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[终末期肌萎缩侧索硬化症患者的肌无力综合征]

[Myasthenic syndrome in a patient with end-stage amyotrophic lateral sclerosis].

作者信息

Kovrazhkina E A, Serdyuk A V, Razinskaya O D, Shurdumova M H, Vyatkina N V, Baranova E A

机构信息

Federal Center for Brain and Neurotechnology, Moscow, Russia.

Pirogov Russian National Research Medical University, Moscow, Russia.

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2023;123(7):102-107. doi: 10.17116/jnevro2023123071102.

Abstract

Amyotrophic lateral sclerosis (ALS) and myasthenia gravis are diseases with similar clinical features but different prognosis and approach to treatment. It is possible as an extremely rare combination of these diseases, as well as myasthenia gravis with signs of ALS (MuSK-positive), as well as ALS, accompanied by myasthenic syndrome. Latter option is the most common. Myasthenic syndrome accompanying the ALS characterized by pathological muscle fatigue signs, symptoms variability during the day, partial sensitivity to neostigmine, M-wave decrements detection during electromyographyc study. We present a case of a patient with terminal ALS and myasthenic syndrome. The main pathogenesis theories of this condition and the differential diagnosis of ALS and myasthenia gravis are discussed.

摘要

肌萎缩侧索硬化症(ALS)和重症肌无力是临床特征相似但预后和治疗方法不同的疾病。这些疾病极罕见地合并出现,以及伴有ALS体征的重症肌无力(MuSK阳性),还有伴有肌无力综合征的ALS,后一种情况最为常见。伴有ALS的肌无力综合征的特征为病理性肌肉疲劳体征、日间症状变异性、对新斯的明部分敏感、在肌电图检查中检测到M波递减。我们报告一例终末期ALS合并肌无力综合征患者的病例。讨论了这种情况的主要发病机制理论以及ALS与重症肌无力的鉴别诊断。

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