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伴有单特异性抗GT1a抗体的急性延髓、颈部和肢体无力:吉兰-巴雷综合征的一种罕见局限性亚型。

Acute bulbar, neck and limb weakness with monospecific anti-GT1a antibody: A rare localized subtype of Guillain-Barré syndrome.

作者信息

Garg Nidhi, Yuki Nobuhiro, Park Susanna B, Barnett Michael H, Kiernan Matthew C

机构信息

Brain and Mind Centre, Sydney Medical School, The University of Sydney, 94 Mallett Street Camperdown, NSW 2050, Australia.

出版信息

Muscle Nerve. 2016 Jan;53(1):143-6. doi: 10.1002/mus.24935. Epub 2015 Nov 23.

Abstract

INTRODUCTION

Acute bulbar, neck, and limb weakness carries several potential differential diagnoses. Although a diagnosis can often be established clinically, investigations such as electrodiagnostic and antibody testing can provide support for the clinical diagnosis and may aid in understanding the pathogenesis. A 65-year-old woman presented with acute bulbar, neck, and rapidly progressive bilateral upper limb weakness.

METHODS

Clinical evaluation, electrophysiological, and serological studies were undertaken.

RESULTS

Neurophysiology demonstrated proximal conduction block. A clinical diagnosis of pharyngeal-cervical-brachial weakness, a localized variant of Guillain-Barré syndrome, was made. The patient received treatment with intravenous immunoglobulin and made a remarkable recovery over the next month. She was found to have serum monospecific anti-GT1a antibodies.

CONCLUSIONS

We report a case of pharyngeal-cervical-brachial weakness with monospecific anti-GT1a antibodies and discuss the differential diagnosis of acute bulbar, neck, and limb weakness.

摘要

引言

急性延髓、颈部和肢体无力有多种潜在的鉴别诊断。尽管通常可通过临床诊断,但诸如电诊断和抗体检测等检查可为临床诊断提供支持,并有助于理解发病机制。一名65岁女性出现急性延髓、颈部及迅速进展的双侧上肢无力。

方法

进行了临床评估、电生理和血清学研究。

结果

神经生理学显示近端传导阻滞。临床诊断为咽颈臂肌无力,这是格林-巴利综合征的一种局部变异型。患者接受了静脉注射免疫球蛋白治疗,并在接下来的一个月内显著康复。发现她血清中有单特异性抗GT1a抗体。

结论

我们报告一例伴有单特异性抗GT1a抗体的咽颈臂肌无力病例,并讨论急性延髓、颈部和肢体无力的鉴别诊断。

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