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[脱髓鞘疾病]

[Demyelinating disorders].

作者信息

Savransky Andrea

机构信息

Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. E-mail:

出版信息

Medicina (B Aires). 2018;78 Suppl 2:75-81.

PMID:30199370
Abstract

Demyelinating diseases are a group of conditions of autoimmune etiology directed against the myelin of the central nervous system. In many cases, the onset of the illness is preceded by a nonspecific viral infection. Multiple sclerosis is a disease that evolves with relapses and remissions with polyfocal neurological deficits, being the most frequent optic neuritis, transverse myelitis and encephalic trunk involvement. Typically, magnetic resonance image (MRI) shows peri-ventricular, peri-callosal, cerebellum, brain stem and spinal cord hyperintensive lesions in T2 and FLAIR weighted images. Optic neuromyelitis is characterized by the presence of optic neuritis and transverse myelitis associated with the postrema and diencephalic area syndrome. MRI lesions are distributed in sectors rich with aquaporine-4 channels (AQP-4): hypothalamus, third and fourth ventricle, optic nerves and spinal cord. Finding anti AQP4 antibodies is useful for the diagnosis although they are not essential for it. Acute disseminated encephalomyelitis is typically a monophasic condition characterized by acute encephalopathy associated with hyperintense MRI large, bilateral and irregular asymmetric lesion in T2 and FLAIR weighted images. In all three cases, cerebral spine fluid (CSF) can show pleocytosis and hyperproteinorrachia. The presence of oligoclonal bands in CSF is characteristic of multiple sclerosis. In all cases, acute treatment includes high dose intravenous corticosteroids and plasmapheresis in non-responsive cases. Both multiple sclerosis and optic neuromyelitis require long-term treatment to prevent relapse and recurrent diseases.

摘要

脱髓鞘疾病是一组针对中枢神经系统髓鞘的自身免疫性病因的病症。在许多情况下,疾病发作之前会有非特异性病毒感染。多发性硬化症是一种呈复发和缓解状态发展的疾病,伴有多灶性神经功能缺损,最常见的是视神经炎、横贯性脊髓炎和脑干受累。典型地,磁共振成像(MRI)在T2加权像和液体衰减反转恢复序列(FLAIR)加权像上显示脑室周围、胼胝体周围、小脑、脑干和脊髓的高信号病变。视神经脊髓炎的特征是存在视神经炎和横贯性脊髓炎,并伴有最后区和间脑区域综合征。MRI病变分布在富含水通道蛋白4(AQP - 4)通道的区域:下丘脑、第三和第四脑室、视神经和脊髓。发现抗AQP4抗体对诊断有用,尽管它们并非诊断所必需。急性播散性脑脊髓炎通常是单相病症,其特征为急性脑病,在T2加权像和FLAIR加权像上伴有MRI大的、双侧且不规则不对称的高信号病变。在所有这三种情况下,脑脊液(CSF)可显示细胞增多和蛋白含量增高。脑脊液中寡克隆带的存在是多发性硬化症的特征。在所有情况下,急性治疗包括大剂量静脉注射皮质类固醇,对无反应的病例采用血浆置换。多发性硬化症和视神经脊髓炎都需要长期治疗以预防复发和反复发病。

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