肾上腺脑白质营养不良：一种表现为橄榄体脑桥小脑萎缩的临床变异型。
Adrenoleukodystrophy: a clinical variant presenting as olivopontocerebellar atrophy.
作者信息
Ohno T, Tsuchida H, Fukuhara N, Yuasa T, Harayama H, Tsuji S, Miyatake T
出版信息
J Neurol. 1984;231(4):167-9. doi: 10.1007/BF00313932.
PMID:6096516
Abstract
A case of adrenoleukodystrophy showing neurological features of olivopontocerebellar atrophy is described. A CT scan demonstrated marked atrophy in both cerebellum and pons. ACTH stimulation produced no rise in the plasma cortisol level but a significant rise in the plasma aldosterone level. The ratios of C26:0 to C22:0 in fatty acids of sphingomyelin from erythrocyte membrane and plasma were increased.
摘要
描述了一例显示橄榄体脑桥小脑萎缩神经学特征的肾上腺脑白质营养不良病例。CT扫描显示小脑和脑桥均有明显萎缩。促肾上腺皮质激素刺激后血浆皮质醇水平未升高，但血浆醛固酮水平显著升高。红细胞膜和血浆中鞘磷脂脂肪酸的C26:0与C22:0比值升高。
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