幼儿神经轴索营养不良MRI诊断

发布时间：2018-07-10 20:31

本文选题：神经轴突营养不良 + 磁共振成像　；参考：《中国临床医学影像杂志》2015年04期

【摘要】：目的:探讨幼儿神经轴索营养不良(Infantile neuroaxonal dystrophy,INAD)的MRI表现特征,进而提高对该病的影像学认识,为临床早期诊断提供帮助。方法:对本院诊断的13例INAD患者的MRI表现进行回顾分析,男9例,女4例,记录小脑萎缩的部位、程度,小脑皮层、白质信号改变,脑干的异常,基底节及幕上白质信号改变。总结INAD的头颅MRI表现特点。结果:1所有病例均有小脑萎缩,8例为轻度萎缩,5例为重度萎缩;22例伴有大脑萎缩,2例伴有脑干萎缩,1例伴有胼胝体萎缩;32例小脑皮层T2-FLAIR呈高信号;45例幕上脑白质T2WI呈高信号;51例双侧苍白球T2WI、T2-FLAIR、DWI呈低信号。结论:INAD的MRI表现有一定的特点,小脑萎缩是最主要的征象,并且呈进展性;同时可以伴有大脑及脑干的萎缩;小脑皮层T2WI信号增高;基底节铁沉积增多所致对称性T2WI信号减低;幕上白质脱髓鞘,主要表现为深部脑白质T2WI信号增高;灰质核团较少受累。
[Abstract]:Objective: to investigate the MRI features of infantile neuroaxonal dystrophy (INAD) in order to improve the imaging understanding of the disease and to provide help for early clinical diagnosis. Methods: MRI findings of 13 patients with INAD diagnosed in our hospital were retrospectively analyzed, including 9 males and 4 females. The location and degree of cerebellar atrophy, the changes of cerebellar cortex and white matter signal, the abnormality of brain stem, the signal changes of basal ganglia and supratentorial white matter were recorded. To summarize the cranial MRI features of INAD. Results all cases had cerebellar atrophy in 8 cases, mild atrophy in 5 cases, severe atrophy in 22 cases, brain stem atrophy in 2 cases, cortical atrophy of corpus callosum in 1 case and corpus callosum atrophy in 32 cases. T2-flair of cerebellar cortex showed high signal intensity in 45 cases of supratentorial white matter. In 51 cases of bilateral globus pallidus, T _ 2 WI T _ 2-FLAIRI DWI showed hypointensity on T _ 2WI. Conclusion there are some characteristics in the MRI features of the brain, cerebellar atrophy is the most important sign and is progressive, it can be accompanied by brain and brain stem atrophy, cerebellar cortex T2WI signal is increased, the basal ganglia iron deposition is increased, the symmetrical T 2WI signal is decreased. Supratentorial white matter demyelination was mainly characterized by increased T _ 2WI signal in the deep white matter and less involvement of gray matter nuclei.
【作者单位】：航天中心医院影像科;北京大学第一医院;
【分类号】：R748;R445.2

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