眼咽肌营养不良症、重症肌无力、系统性红斑狼疮:重叠与相互作用。

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL BMJ Case Reports Pub Date : 2024-11-12 DOI:10.1136/bcr-2024-262742
Joana M Barbosa, Pedro A Pereira
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引用次数: 0

摘要

包括眼咽肌营养不良症(OPMD)和重症肌无力症(MG)在内的多种疾病都可能导致眼睑下垂和近端无力。由于这两种疾病在临床和神经生理学上可能存在重要的重叠,因此很难将它们区分开来。系统性红斑狼疮(SLE)是一种常见的自身免疫性疾病,也被描述为与重症肌无力症有关。我们为您介绍一位60多岁的妇女,她患有波动性双侧上睑下垂和近端肌无力。单纤维肌电图显示抖动加剧。她的兄弟经遗传证实患有 OPMD。调查显示,她的 OPMD 基因检测呈阳性,乙酰胆碱受体抗体呈阳性。本病例强调了周到地了解个人和家族病史的重要性,并提高了人们对三种病症并存的罕见情况的认识,这三种病症具有一些共同的临床特征,但治疗和管理方法却各不相同。
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Oculopharyngeal muscular dystrophy, myasthenia gravis, systemic lupus erythematosus: overlap and interactions.

Ptosis and proximal weakness may develop in multiple diseases including oculopharyngeal muscular dystrophy (OPMD) and myasthenia gravis (MG). These two entities can be challenging to differentiate since they may have important clinical and neurophysiological overlap. Systemic lupus erythematosus (SLE), a common autoimmune disease, has been described in association with MG.We present a woman in her 60s with fluctuating bilateral ptosis and proximal muscle weakness. Single fibre electromyography showed increased jitter. Her brother has genetically proven OPMD. The investigation revealed a positive genetic test for OPMD and positive antibodies for acetylcholine receptor. Additionally, she has SLE, treated with hydroxychloroquine for more than 30 years.This case highlights the importance of a thoughtful anamnesis with personal and familial history and raises awareness for the rare coexistence of three pathologies with some common clinical characteristics but different treatments and management.

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来源期刊
BMJ Case Reports
BMJ Case Reports Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
1588
期刊介绍: BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.
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