儿童黏多糖贮积症颅脑MRI影像学表现

MRI Imaging Findings of Brain in Children with Mucopolysaccharidosis

目的探讨儿童黏多糖贮积症(MPS)的颅脑MRI表现。方法回顾性分析2014年至2017年本院诊治为MPS的9例患儿的颅脑MRI影像资料。检查序列包括T_1WI、T_2WI、液体衰减反转恢复(FLAIR)、扩散加权成像(DWI)序列。结果 9例颅脑内均可见多发小囊状信号呈放射状分布;9例脑白质内均可见片状长T_1、长T_2信号,FLAIR呈高信号,无扩散受限;7例幕上脑室扩张,其中1例合并脑萎缩;蛛网膜下腔间隙增宽以后颅窝及蝶鞍为主,其中1例合并后颅窝蛛网膜囊肿;4例T_2WI矢状位示颅颈交界区椎管前后径缩小,脑脊液空间呈受压改变;6例头型狭长,其中4例颅骨板障增厚。结论 MPS颅脑MRI表现总体可归纳为:血管周围间隙扩大、脑白质异常信号、脑脊液空间增大以及颅颈交界区狭窄。

Objective To study the MRI features of brain in children with mucopolysaccharidosis(MPS). Methods A retrospective analysis was performed on MRI findings of brain in 9 cases of children with mucopolysaccharidosis diagnosed in our hospital between 2014 and 2017. The sequence groups included T1WI, T2WI, FLAIR, and DWI. Results 9 patients had multiple cystic areas radially,plaque-like white matter lesions displaying long T1 and long T2 signal intensities were revealed in 9 patients,and FLAIR sequence showed high signal without diffusion restriction. 7 patients showed supratentorial ventricular dilatation,and 1 patient showed cerebral atrophy. Cranial fossa and sella turcica were the main types of subarachnoid space widening. One of them had posterior cranial fossa arachnoid cyst. T2WI showed that the diameter of the vertebral canal decreased and cerebrospinal fluid space had visible compression in 4 patients with craniocervical junction area stenosis. 6 patients had long and narrow head-shapes,in which 4 patients had thickened skull plates. Conclusion The brain MRI findings of MPS can be summarized into four main points:enlarged perivascular space,abnormal white matter signal,increased cerebrospinal fluid space and narrow craniocervical junction.