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[1例伴有抗肌肉特异性酪氨酸激酶抗体的胸腺瘤相关性重症肌无力]

[A case of thymoma-associated myasthenia gravis with anti-MuSK antibodies].

作者信息

Ito Ai, Sasaki Ryogen, Ii Yuichiro, Nakayama Shigeho, Motomura Masakatsu, Tomimoto Hidekazu

机构信息

Department of Neurology, Mie University Graduate School of Medicine.

出版信息

Rinsho Shinkeigaku. 2013;53(5):372-5. doi: 10.5692/clinicalneurol.53.372.

Abstract

We report a 60-year-old male with thymoma-associated myasthenia gravis with anti-MuSK antibodies. In October 2010, he had diplopia, ptosis, and dysphagia. He was diagnosed to have MG in February 2011. The neurological examination disclosed external ophthalmoplegia, bilateral ptosis, mild dysphagia, and fatigability. Repetitive nerve stimulation of the right facial nerve showed CMAP decrement greater than 10%. Patients showed an improvement in ptosis after administration of edrophonium.Anti-acetylcholine receptor antibody was negative, and anti-muscle specific receptor tyrosine kinase antibody was 66.8 nmol/l (cut-off value: 0.05 nmol/l). Prednisolone (50 mg every other day) were started. Contrast-enhanced chest MRI showed a mediastinal mass suggestive of thymoma. Extended thymectomy was performed in March 2011. Histological examination disclosed a type B1 thymoma. After resection of the tumor, the symptoms of MG improved with prednisolone (100 mg every other day). This is a rare case of MG with anti-MuSK antibodies and thymoma, which has been reported previously only in 2 cases.

摘要

我们报告了一名60岁患有抗MuSK抗体的胸腺瘤相关重症肌无力男性患者。2010年10月,他出现复视、上睑下垂和吞咽困难。2011年2月被诊断为重症肌无力。神经系统检查发现有眼外肌麻痹、双侧上睑下垂、轻度吞咽困难和疲劳感。右侧面神经重复神经电刺激显示复合肌肉动作电位(CMAP)递减大于10%。给予依酚氯铵后患者上睑下垂症状改善。抗乙酰胆碱受体抗体阴性,抗肌肉特异性受体酪氨酸激酶抗体为66.8 nmol/l(临界值:0.05 nmol/l)。开始使用泼尼松龙(隔日50 mg)。增强胸部MRI显示纵隔肿物,提示胸腺瘤。2011年3月进行了扩大胸腺切除术。组织学检查显示为B1型胸腺瘤。肿瘤切除后,重症肌无力症状在泼尼松龙(隔日100 mg)治疗下有所改善。这是一例罕见的伴有抗MuSK抗体和胸腺瘤的重症肌无力病例,此前仅有2例报道。

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