肿瘤样脱髓鞘病3例临床病理分析

目的探讨肿瘤样脱髓鞘病临床和病理学特点。方法对3例肿瘤样脱髓鞘病患者的临床特征进行了分析,并对活检脑组织进行了组织病理学和免疫组化研究。结果3例患者均呈亚急性起病,临床表现各不相同,有复发缓解过程。头部MRI显示3例患者分别在额、顶叶及小脑半球单发大面积病变。组织病理学观察均显示中心区组织高度水肿,呈空泡样变,有囊性坏死区。白质髓鞘高度崩解,轴索相对保留完好。免疫组化染色显示大量炎性细胞主要是淋巴细胞及少量单核吞噬细胞围绕血管呈袖套样浸润。结论肿瘤样脱髓鞘病临床表现各异,当影像学表现为孤立、单发、巨大病灶时,应想到脱髓鞘疾病的可能,病理学检查对确定诊断有重要价值。

Clinicopathological study of tumor-like demyelinating diseases

Objective To explore the clinical and neuropathologic characteristics of tumor-like demyelination diseases.Methods We analyzed the clinical features of three patients with tumor-like demyelination diseases,and observed the pathological findings in brain biopsy by histological and immunohistochemical methods.Results Three patients were all presented with subacute onset,various clinical manifestation and relapse-remitting process.Brain MRI showed a isolated,enormous lesion in frontal,parietal lobe and cerebella respectively in three patients.Pathologically,highly edema,vacuolar change and cystal necrosis were recognized in core area.A highly demyelinating changes with well preserved axons were disclosed in the white matter.Immunohistochemical staining demonstrated that a large number of inflammatory cells,predominantly the lymphocytes and monophagocytes infiltrated perivascularly.Conclusion Clinical manifestation of tumor-like demyelination diseases is different.The diagnosis is highly suspected when brain MRI discloses a isolated,single,enormous lesion,and the pathological examination might help to make the final diagnosis.