肾上腺脑白质营养不良(Adreno-enceleucodystrophy,简称AELD)是一种性连隐性遗传性疾病。其特征为脑白质进行性脱髓鞘病变,肾上腺皮质功能低下,细胞膜结合的微器官过氧化小体存在有先天缺陷,以致对于体内的多种脂肪酸、胆酸或二羧基酸等不能充分氧化而在体内累积。本病罕见,报告如下。 患者男,8岁半,学生。视、听障碍10月,发作性抽搐、智能障碍4月,于1990年6月22日入院。1989年8月下旬有视物不清,听力下降,10月右腿无力,走路时拖步;1990年2月24日出现癫痫大 Adreno-enceleucodystrophy (AELD) is a sex-linked recessive hereditary disease. It is characterized by progressive demyelination of the white matter, poor adrenocortical function, and inherent defects in the membrane-bound micro-organ peroxisomes, so that various kinds of fatty acids, bile acids or dicarboxylic acids in the body can not be sufficiently oxidized Accumulate in the body. The disease is rare, the report is as follows. Patient male, 8 and a half years old, student. Visual, hearing disorders in October, seizures, mental retardation April, admitted on June 22, 1990. In late August 1989, there was unclear visual acuity, decreased hearing ability, weakness in the right leg in October and dragging during walking. On February 24, 1990, epileptic large