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非典型炎症性脱髓鞘病变和非典型多发性硬化症。

Atypical inflammatory demyelinating lesions and atypical multiple sclerosis.

机构信息

Department of Neurology, Montpellier University Hospital, 80 rue Augustin Fliche, 34295 Montpellier, Cedex 05, France.

Department of Neurology, Montpellier University Hospital, 80 rue Augustin Fliche, 34295 Montpellier, Cedex 05, France.

出版信息

Rev Neurol (Paris). 2018 Jun;174(6):408-418. doi: 10.1016/j.neurol.2018.03.007. Epub 2018 Apr 16.

Abstract

Atypical idiopathic inflammatory demyelinating disorders (IIDDs) of the brain have long been known to be disorders closely related to multiple sclerosis (MS), despite having distinctive clinical and radiological characteristics. Originally, they mostly corresponded to acute-onset variants of MS that classically had poor prognoses, such as Baló's concentric sclerosis, Marburg variant of MS and Schilder's disease, and their relationship with MS was based on their shared pathological findings and the co-occurrence of these variants in patients with typical MS. More recently, other atypical disorders, such as solitary sclerosis, have also been described as belonging to the MS spectrum, raising the question of their links with MS. Meanwhile, multiple MS mimics have been described and need to be considered in the differential diagnosis of MS. In addition, thorough characterization of these atypical entities, including advanced MRI and biological studies, is now warranted to further improve their management.

摘要

脑的非典型特发性炎症性脱髓鞘疾病(IIDD)长期以来被认为与多发性硬化症(MS)密切相关,尽管它们具有独特的临床和影像学特征。最初,它们主要对应于经典预后较差的 MS 急性发作变体,如 Baló 同心圆性硬化、Marburg 型 MS 和 Schilder 病,它们与 MS 的关系基于其共同的病理发现和这些变体在典型 MS 患者中的共同发生。最近,其他非典型疾病,如单发硬化症,也被描述为属于 MS 谱系,这引发了它们与 MS 的关系问题。同时,已经描述了多种 MS 模拟物,在 MS 的鉴别诊断中需要考虑这些模拟物。此外,现在需要对这些非典型实体进行全面表征,包括先进的 MRI 和生物学研究,以进一步改善其管理。

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