.

摘要

将吉兰-巴雷综合征(GBS)与遗传性神经病及其他获得性周围神经病区分开来,需要了解GBS的非典型表现及其变异形式,以及提示遗传性神经病的病史和体格检查特征。GBS的典型特征是急性起病的上行性弛缓性麻痹、腱反射消失,以及继发于周围神经纤维脱髓鞘的感觉异常。该疾病通常在良性胃肠道或呼吸道疾病后出现,为单相性,在数周内达到最低点,并对免疫调节治疗有反应。本文综述了成年前起病、表现可能酷似吉兰-巴雷综合征的遗传性神经病。

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