Kawai M, Negoro K, Matsumoto K, Morimatsu M
Department of Neurology, Yamaguchi University School of Medicine, Ube, Japan.
No To Shinkei. 1993 Aug;45(8):769-71.
We reported a 28-year-old man with adrenoleukodystrophy showing neurological features of olivopontocerebellar atrophy. He had a 11-year history of Addison's disease. ACTH stimulation produced no rise in the plasma cortisol level. The ratios of C24:0/C22:0, C25:0/C22:0, and C26:0/C22:0 in fatty acids of sphingomyelin from plasma were all increased. MRI showed the atrophy of brainstem and cerebellum and the abnormal hyperintense lesions of the bilateral pyramidal tracts in the brainstem and internal capsule. 99mTc-HM PAO SPECT showed hypoperfusion of the deep white matter, frontal lobes, temporal lobes, and cerebellum. We suggest that SPECT may be useful for detection of subclinical lesions in ALD.
我们报告了一名28岁患有肾上腺脑白质营养不良的男性，其表现出橄榄脑桥小脑萎缩的神经学特征。他有11年的艾迪生病史。促肾上腺皮质激素刺激后血浆皮质醇水平未升高。血浆中鞘磷脂脂肪酸的C24:0/C22:0、C25:0/C22:0和C26:0/C22:0比值均升高。MRI显示脑干和小脑萎缩，以及脑干和内囊双侧锥体束的异常高信号病变。99mTc-HM PAO SPECT显示深部白质、额叶、颞叶和小脑灌注不足。我们认为SPECT可能有助于检测肾上腺脑白质营养不良的亚临床病变。
