本文报告一家四兄弟患肾上腺脊髓神经病。他们分别于5～10岁左右出现肾上腺皮质功能减退的突出症状:皮肤变黑,并于此后10年内逐渐发生进行性痉挛性轻度截瘫,后期有相对急性加重的特点。先证者的长兄27岁时死于感染,次兄结婚后有明显的性功能减退。家系调查结果提示为伴性隐性遗传,其3个姨表兄弟有死于“Schilder’s型”肾上腺脑白质营养不良症之疑。 This article reports a four-brother suffering from adrenal spinal cord neuropathy. They were prominent symptoms of adrenal insufficiency at about 5 to 10 years of age: the skin darkens, and gradually progressive spastic paraplegia within 10 years, the latter has the characteristics of a relatively acute exacerbation. The elder brother of the proband dies at the age of 27 and has significant sexual dysfunction after his next marriage. The pedigree investigation suggested a sex-linked recessive inheritance. The three aunt cousins ​​died of “Schilder’s type” adrenoleukodystrophy.