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肌阵挛性肌肉病伴不规则趾阵挛和抗乙酰胆碱受体抗体:胸腺扩大切除术后缓解。

Rippling Muscle Disease with Irregular Toe Jerks and Anti-acetylcholine Receptor Antibodies: Remission after Extended Thymectomy.

机构信息

Department of Neurology, NTT Medical Center Tokyo, Japan.

Department of Neurology, Graduate School of Medicine, The University of Tokyo, Japan.

出版信息

Intern Med. 2022 May 1;61(9):1439-1442. doi: 10.2169/internalmedicine.8146-21. Epub 2021 Oct 19.

Abstract

We herein report a 63-year-old rippling muscle disease (RMD) patient who presented with painless stiffness, muscle hypertrophy and muscle contractions elicited by mechanical stimulation. He also showed irregular toe jerks and a slightly elevated level of anti-acetylcholine receptor antibody (AChR-Ab). Since he had a mediastinal mass mimicking thymoma, which was later revealed to be a bronchial cyst, he underwent extended thymectomy. The irregular toe jerks disappeared within a week after the operation. The other muscle symptoms completely remitted 27 months after the onset. This is the first report of a sporadic case of RMD with irregular toe jerks that resolved after extended thymectomy.

摘要

我们在此报告一例 63 岁的波纹状肌病(RMD)患者,其表现为无痛性僵硬、肌肉肥大和机械刺激诱发的肌肉收缩。他还表现出不规则的脚趾反射亢进和稍高的乙酰胆碱受体抗体(AChR-Ab)水平。由于他有纵隔肿块类似于胸腺瘤,后来证实为支气管囊肿,他接受了扩大胸腺切除术。手术后一周内不规则的脚趾反射亢进消失。其他肌肉症状在发病后 27 个月完全缓解。这是首例报道的散发型 RMD 病例,伴有不规则的脚趾反射亢进,经扩大胸腺切除术缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed1e/9152870/e3e5d61731f3/1349-7235-61-1439-g001.jpg

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