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非多发性硬化复发性脱髓鞘疾病。

Non-MS recurrent demyelinating diseases.

作者信息

Brinar Vesna V

机构信息

Department of Neurology, Faculty of Medicine, Neurology Service, REBRO Hospital Centre, Kispaticeva 12, 10.000 Zagreb, Croatia.

出版信息

Clin Neurol Neurosurg. 2004 Jun;106(3):197-210. doi: 10.1016/j.clineuro.2004.02.016.

Abstract

The introduction of MRI has shown that the acute, recurrent (R), and multiphasic (M) forms of disseminated encephalomyelitis (DEM) are more common than suspected in adults, and that their MR images are sufficiently characteristic in most instances to make differentiation from multiple sclerosis (MS) possible. In addition, a number of clinical features of DEM are rarely seen in MS: fever, malaise, nausea, vomiting, positional vertigo, convulsions, aphasia, meningism, bilateral optic neuritis, and CSF leukocytosis and elevated protein. CSF oligoclonal bands are usually absent. It is remarkable that confusion between R- and MDEM and MS persists despite the numerous published reports on recurrent DEM dating back 70 years, many illustrating the characteristic MRIs. There are many case reports of DEM erroneously diagnosed as MS, Schilder's, Marburg's, Devic's, and Baló's disease, and, in particular brain tumors. It is probable that acute DEM is occasionally mistaken for a clinically isolated symptom of MS. Possible mechanisms for recurrence include localization at the site of a previous injury to the nervous system, or by the phenomenon of molecular mimicry. The importance of differentiating R- and MDEM from MS is greater today due to the recommendation that immunodulatory treatment be initiated in patients with a clinically isolated syndrome, or when the occurrence of a second clinical episode establishes the diagnosis of MS.

摘要

磁共振成像(MRI)的引入表明,播散性脑脊髓炎(DEM)的急性、复发型(R)和多相型(M)在成人中比人们怀疑的更为常见,并且在大多数情况下其MRI图像具有足够的特征,能够与多发性硬化症(MS)进行区分。此外,DEM的一些临床特征在MS中很少见:发热、不适、恶心、呕吐、体位性眩晕、惊厥、失语、脑膜刺激征、双侧视神经炎以及脑脊液白细胞增多和蛋白质升高。脑脊液寡克隆带通常不存在。值得注意的是,尽管有许多关于复发型DEM的报道可追溯到70年前,其中许多还展示了其特征性的MRI表现,但R-DEM和M-DEM与MS之间的混淆仍然存在。有许多关于DEM被错误诊断为MS、希尔德病、马尔堡病、德维克病、巴洛病,尤其是脑肿瘤的病例报告。急性DEM偶尔可能被误诊为MS的临床孤立症状。复发的可能机制包括定位于先前神经系统损伤的部位,或分子模拟现象。如今,将R-DEM和M-DEM与MS区分开来的重要性更大了,因为有建议指出,对于临床孤立综合征患者,或当第二次临床发作确诊为MS时,应开始免疫调节治疗。

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