亚急性坏死性基底节脑病36例报告

36 Cases Report of Subacute Necro Tizing Basal Ganglia Encephalopathy

目的;探讨亚急性坏死性基底节脑病(ＳＮＢＧＥ)与Ｌｅｉｇｈ病的关系。方法:对３６例ＳＮＢＧＥ的病因、临床特点、ＣＴ/ＭＲＩ进行分析。结果:发现３６例均因各种不同病因引起临床亚急性经过,出现锥体外系、锥体束损害等多种多样的症状、体征,而在颅脑ＣＴ或ＭＲＩ均呈双侧基底节对称性坏死、软化性改变。结论:颅脑ＣＴ或ＭＲＩ对ＳＮＢＧＥ可作生前诊断,根据病因不同,可分为原发性(即Ｌｅｉｇｈ病)和继发性,后者又可根据明确病因,冠以中毒性、缺氧性或感染性ＳＮＢＧＥ。

Aim:To explore the relationship between subacute necrotizing basal ganglia encephalopathy (SNBGE) and the Leigh's disease. Methods: Analyzing the causes, the clincial charcteristics, cranial CT and MRI of SNBGE in the 36 patients. Results:The progress of all 36 patients of SNBGE was subacute and caused by various etiological factors,the symptoms and signs of all patients showed impairment of extrapyramidal tract, pyramidal tract, etc. The cranial CT and MRI of all patients showed bilateral symmetry necrosis and softening change. Conclusion: The examination of cranial CT and MRI could diagnose SNBGE in life. SNBGE could be classified as primary SNBGE (Leigh' s disease), toxic SNBGE, hypoxic SNBGE and infective SNBGE.