儿童型肾上腺脑白质营养不良的神经影像学诊断

Neuroradiology of Adrenoleukodystrophy in Children

作者对１０例儿童型肾上腺脑白质营养不良患儿头颅ＣＴ和ＭＲＩ进行了观察，总结出这种病变影像学典型表现特征：（１）蝶形病灶。在双侧三角区周围呈现对称的、大片状、局限于脑白质的蝶形病变。（２）花边样强化条带将蝶形病灶分隔成较大片的中央区和外周区，两个区域的信号强度与密度不同。（３）整个胼胝体压部信号异常。（４）ＭＲＩ优于ＣＴ，能显示视觉、听觉传导通路和运动传导通路上的病灶。作者分析了影像学表现与临床症状之间，以及与病理学之间的关系，并就近年来的治疗进展进行了讨论。

The authors reported the MRI and CT findings of 10 children with a- drenoleukodystrophy and summarized the imaging characteritics of this kind of disease. They were butterfuly-like lesions;the big central area and peripheral zones seperated by the thin stripe of lace- like enhancement and abnormal signals in the splenium of corpus callosum,MRI was superior to CT in detection of special lesions such as lesions on the pathway of pyramidal tract,auditory tract and vi- sual pathway. The relationship between the neuroradiological findings and neuropathology was stud-ied.Finally some therapeutic methods were discussed;