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一名儿童的复发性瘤样脱髓鞘病变

Recurrent tumefactive demyelination in a child.

作者信息

Puri Vinod, Chaudhry Neera, Gulati Parveen, Tatke Medha, Singh Daljit

机构信息

Department of Neurology, G.B.Pant Hospital, New Delhi, India.

出版信息

J Clin Neurosci. 2005 May;12(4):495-500. doi: 10.1016/j.jocn.2004.07.001.

Abstract

A 13-year-old female presented with two episodes of hemiplegia and hemianopia involving opposite sides, each time associated with seizures. On both occasions, the magnetic resonance (MR) scan showed a giant demyelinating, peripherally enhancing lesion with mass effect. MR spectroscopy (MRS) was indistinguishable from a tumor. At the first episode, she had undergone tumor decompression but the histopathology revealed an acute demyelinating lesion with no evidence of tumor. Each time there was partial clinical recovery and resolution of the radiological lesion, the patient having received corticosteroids during both of the episodes. She also developed hemiballismus postoperatively which resolved on withdrawing phenytoin. It is suggested that a trial of corticosteroids be given in suspected tumefactive demyelinating lesions, although they may be indistinguishable from a tumor.

摘要

一名13岁女性出现两次偏瘫和偏盲,累及身体对侧,每次均伴有癫痫发作。两次发作时,磁共振(MR)扫描均显示一个巨大的脱髓鞘病变,周边强化,有占位效应。磁共振波谱(MRS)表现与肿瘤难以区分。首次发作时,她接受了肿瘤减压手术,但组织病理学显示为急性脱髓鞘病变,无肿瘤证据。每次发作时患者在接受皮质类固醇治疗后,临床症状部分缓解,影像学病变消退。她术后还出现了偏身投掷症,停用苯妥英钠后症状缓解。建议对于疑似瘤样脱髓鞘病变患者进行皮质类固醇试验性治疗,尽管这些病变可能与肿瘤难以区分。

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