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[1例重症肌无力胸腺切除术后抗水通道蛋白4抗体阳性的亚急性脊髓炎:胸腺切除术后自身免疫性疾病综述]

[A case of subacute myelitis with anti-aquaporin 4 antibody after thymectomy for myasthenia gravis: review of autoimmune diseases after thymectomy].

作者信息

Kohsaka Masayuki, Tanaka Masami, Tahara Masayuki, Araki Yasukiyo, Mori Satoru, Konishi Teturo

机构信息

MS Center, Utano National Hospital.

出版信息

Rinsho Shinkeigaku. 2010 Feb;50(2):111-3. doi: 10.5692/clinicalneurol.50.111.

Abstract

We report the case of a 60-year-old woman with myasthenia gravis (MG) and Basedow's disease who seven years after thymectomy developed subacute myelitis, a limited form of neuromyelitis optica (NMO). The patient presented with a centrally located long spinal cord lesion (LCL) on cervical cord MRI, anti-aquaporin 4 (AQP4) antibody in serum, and HLA DPB1*0501. Brain MRI showed no specific findings of classic multiple sclerosis (MS). CSF study showed elevated protein (67 mg/dl) but a normal IgG index (0.63) and no oligoclonal IgG bands. After repeated methylprednisolone pulse and immunoadsorption therapies, the T2-high signal lesion shrunk and tetraparesis improved. We reviewed the English and Japanese literature and found reports of 30 patients showing MS including NMO complicated with MG; 27 had been diagnosed as MS after thymectomy. Among these 27, 16 of 17 who were examined by spinal cord MRI and for anti-AQP4 antibodies were NMO. Only one patient with signs and symptoms localized to the optic nerves and spinal cord showed no LCL and was not examined for anti-AQP4 antibodies. In autoimmune disorders of the central nervous system after thymectomy in patients with MG, NMO is more predominant than classic MS.

摘要

我们报告了一例60岁患有重症肌无力(MG)和突眼性甲状腺肿的女性病例,该患者在胸腺切除术后7年发生了亚急性脊髓炎,这是视神经脊髓炎(NMO)的一种局限性形式。患者颈椎MRI显示脊髓中央有一个长节段脊髓病变(LCL),血清中存在抗水通道蛋白4(AQP4)抗体,且携带HLA DPB1*0501。脑部MRI未显示典型多发性硬化(MS)的特异性表现。脑脊液检查显示蛋白升高(67mg/dl),但IgG指数正常(0.63)且无寡克隆IgG带。经过反复甲泼尼龙冲击和免疫吸附治疗后,T2高信号病变缩小,四肢瘫得到改善。我们查阅了英文和日文文献,发现有30例显示为MS(包括合并MG的NMO)的患者报告;其中27例在胸腺切除术后被诊断为MS。在这27例中,17例接受脊髓MRI检查及抗AQP4抗体检测的患者中有16例为NMO。仅1例症状局限于视神经和脊髓的患者未显示LCL且未检测抗AQP4抗体。在MG患者胸腺切除术后的中枢神经系统自身免疫性疾病中,NMO比典型MS更常见。

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