亚急性坏死性脑脊髓病(SNE,Leigh病)是一种病因和治疗方法不明的退行性神经系统疾病,本文报告1例在临床放射学和生化方面很难与其鉴别的重症脚气病。该例系出生体重2.9公斤的健康足月婴儿,母乳喂养,3月龄始食用非奶制品厂生产的合成Vitarich食品,6月龄始食欲减退、呕吐、并因内斜视和颈强直住院。体检示不发热、轻度脱水,易怒、体重 Subacute necrotizing encephalomyelopathy (SNE, Leigh’s disease) is an etiologic and untreated degenerative neurological disease. This article reports one case of severe beriberi that is difficult to identify in clinical radiology and biochemistry. The case is a healthy full-term infant weighing 2.9 kg. Breastfeeding, 3-month-old synthetic Vitarich food produced at a non-dairy plant begins anorexia and vomiting at 6 months of age and is hospitalized for esotropia and neck stiffness. Physical examination showed no fever, mild dehydration, irritability, weight