儿童型肾上腺脑白质营养不良的MR诊断  

Diagnosis of MR Images in Childhood Adrenoleukodystrophy

作　　者：肖江喜[1] 杨开颜[1] 蒋学祥[1] 王霄英[1] 高玉洁[1] 

机构地区：[1]北京医科大学第一医院医学影像科,北京100034

出　　处：《实用放射学杂志》2000年第6期327-330,共4页Journal of Practical Radiology

摘　　要：目的 :通过对 AL D的 MR图像上病变分布和信号特点进行分析 ,提高 MR对 AL D诊断的准确性。方法 :本组收集我院 1993～ 1999年间经生化和临床证实的 AL D共 18例 ,所有病例均在我院行 MR检查。结果 :18例病人均表现为对称性双侧脑室三角区白质病变 ,呈 T1 WI低信号 ,T2 WI高信号 ,其中 2例 (11% )仅累及枕叶 ,9例 (5 0 % )累及顶枕叶 ,7例 (39% )累及顶枕颞叶。18例 (10 0 % )累及胼胝体压部和脑干皮质脊髓束 ,16例 (89% )累及丘脑后外部。 4例增强扫描表现为病灶周边环状强化 ,脑干病灶亦强化。结论 :AL D典型表现为双侧对称性侧脑室三角区白质病变并累及脑干和胼胝体压部。 MR为一种无创性的敏感方法 ,能准确反映 ALObjective:To analyse the anatomical distribution and signal feature of childhood adrenoleukodystrophy(ALD)on MR images.Methods:18 cases of ALD proved both biochemically and clinically from 1993 to 1999 was reviewed.All of them underwent MR examination in our hospital.Results:All the case showed a bilaterial symmetrical T 2 and T 1 prolongation changes in the periventricular occipital white matter.There was low signal intensity in T 1 weighted images,and high signal intensity in T 2 weighted images,with only 2 cases(11%) involved the occipital lobe,9 patients(50%)the occipitoparietal lobe and 7 patients(39%) the occipitoparietotemporal lobe.The brain stem and calosal splenium involvement was found in all patients(100%),the lateral posterior part of thalamus in 16 patients(89%).The ring-enhancement of bilaterial occipital lobe was found in all four patients with contrast-enhanced scanning.Conclusion:The typical MR images change of ALD is bilaterial and symmetrical T 1、T 2 prolongation in periventricular occipital white matter with brain stem corticospinal tract and callosal splenium involvement.MR is noninvasive and sensitive diagnostic method for ALD,and can corretly demonstrate the lesion and progression of ALD.

关 键 词：肾上腺脑白质营养不良 儿童 NMR 成像 诊断 

分 类 号：R586.06[医药卫生—内分泌] R742.04[医药卫生—内科学]