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[抗肌肉特异性受体酪氨酸激酶(MuSK)抗体滴度与抗MuSK抗体阳性重症肌无力患者对皮质类固醇治疗反应的症状改善相关:一例报告]

[Titer of anti-muscle-specific receptor tyrosine kinase (MuSK) antibody correlated with symptomatic improvement in response to corticosteroid therapy in a patient with anti-MuSK antibody-positive myasthenia gravis: a case report].

作者信息

Nakahama Yutaka, Kawajiri Masakazu, Ochi Masayuki, Kohara Katsuhiko, Ohta Kiyoe, Miki Tetsurou

机构信息

Department of Geriatric Medicine, Medicine and Bioscience, Graduate School of Medicine, Ehime University.

出版信息

Rinsho Shinkeigaku. 2007 Jun;47(6):356-8.

Abstract

A 54-year-old woman was admitted to our hospital because of diplopia, dysphagia, dropped head, and muscle weakness with easy fatigability. A neurological examination showed bilateral ptosis, ocular motility disorder, dysphagia, and weakness of the neck extensor muscles. Edrophonium and repetitive nerve stimulation tests of the thenar muscles showed positive results. The serum titer of anti-acetylcholine receptor antibody was negative. A thymoma was not detected in her chest CT. Finally, she was diagnosed with anti-MuSK antibody-positive myasthenia gravis based on the high serum titer of anti-MuSK antibody (239 nmol/l). Her symptoms improved after administration of prednisolone. However, the symptoms were aggravated when the prednisolone dosage was reduced, and the titer of anti-MuSK antibody rose at the same time. We evaluated the possible association between changes in the severity of her clinical symptoms and the titer of the antibody during prednisolone therapy. It was revealed that the titer of the antibody was correlated to the severity of clinical symptoms expressed by a QMG (Quantitative Myasthenia Gravis) score. These findings indicate that monitoring the titer of anti-MuSK antibody can be useful for assessing disease activity as well as decision making during treatment.

摘要

一名54岁女性因复视、吞咽困难、垂头及肌肉无力伴易疲劳而入住我院。神经系统检查显示双侧上睑下垂、眼球运动障碍、吞咽困难及颈伸肌无力。新斯的明试验及大鱼际肌重复神经电刺激试验结果均为阳性。抗乙酰胆碱受体抗体血清滴度为阴性。胸部CT未发现胸腺瘤。最终,基于抗MuSK抗体高血清滴度(239 nmol/l),她被诊断为抗MuSK抗体阳性重症肌无力。给予泼尼松龙后症状改善。然而,泼尼松龙剂量减少时症状加重,同时抗MuSK抗体滴度升高。我们评估了泼尼松龙治疗期间她临床症状严重程度变化与抗体滴度之间的可能关联。结果显示,抗体滴度与由重症肌无力定量评分(QMG)表示的临床症状严重程度相关。这些发现表明,监测抗MuSK抗体滴度对于评估疾病活动度以及治疗期间的决策可能有用。

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