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吉兰-巴雷综合征合并重症肌无力。

Combined Guillain-Barré syndrome and myasthenia gravis.

作者信息

Hsieh Meng-Ying, Chan Oi-Wa, Lin Jainn-Jim, Lin Kuang-Lin, Hsia Shao-Hsuan, Wang Huei-Shyong, Chiu Cheng-Hsun

机构信息

Division of Pediatric Neurology, College of Medicine, Chang Gung University, Taoyuan, Taiwan.

出版信息

Brain Dev. 2013 Oct;35(9):865-9. doi: 10.1016/j.braindev.2012.10.016. Epub 2012 Nov 22.

Abstract

BACKGROUND

Guillain-Barré syndrome and myasthenia gravis both lead to muscle weakness but the two combined is uncommon. Detection of these entities can help identify forms of autoimmune neuromuscular diseases that may respond to immunotherapy. This report sought to characterize the clinical features of these two entities when combined.

METHODS

This report is of a case of combined Guillain-Barré syndrome and myasthenia gravis. The clinical features were analyzed and correlated to those published in English literature from 1960 to 2012. Ten reports and 12 cases, including the present case, were reviewed.

RESULTS

There were 12 patients (4 women and 8 men), aged 17 to 84 years, with combined Guillain-Barré syndrome and myasthenia gravis. Four had post-infectious Guillain-Barré syndrome followed by the development of myasthenia gravis concurrently or concomitantly within one month. All cases had symptoms of ptosis and areflexia. The other common presentations were limb weakness, oculobulbar weakness, and respiratory involvement. Functional outcome was mentioned in 10 patients and seven had good outcome (Hughes scale ≤ 2).

CONCLUSION

Detection of ptosis with or without ophthalmoplegia, distribution of limb weakness, and reflex can help in recognizing combined Guillain-Barré syndrome and myasthenia gravis. The early recognition of this combination of peripheral nervous and neuro-muscular junction inflammation is important for initial treatment and prognosis.

摘要

背景

吉兰 - 巴雷综合征和重症肌无力均可导致肌肉无力,但二者合并出现的情况并不常见。对这些疾病的检测有助于识别可能对免疫治疗有反应的自身免疫性神经肌肉疾病形式。本报告旨在描述这两种疾病合并时的临床特征。

方法

本报告为一例吉兰 - 巴雷综合征合并重症肌无力的病例。分析其临床特征,并与1960年至2012年英文文献中发表的特征进行对比。回顾了包括本病例在内的10篇报告及12个病例。

结果

共有12例患者(4名女性和8名男性),年龄在17至84岁之间,患有吉兰 - 巴雷综合征合并重症肌无力。4例患者为先发性感染性吉兰 - 巴雷综合征,随后在1个月内同时或相继出现重症肌无力。所有病例均有上睑下垂和腱反射消失的症状。其他常见表现为肢体无力、眼外肌麻痹和呼吸受累。10例患者提及了功能转归情况,其中7例转归良好(休斯量表评分≤2)。

结论

检测有无上睑下垂及眼肌麻痹、肢体无力的分布情况和反射,有助于识别吉兰 - 巴雷综合征合并重症肌无力。早期识别这种周围神经与神经肌肉接头炎症的组合对于初始治疗和预后很重要。

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