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脱髓鞘疾病

Demyelinating diseases.

作者信息

Love S

机构信息

Department of Neuropathology, University of Bristol Institute of Clinical Neurosciences, Frenchay Hospital, Bristol BS16 1LE, UK.

出版信息

J Clin Pathol. 2006 Nov;59(11):1151-9. doi: 10.1136/jcp.2005.031195.

Abstract

A diagnosis of demyelination carries important therapeutic and prognostic implications. In most cases the diagnosis is made clinically, and involvement of the histopathologist is largely confined to postmortem confirmation and clinicopathological correlation. However, every now and then, accurate diagnosis of the presence or cause of demyelination before death hinges on the histopathological assessment. Recognition of demyelination depends on an awareness of this as a diagnostic possibility, and on the use of appropriate tinctorial and immunohistochemical stains to identify myelin, axons and inflammatory cells. In biopsy specimens, the critical distinction is usually from ischaemic or neoplastic disease, and the types of demyelinating disease most likely to be encountered are multiple sclerosis, acute-disseminated encephalomyelitis, progressive multifocal leucoencephalopathy and extrapontine myelinolysis. Interpretation of the pathology has to be made in the context of the clinical, radiological and biochemical findings. Freezing of a small amount of fresh tissue allows for later virological studies, and electron microscopy is occasionally helpful for demonstration of viral particles.

摘要

脱髓鞘诊断具有重要的治疗和预后意义。在大多数情况下,诊断是临床作出的,组织病理学家的参与主要限于死后确认和临床病理相关性分析。然而,偶尔在死亡前准确诊断脱髓鞘的存在或病因取决于组织病理学评估。脱髓鞘的识别取决于对这种诊断可能性的认识,以及使用适当的染色剂和免疫组化染色剂来识别髓鞘、轴突和炎症细胞。在活检标本中,关键的鉴别通常是与缺血性或肿瘤性疾病,最有可能遇到的脱髓鞘疾病类型是多发性硬化症、急性播散性脑脊髓炎、进行性多灶性白质脑病和脑桥外髓鞘溶解症。病理学解释必须结合临床、放射学和生化检查结果。冷冻少量新鲜组织以便日后进行病毒学研究,电子显微镜偶尔有助于显示病毒颗粒。

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