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多相性急性播散性脑脊髓炎及其与早发性多发性硬化症的鉴别

Multiphasic acute disseminated encephalomyelitis and differential with early onset multiple sclerosis.

作者信息

Stokes Brackett Arla Cinderella, Hernández-Fustes Otto Jesus, Arteaga Rodríguez Carlos, Hernandez Fustes Olga Judith

机构信息

Neurology Service, Hospital San Juan de Dios, Guatemala.

Neurology Department, InNeuro, Curitiba, Brasil.

出版信息

Intractable Rare Dis Res. 2020 Feb;9(1):61-63. doi: 10.5582/irdr.2020.01009.

Abstract

Multiple sclerosis is considered the most frequent demyelinating disorder of the Central Nervous System (CNS) among young adults, yet is very rare before 10 years old. Acute disseminated encephalomyelitis is a monophasic, polysymptomatic disorder that involves the CNS white matter with demyelinating lesions, which usually occurs after systemic viral infections. These two demyelinating diseases can present initially as an acute focal neurological syndrome and they can be difficult to distinguish. We describe a case of a nine-year-old girl that presented initially with dysphonia, gait ataxia, eyelid myokymia and brainstem disturbances. This was her second episode; the first episode was at the age of four years old. She recovered without neurological sequelae. The brain magnetic resonance imaging (MRI) demonstrated multiple demyelinating lesions in the white matter, cortical regions of the frontal lobe, periventricular distribution, internal capsule, corpus callosum and cerebellum. The purpose of the presentation of this case was to highlight the similarities between these two entities, since the clinical picture and neuroimaging are difficult to distinguish, mainly in relation to the first episode.

摘要

多发性硬化症被认为是青壮年中最常见的中枢神经系统(CNS)脱髓鞘疾病,但在10岁之前极为罕见。急性播散性脑脊髓炎是一种单相、多症状的疾病,累及中枢神经系统白质并伴有脱髓鞘病变,通常发生在全身性病毒感染之后。这两种脱髓鞘疾病最初都可能表现为急性局灶性神经综合征,且难以区分。我们描述了一例9岁女孩的病例,她最初表现为发音困难、步态共济失调、眼睑肌纤维颤搐和脑干功能障碍。这是她的第二次发作;第一次发作是在4岁时。她康复后没有神经后遗症。脑部磁共振成像(MRI)显示白质、额叶皮质区域、脑室周围分布、内囊、胼胝体和小脑中存在多个脱髓鞘病变。展示该病例的目的是强调这两种疾病之间的相似性,因为临床表现和神经影像学表现很难区分,尤其是在首次发作时。

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本文引用的文献

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Treatment Approaches for MOG-Ab-Associated Demyelination in Children.儿童MOG抗体相关脱髓鞘的治疗方法
Curr Treat Options Neurol. 2019 Jan 22;21(1):2. doi: 10.1007/s11940-019-0541-x.
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Pediatric multiple sclerosis.小儿多发性硬化症
Curr Opin Neurol. 2017 Jun;30(3):216-221. doi: 10.1097/WCO.0000000000000452.
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Acute disseminated encephalomyelitis.急性播散性脑脊髓炎
Neurology. 2007 Apr 17;68(16 Suppl 2):S23-36. doi: 10.1212/01.wnl.0000259404.51352.7f.

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