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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。
Objective To investigate the relationship between subacute necrotizing basal encephalopathy (SNBGE) and Leigh’s disease. Methods: The etiology, clinical features and CT / MRI of 36 cases of SNBGE were analyzed. Results: All 36 cases were found to be clinically subacute because of various etiologies. There were various symptoms and signs such as extrapyramidal and pyramidal tract lesions, and bilateral basal ganglia symmetry Necrosis, softening changes. Conclusion: Brain CT or MRI SNBGE can be used for prenatal diagnosis, according to different causes, can be divided into primary (ie, Leigh disease) and secondary, the latter can be based on a clear cause, crown toxic, hypoxic Or infectious SNBGE.