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双侧上肢缓慢进行性远端肌肉萎缩(奥沙利文-麦克劳德综合征),静脉注射免疫球蛋白治疗可部分缓解。

Slowly progressive distal muscular atrophy of the bilateral upper limbs (O'Sullivan-McLeod syndrome) partially alleviated by intravenous immunoglobulin therapy.

作者信息

Kawano Yuji, Nagara Yuko, Murai Hiroyuki, Kikuchi Hitoshi, Ohyagi Yasumasa, Kira Jun-ichi

机构信息

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka.

出版信息

Intern Med. 2007;46(8):515-8. doi: 10.2169/internalmedicine.46.6221. Epub 2007 Apr 17.

DOI:10.2169/internalmedicine.46.6221
PMID:17443046
Abstract

We report a case of O'Sullivan-McLeod syndrome in a 59-year-old man, who had experienced slowly progressive weakening of both hands since he was 20 years of age. Mild hyperIgEemia and eosinophilia were present. Nerve conduction studies revealed reduced F wave-evoked frequencies for the median and ulnar nerves. Intravenous immunoglobulin (IVIG) at a dose of 400 mg/kg/day was given for 5 days. After IVIG, the muscle weakness of the distal upper extremities improved together with increased F wave-evoked frequencies. These effects lasted for a few months. These observations suggest that immune-mediated neural damage partially contributes to O'Sullivan-McLeod syndrome.

摘要

我们报告了一例59岁男性的奥沙利文-麦克劳德综合征病例,该患者自20岁起双手出现缓慢进行性无力。存在轻度高免疫球蛋白E血症和嗜酸性粒细胞增多。神经传导研究显示正中神经和尺神经的F波诱发频率降低。给予剂量为400mg/kg/天的静脉注射免疫球蛋白(IVIG),持续5天。IVIG治疗后,远端上肢的肌肉无力得到改善,同时F波诱发频率增加。这些效果持续了几个月。这些观察结果表明,免疫介导的神经损伤部分导致了奥沙利文-麦克劳德综合征。

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Slowly progressive distal muscular atrophy of the bilateral upper limbs (O'Sullivan-McLeod syndrome) partially alleviated by intravenous immunoglobulin therapy.双侧上肢缓慢进行性远端肌肉萎缩(奥沙利文-麦克劳德综合征),静脉注射免疫球蛋白治疗可部分缓解。
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