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急性播散性脑脊髓炎(ADEM)的神经病理学和神经放射学方面

[Neuropathological and neuroradiological aspects of acute disseminated encephalomyelitis (ADEM)].

作者信息

Niedermayer I, Deinzer M, Moringlane J R, Feiden W

机构信息

Abteilung für Neuropathologie, Pavillon Kraepelin, Gebäude 90.3, Universitätskliniken des Saarlandes, 66421 Homburg/Saar.

出版信息

Radiologe. 2000 Nov;40(11):1030-5. doi: 10.1007/s001170050875.

Abstract

Among non-neoplastic lesions of the central nervous system, demyelinating pseudotumors of the group of acute disseminated encephalomyelitis (ADEM) most frequently occasion neurosurgical intervention for purposes of definitive diagnosis and thus enter the domain of the surgical pathologist. Typically, ADEM presents with multifocal, bilateral lesions in an asymmetrical distribution. Especially monolocular manifestations may be diagnostically challenging. Due to the acuteness of clinical symptoms and the expansive, space-occupying character of the lesions a diffuse glioma, a metastatic disease, a primary cerebral Non-Hodgkin's lymphoma, brain abscess, a parasitosis or an ischemic brain tissue necrosis may be suspected. This impression is supported by uptake of contrast-medium most pronounced at the periphery of the lesion and the subcortical location. The histomorphologic feature of relative axonal preservation in areas with acute myelin breakdown and lymphocytic infiltrates make the diagnosis of an acute primary demyelinating disease probable. A diagnosis of glioma may be prompted by the florid, cytologically atypical astrogliosis especially in intraoperative request. Based on a series of 14 cases of radiologically and bioptically documented cases of ADEM typical examples will be demonstrated and discussed.

摘要

在中枢神经系统的非肿瘤性病变中,急性播散性脑脊髓炎(ADEM)组的脱髓鞘假瘤最常需要进行神经外科干预以明确诊断,从而进入外科病理学家的领域。通常,ADEM表现为多灶性、双侧病变,分布不对称。尤其是单眼表现可能在诊断上具有挑战性。由于临床症状的急性发作以及病变的膨胀性、占位性特征,可能会怀疑是弥漫性胶质瘤、转移性疾病、原发性脑非霍奇金淋巴瘤、脑脓肿、寄生虫病或缺血性脑组织坏死。病变周边和皮质下位置对比剂摄取最明显支持了这种印象。急性髓鞘破坏和淋巴细胞浸润区域相对轴突保留的组织形态学特征使得急性原发性脱髓鞘疾病的诊断成为可能。特别是在术中要求下,明显的、细胞学上非典型的星形胶质细胞增生可能提示胶质瘤的诊断。基于14例经放射学和活检记录的ADEM病例系列,将展示并讨论典型病例。

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