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儿童炎性脱髓鞘疾病:病例报告及文献综述

Inflammatory Demyelinating Diseases of Childhood: Case Report and Literature Review.

作者信息

Ekizoğlu Esme, Tektürk Topaloğlu Pınar, Yapici Zuhal, Eraksoy Mefkûre

机构信息

Department of Neurology, İstanbul University, Istanbul Faculty of Medicine, İstanbul, Turkey.

出版信息

Noro Psikiyatr Ars. 2014 Mar;51(1):74-78. doi: 10.4274/npa.y6304. Epub 2014 Mar 1.

Abstract

Multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) are demyelinating inflammatory diseases, considered to have a striking pathophysiological resemblance. However, due to the differences in both clinical course and clinical approaches, it is important to differentiate between the two conditions, to plan further investigations and therapy protocols. These diseases have similar but also distinct clinical, radiological and cerebrospinal fluid (CSF) findings. ADEM is typically a monophasic disease of children. MS occurs generally in adult age, but uncommonly may develop in childhood with variable features. Our case is a 14 year-old-girl, presented with a three-month history of left hemiparesis, followed by right hemiparesis, cerebellar signs, myelitis and cortical visual disturbances. Based on the clinical follow-up, MR and CSF findings, our patient was diagnosed with relapsing tumefactive multiple sclerosis. Steroid treatment was not significantly effective, however the patient has benefited from plasmapheresis clinically and radiologically. Our patient is still being followed under the disease modifying therapy without any relapse.

摘要

多发性硬化症(MS)和急性播散性脑脊髓炎(ADEM)是脱髓鞘性炎性疾病,被认为在病理生理方面有显著相似性。然而,由于临床病程和临床处理方法存在差异,区分这两种疾病对于规划进一步的检查和治疗方案很重要。这些疾病有相似但也有不同的临床、放射学和脑脊液(CSF)表现。ADEM通常是儿童的单相疾病。MS一般发生于成年期,但罕见情况下也可能在儿童期发病,表现各异。我们的病例是一名14岁女孩,有三个月的左侧偏瘫病史,随后出现右侧偏瘫、小脑体征、脊髓炎和皮质视觉障碍。根据临床随访、磁共振成像(MR)和脑脊液检查结果,我们的患者被诊断为复发性瘤样多发性硬化症。类固醇治疗效果不显著,然而患者在临床和放射学方面从血浆置换中获益。我们的患者仍在疾病修正治疗下接受随访,未出现任何复发情况。

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本文引用的文献

2
A comparison of MRI criteria for diagnosing pediatric ADEM and MS.儿童 ADEM 和 MS 的 MRI 诊断标准比较。
Neurology. 2010 May 4;74(18):1412-5. doi: 10.1212/WNL.0b013e3181dc138b. Epub 2010 Mar 24.
4
Multiple sclerosis in childhood: clinical and radiological features.
J Child Neurol. 2009 Jan;24(1):56-62. doi: 10.1177/0883073808321042. Epub 2008 Oct 21.
8
Pediatric multiple sclerosis.小儿多发性硬化症
NeuroRx. 2006 Apr;3(2):264-75. doi: 10.1016/j.nurx.2006.01.011.
9
Relapsing-remitting tumefactive multiple sclerosis.
Mult Scler. 2005 Dec;11(6):731-4. doi: 10.1191/1352458505ms1214cr.
10
Acute disseminated encephalomyelitis: an update.急性播散性脑脊髓炎:最新进展
Arch Neurol. 2005 Nov;62(11):1673-80. doi: 10.1001/archneur.62.11.1673.

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