以双侧弥漫性脑白质病变为特征的脑淋巴瘤病的影像学研究  

Imaging study of lymphomatosis cerebri characterized by diffuse lesions of bilateral cerebral white matter

作　　者：阮志兵 楚兰 刘春风[3] 胡雨 黄瑾瑾 Ruan Zhibing;Chu Lan;Liu Chunfeng;Hu Yu;Huang Jinjin(Department of Radiology,the Affiliated Hospital of Guizhou Medical University,Guiyang 550004,China;Department of Neurology,the Affiliated Hospital of Guizhou Medical University,Guiyang 550004,China;Department of Neurology,the Second Affiliated Hospital of Soochow University,Suzhou 215004,China;School of Medical Imaging,Guizhou Medical University,Guiyang 550004,China;Department of Pathology,Guizhou Medical University,Guiyang 550004,China)

机构地区：[1]贵州医科大学附属医院影像科,贵阳550004 [2]贵州医科大学附属医院神经内科,贵阳550004 [3]苏州大学附属第二医院神经内科,215004 [4]贵州医科大学医学影像学院,贵阳550004 [5]贵州医科大学病理学教研室,贵阳550004

出　　处：《中华神经科杂志》2021年第11期1168-1175,共8页Chinese Journal of Neurology

基　　金：贵州省科技厅科学基金(黔科合平台人才〔2018年〕5779-72)。

摘　　要：目的探讨脑淋巴瘤病(LC)的多模态影像学特征及临床误诊原因,以提高对该病的认识及早期诊断率。方法连续回顾性收集2011年11月30日至2020年12月28日在贵州医科大学附属医院经病理证实的11例LC患者的临床与影像学资料,对照病理进行分析。结果 LC的主要临床表现为快速进展的认知功能障碍(8/11)、步态不稳(9/11)、行为异常及人格改变(5/11);脑脊液检查结果提示患者有细胞数增多(8/10)、蛋白升高(8/10)、糖含量降低(2/10)、氯化物降低(4/10)。11例LC患者的影像学表现为双侧大脑半球深部脑白质弥漫病变,同时累及脑皮质及皮质下白质8例(8/11)、基底节区7例(7/11)、丘脑5例(5/11)、小脑6例与脑干6例(6/11);11例LC患者的病灶呈等/稍低密度影与稍长T1、稍长T2信号,6例(6/11)早期无明显强化,5例(5/11)以及6例随诊病变出现不均匀斑点、斑片状、结节或团块状明显强化;弥散加权成像显示有9例(9/11)初诊扩散不受限,2例(2/11)及9例随诊病变出现扩散受限呈高信号、表观弥散系数图低信号;氢质子磁共振波谱检查显示5例有不同程度胆碱复合物(Cho)峰升高伴N-乙酰天冬氨酸(NAA)峰与NAA/肌酸下降(5/5),3例出现脂质峰(3/5);行正电子发射断层摄影术/电子计算机体层扫描检查者3例,其中2例(2/3)病灶呈稍高摄取,1例(1/3)未见代谢增高。结论 LC的典型影像学表现为双侧弥漫性脑白质病变,无明显强化及扩散受限,NAA/肌酸降低、Cho/肌酸增加伴脂质峰;对LC认识不足以及穿刺活组织检查不当是误诊的主要原因。Objective To investigate multimodality imaging characteristics and clinical features of lymphomatosis cerebri(LC)and reasons for misdiagnosis,with the goal of potentially facilitating an early and accurate diagnosis for this often-missed disease.Methods Clinical data and cerebral multimodality imaging findings from 11 patients with LC proven basing on pathology in the Affiliated Hospital of Guizhou Medical University from November 30,2011 to December 28,2020 were retrospectively extracted,analyzed,and reviewed in combination with the literatures.Results The common presenting symptoms with subacute onset included cognitive decline(8/11),gait disturbance(9/11),and behavioral disturbance(5/11).Test of cerebrospinal fluid showed that the number of cells and the level of protein increased(8/10),the sugar content(2/10)and chloride(4/10)decreased.The imaging manifestations of 11 patients with LC were diffuse lesions of bilateral cerebral white matter in the both deep and lobar lesion distribution,involving the cerebral cortex and subcortical white matter in eight cases(8/11),basal ganglia in seven cases(7/11),thalamus in five cases(5/11),cerebellum in six cases and brain stem in six cases(6/11).All 11 patients showed equal or slightly low-density shadows on CT plain scan and slightly longer T1WI and T2WI signals on magnetic resonance imaging.Six cases(6/11)had no obvious enhancement in the early stage,and five cases and six follow-up cases showed heterogenous spots,patches,nodules or clusters of distinct enhancement.Diffusion-weighted imaging showed non restricted diffusion in nine(9/11)cases initially diagnosed,and restricted diffusion in two cases(2/11)and nine follow-up cases,which were hyperintense on diffusion-weighted imaging and hypointense on apparent diffusion coefficient maps.Five patients(5/5)presented a marked decrease in N-acetyl aspartic acid(NAA)/creatine(Cr)and increase in choline(Cho)/Cr on hydrogen proton magnetic resonance spectrum,including an increase in lipid/Cr in three cases.One case(1/3)showed n

关 键 词：脑淋巴瘤病 原发性中枢神经系统淋巴瘤 弥漫性脑白质病变 多模态影像学 

分 类 号：R739.4[医药卫生—肿瘤]