肾上腺脑白质营养不良的影像分析

目的：分析肾上腺脑白质营养不良的影像特征及诊断价值。 材料和方法：回顾性分析16例临床证实的肾上腺脑白质营养不良患者的CT和MR影像；MRI检查10例，4例增强成像，CT扫描12例，3例增强。结果：16例患者均有典型的双侧侧脑室枕角旁白质内大片对称性低密度或中长T1长T2信号病变，经胼胝体压部相连，呈蝶翼状分布；此外，病灶还见于额、顶、颞叶、内囊、小脑、脑干，视路和听路病灶对称。5例见低密度灶内细点状、簇状钙化灶。6例增强均见花边样强化，平扫等T1长T2信号灶见强化；1例连续观察见病变向前、向下、向周围发展，并沿传导束分布。结论：肾上腺脑白质营养不良的CT、MRI表现多有特征性，包括典型蝶翼状分布、花边样强化、对称性侵犯双侧视路和听路、沿传导束分布，有助于明确诊断。

Image Analysis of Adrenoleukodystrophy

Purpose:To study the image features of adrenoleukodystrophy(ALD) and its diagnostic values.Materials and Method:Retrospectively analyzed CT and MR features of 16 cases of ALD proved clinically.MRI and CT examinations were undergone in 10 and 12 cases,respectively,and enhanced scans in 4 and 3,respectively.Results:All had classical symmetric T1 and T2 prolonged or hypodensity changes in posterior paraventricular white matter,which appears as butterfly with corpus callosum infiltration.Lesions were yet seen in frontal,parietal and temporal lobes,internal capsule,brain stem and cerebellum,and yet symmetrically in visual and auditory pathway.Dotted,clustered calcification inside lesion were found in 4 cases.All 6 had edge enhancement on postcontrast scans.On follow-up image of 1 case,we found the progress of lesions were anterior,inferior,peripheral and along the tracts.Conclusion:ALD had the characteristic CT and MR findings,including butterfly lesions,edge enhancement,symmetrical visual and auditory pathway infiltration and tracts distribution,which was helpful to diagnosis.