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亚急性坏死性脑脊髓病(SNE)为常染色体隐性遗传性疾病,首例由Leigh于1951年报告。大部分为婴儿型,近年文献报告生存活期较长的青少年型和成人型有屡见增加。以往SNE的诊断仅由尸解证实,但近年CT扫描的应用,此病有可能在生前诊断。本文就疑诊SNE的6例患者作CT扫描,结合临床资料作对比研究。女性5例、男性1例,发病时平均年龄6.2(3～12)岁,CT扫描时平均年龄13.8(7～27)岁。
Subacute necrotizing encephalomyelopathy (SNE) is an autosomal recessive disease, the first case reported by Leigh in 1951. Most of the infants, in recent years, the literature reported longer survival life of adolescents and adults have repeatedly increased. In the past the diagnosis of SNE was confirmed only by autopsy, but in recent years the application of CT scan, the disease may be diagnosed before birth. In this paper, 6 patients suspected of SNE for CT scan, combined with clinical data for comparative study. Five were female and one was male, with an average age of 6.2 (3-12) years at onset and an average age of 13.8 (7-27) years at CT scan.