脑淋巴瘤病临床特点及MRI表现的系统评价

A systematic review of clinical and MRI features of lymphomatosis cerebri

目的:总结脑淋巴瘤病的临床及影像学特征。方法:结合我院3例脑淋巴瘤,检索Pubmed数据库中有关脑淋巴瘤的文献,筛选后提取临床资料、影像表现及病理结果等进行总结分析。结果:共纳入42篇文献(50例),加我院3例,共分析53例脑淋巴瘤。53例中,男女比例0.96∶1,年龄19~85岁,平均(48.0±20.4)岁。临床以进行性认知下降(50.9%,27/53)和步态不稳(37.7%,20/53)为主要表现。首次MRI检查92.5%(49/53)的患者表现为双侧大脑半球深部白质非对称性弥漫性信号异常,其中47.2%(25/53)的患者同时有幕上及幕下浸润。首次MRI增强扫描常表现为无强化(60.4%,32/53)或轻微线样或补丁样强化(39.6%,21/53)。脑淋巴瘤有沿皮质脊髓束侵犯的趋势,可累及基底节、丘脑、脑干、小脑,甚至脊髓。结论:脑淋巴瘤有一定的临床及影像学特征,提高认识,及早活检,有助于改善患者预后。

Objective:To characterize the clinical and neuroradiological features of lymphomatosis cerebri(LC)in order to improve early diagnosis.Methods:Combined with 3 LC cases in our hospital,the literatures on LC in the Pubmed databases were reviewed.After screening,the clinical data,imaging findings and pathological results were extracted and summarized.Results:42 articles were included for 50 previously reported cases,adding our own 3 cases,53 LC cases were analyzed.The average age of onset of LC was(48.0±20.4)years old(range:19~85 years old)with neither sex being predominant(male-to-female ratio 0.96∶1).The most common presenting symptom was progressive cognitive decline(50.9%)and gait instability(37.7%).The first MRI showed bilateral hemispheric involvement in 92.5%and supratentorial and infratentorial infiltration in 47.1%of patients.60.4%patients without contrast enhancement and 39.6%who presented slight non-mass-like enhancement in the first MRI.Lesion extension was described with a tendency spreading along the corticospinal tract to the basal ganglia,thalamus,brainstem,cerebellum,and even the spinal cord.Conclusions:LC has certain clinical and imaging features.Prompt recognition of these imaging patterns may lead to early diagnosis of LC and brain biopsy with improved prognosis.