An unusual complication of immunosuppression in myasthenia gravis: Progressive multifocal leukoencephalopathy

An unusual complication of immunosuppression in myasthenia gravis: Progressive multifocal leukoencephalopathy
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DOI:
10.1016/j.nmd.2008.09.019
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发表时间:
2009-02-01
影响因子:
2.8
通讯作者:
Sivasli, Izlem Evren
Sivasli, Izlem Evren
中科院分区:
医学4区
文献类型:
--
作者:
Gedizhoglu, Muhtesem;Coban, Pinar;Sivasli, Izlem Evren

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我们介绍了一名患有全身性重症肌无力的 44 岁女性患者,她出现了进行性多灶性白质脑病。在复发期间,她接受高剂量皮质类固醇、每日 200 毫克硫唑嘌呤和高剂量静脉注射免疫球蛋白。胸腺切除术后两个月,她出现进行性认知能力下降、不对称性四肢瘫痪和共济失调。两个月后,她卧床不起。头颅 MRI 显示皮质和皮质下结构存在大的不对称 T2 和 FLAIR 高信号病变。 JC 病毒脑脊液 PCR 呈阳性证实了诊断。患者幸存下来并留下严重的后遗症,这证实了非艾滋病病例中典型的缓慢进展。这是第二例肌无力患者出现进行性多灶性白质脑病。 (C) 2008 Elsevier B.V. 保留所有权利。
We present a 44-year-old female patient with generalised myasthenia gravis who developed progressive multifocal leukoencephalopathy. She was receiving high dose corticosteroids, azathioprine 200 mg daily and high dose intravenous immunoglobulin during relapses. Two months after thymectomy she presented with progressive cognitive decline, asymmetric quadriparesis and ataxia. Two months later she was bedridden. Cranial MRI showed large asymmetric T2 and FLAIR hyperintense lesions in cortical and subcortical structures. Positive CSF PCR of JC Virus confirmed the diagnosis. The patient survives with severe sequela which confirms slow progression as typical in nonAIDS cases. This is the second case of progressive multifocal leukoencephalopathy in a myasthenic patient. (C) 2008 Elsevier B.V. All rights reserved.