Waragai M, Takaya Y, Hayashi M, Shibata N, Kobayashi M
Department of Neurology, Kofu City Hospital, Yamanashi, Japan.
Neuroradiology. 1996 Nov;38(8):788-91. doi: 10.1007/s002340050349.
A case of adult-onset adrenoleukodystrophy involving predominantly the cerebellum and brain stem is described. The patient was a 30-year-old man with no family history of adrenoleukodystrophy. His neurological symptoms were progressive cerebellar ataxia and spastic paraparesis. CT showed atrophy of the cerebellum and brain stem. MRI disclosed lesions, enhancing partially with gadolinium, in the dentate nuclei and the pyramidal tracts from the internal capsule to the brain stem. The biochemical findings were compatible with typical adrenoleukodystrophy, but the distribution of the lesions on MRI was distinctly different. After steroid administration, the patient's symptoms improved slightly and contrast enhancement of the lesions was markedly reduced.
本文描述了一例主要累及小脑和脑干的成人起病型肾上腺脑白质营养不良病例。患者为一名30岁男性，无肾上腺脑白质营养不良家族史。其神经系统症状为进行性小脑共济失调和痉挛性截瘫。CT显示小脑和脑干萎缩。MRI显示齿状核以及从内囊到脑干的锥体束有病变，部分病变在钆增强扫描时有强化。生化检查结果与典型的肾上腺脑白质营养不良相符，但MRI上病变的分布明显不同。给予类固醇治疗后，患者症状稍有改善，病变的对比增强明显减轻。
