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肌萎缩侧索硬化症与重症肌无力重叠综合征:两例病例及相关文献综述

Amyotrophic Lateral Sclerosis and Myasthenia Gravis Overlap Syndrome: A Review of Two Cases and the Associated Literature.

作者信息

Tai Hongfei, Cui Liying, Guan Yuzhou, Liu Mingsheng, Li Xiaoguang, Huang Yan, Yuan Jing, Shen Dongchao, Li Dawei, Zhai Feifei

机构信息

Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.

Neuroscience Center, Chinese Academy of Medical Sciences, Beijing, China.

出版信息

Front Neurol. 2017 May 22;8:218. doi: 10.3389/fneur.2017.00218. eCollection 2017.

Abstract

OBJECTIVE

To describe the characteristics of patients with amyotrophic lateral sclerosis (ALS) and myasthenia gravis (MG) overlap syndrome and explore the relationship between the two diseases.

METHODS

We conducted a search of medical records at Peking Union Medical University Hospital from 1983 to 2015 for coexistence of ALS and MG and searched the PubMed database for all literature describing ALS and MG overlap syndrome published through December 2016. We analyzed the clinical and neurophysiological characteristics of patients by groups according to strict diagnostic criteria.

RESULTS

We presented 2 patients in our database with combined ALS and MG, and together with 25 cases reported in the literature, the patients were divided into 4 groups: 12 patients with MG followed by ALS, 8 patients with ALS followed by MG, 5 ALS patients with false-positive anti-acetylcholine receptor, and the other 2 ALS patients with only myasthenia symptoms. Most patients had limb onset ALS, and myasthenia symptoms mainly affected ocular and bulbar muscles. Clinical and neurophysiological characteristics were summarized.

CONCLUSION

These findings support the conclusion that immunological mechanisms and alterations in the neuromuscular junction are related to ALS pathogenesis.

摘要

目的

描述肌萎缩侧索硬化症(ALS)与重症肌无力(MG)重叠综合征患者的特征,并探讨这两种疾病之间的关系。

方法

我们检索了北京协和医院1983年至2015年病历中ALS与MG共存的情况,并检索了PubMed数据库中截至2016年12月发表的所有描述ALS与MG重叠综合征的文献。我们根据严格的诊断标准对患者的临床和神经生理学特征进行分组分析。

结果

我们数据库中有2例ALS与MG合并患者,结合文献报道的25例,将患者分为4组:12例MG后发ALS患者,8例ALS后发MG患者,5例ALS患者抗乙酰胆碱受体呈假阳性,另外2例仅表现为肌无力症状的ALS患者。大多数患者以肢体起病的ALS为主,肌无力症状主要影响眼肌和延髓肌。总结了临床和神经生理学特征。

结论

这些发现支持免疫机制和神经肌肉接头改变与ALS发病机制相关的结论。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/57df/5439131/3dc78d171613/fneur-08-00218-g001.jpg

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