亚急性坏死性基底节脑病(附50例临床分析)

50 cases report of subacute necrotizing basal ganglia encephalopathy

目的探讨亚急性坏死性基底节脑病(SNBGE)的病因学、临床特征及其与Leigh's病的关系。方法对50例SNBGE的病因、临床特征、实验室检查及CT/MRI进行分析。结果50例SNBGE均因各种不同病因引起,呈亚临床急性发病,表现锥体外系及锥体系损害等多种多样的症状、体征,头部CT、MRI显示非梗死性双侧基底节对称性坏死、软化,治疗部分有效。结论头部CT、MRI可对SNBGE生前作出诊断,分为原发性(即Leigh's病)和继发性(病因有中毒、缺氧及感染等),临床需与肝豆状核变性、Hallervorden-spatz病(HSD)等鉴别。

Objective To explore the etiology,clinical characters of subacute necrotizing basal ganglia encephalopathy（SNBGE） and the relationship between SNBGE and the Leigh＇s disease. Methods Analyzing the causes,clinical characters, laboratory examination and cranial CT/MRI of SNBGE in the 50 patients. Results The progress of all 50 patients of SNBGE was subacute and caused by various etiological factors,the symptoms and signs of all patients showed impairment of extropyramidal tract ,pyramidal tract ,etc. The cranial CT/MRI of all patients showed bilateral symmetry necrosis and softening change. Part of these patients was cured effectually. Conclusion The examination of cranial CT/MRI could diagnose SNBGE in life. SNBGE could be classified as primary SNBGE （Leigh＇s disease） and secondary SNBGE （the causes were toxicosis,anoxemia and infection）. SNBGE should be identified with such diseases as hepatolenticulardegeneration,Hallervorden-spatz disease etc.