肾上腺—脑白质营养不良(ALD)是罕见的全身代谢障碍性疾病,也是仅侵犯男性小儿的性联遗传性疾病。我们报告1例11岁男孩病例有两点不同于文献所见:1.脑干发生的病变比大脑两半球严重。2.本例有肝纤维化及淋巴腺组织细胞增生,此种改变不见于儿童型ALD,见于新生生儿型ALD。因而,我们怀疑上述两型之间可能存在界限型。 Adrenal - Leukodystrophy (ALD) is a rare systemic metabolic disorder that is also associated with sex-linked hereditary diseases that infiltrate only male children. We report a case of 11-year-old boy two different from the literature: 1. The lesions of the brain stem than the two hemispheres severe. 2. This case of liver fibrosis and lymphoid tissue cell proliferation, such changes are not seen in children with ALD, found in neonatal ALD. Therefore, we suspect there may be a boundary between the two types.