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吞咽困难作为抗合成酶综合征相关肌炎的孤立表现?

Dysphagia as Isolated Manifestation of Jo-1 Associated Myositis?

作者信息

Labeit Bendix, Muhle Paul, Suntrup-Krueger Sonja, Ahring Sigrid, Ruck Tobias, Dziewas Rainer, Warnecke Tobias

机构信息

Department of Neurology, University Hospital Muenster, Muenster, Germany.

出版信息

Front Neurol. 2019 Jul 9;10:739. doi: 10.3389/fneur.2019.00739. eCollection 2019.

Abstract

Dysphagia can be predominant or sole symptom of myositis. However, diagnostic evaluation is difficult in such cases. Here, we present evidence for dysphagia as sole manifestation of Jo-1 associated myositis. A 77-year-old patient suffering from isolated dysphagia was assessed by flexible endoscopic evaluation of swallowing, videofluoroscopy, high resolution esophageal manometry, whole body muscle MRI, electroneurographic and electromyographic examination, cerebrospinal fluid analysis, screening for autoantibodies, and body plethysmography. We detected isolated oropharyngeal dysphagia including a decreased pressure of the upper esophageal sphincter leading to cachexia in an anti-Jo-1 positive patient without any abnormalities in the other diagnostics. Immunosuppressive therapy with cortisone and azathioprine led to long-term improvement of dysphagia. This is the first report of isolated dysphagia as manifestation of Jo-1 associated myositis. Therefore, Jo-1 associated myositis should be considered as a possible differential diagnosis for isolated dysphagia. Typical signs for myositis in instrumental dysphagia assessment are presented.

摘要

吞咽困难可能是肌炎的主要症状或唯一症状。然而,在这种情况下进行诊断评估很困难。在此,我们提供证据证明吞咽困难是抗Jo-1相关肌炎的唯一表现。一名77岁患有孤立性吞咽困难的患者接受了吞咽的软性内镜评估、电视荧光吞咽造影、高分辨率食管测压、全身肌肉MRI、神经电图和肌电图检查、脑脊液分析、自身抗体筛查以及人体体积描记法评估。我们在一名抗Jo-1阳性患者中检测到孤立性口咽吞咽困难,包括食管上括约肌压力降低导致恶病质,而其他诊断检查未发现任何异常。使用可的松和硫唑嘌呤进行免疫抑制治疗使吞咽困难得到长期改善。这是关于孤立性吞咽困难作为抗Jo-1相关肌炎表现的首例报告。因此,抗Jo-1相关肌炎应被视为孤立性吞咽困难的一种可能鉴别诊断。本文还介绍了器械性吞咽困难评估中肌炎的典型体征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1d0e/6629889/098a76020e1e/fneur-10-00739-g0001.jpg

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