Abstract: Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma. It presents usually as rapidly progressive dementia and is accompanied by extensive white matter changes without formation of a cohesive mass in magnetic resonance imaging (MRI). The main pathological feature is diffuse infiltration in the white matter by individual neoplastic cells without formation of a cohesive tumor mass. The neurobehavioral deficits manifested by the patients demonstrate that lymphomatosis cerebri is an additional neoplastic cause of white matter dementia and can be added to the growing list of disorders responsible for this syndrome. Early pathological examination is important for specific treatment and interventions. Although more attention has been paid on lymphomatosis cerebri in clinical course, the knowledge about this disease is still on the base of case reports and lacks of systematic analysis both at home and abroad. We reviewed the case reports abroad and collected clinical and pathological data of 17 individuals who were diagnosed lymphomatosis cerebri through biopsy and (or) autopsy in the hope of deep and overall recognition of this disease.

Key words: Lymphoma, Central nervous system neoplasms, Leukoencephalopathy, progressive multifocal, Review

摘要： 大脑淋巴瘤病是中枢神经系统淋巴瘤的特殊类型。临床症状主要表现为进展迅速的认知功能障碍，影像学显示弥漫性脑白质病变，组织病理学呈现脑白质内恶性淋巴细胞弥漫性浸润。大脑淋巴瘤病已经成为脑白质病变鉴别诊断的重要病因之一，因此尽早进行病理检查对疾病预后具有重要意义。目前国内外关于大脑淋巴瘤病的认识仅基于一些病例报道，系统分析较少。在本文中，我们对国外相关病例进行回顾，共收集17 例大脑淋巴瘤病患者的临床及病理资料并对其进行总结，以期对该病能有更深入全面的了解。

关键词: 淋巴瘤； 中枢神经系统肿瘤； 脑白质病, 进行性多灶性； 综述