进行性多灶性白质脑病的临床诊治分析:附病例报道

Diagnosis and Treatment of Progressive Multifocal Leukoencephalopathy:A Case Report

目的探讨进行性多灶性白质脑病(PML)的临床诊治特点,以早期诊断和治疗。方法回顾性分析温州医科大学附属第一医院2009-12-27收治的1例弥漫大B细胞淋巴瘤并发PML患者的临床资料,并结合文献总结其入院查体、实验室检查、影像学检查、病理学检查结果及诊治经过。结果本例患者临床表现为视觉障碍、认知功能减退和精神行为异常等常见PML症状;脑脊液中检测到JC病毒;颅脑MRI显示两侧半卵圆中心、侧脑室旁多发T1FLAIR低信号、T2WI和弥散加权成像(DWI)高信号病灶,颅脑MRI增强后未见明显强化改变,无占位效应,符合典型的PML影像学表现。给予退热、补液,纠正贫血、低蛋白血症等积极治疗,但患者病情仍迅速进展,自动出院1d后死亡。结论 PML临床表现多样,神经系统表现多为高级皮质功能减退,脑脊液JC病毒及颅脑MRI检查有利于早期诊断。治疗以对症治疗为主,但预后较差。

Objective To investigate the diagnosis and treatment of progressive multifocal leukoencephalopathy （PML） and its early diagnosis and treatment. Methods This study analyzed retrospectively the clinical data of 1 patient with diffuse large B - cell lymphoma complicated by PML and combined with literatures, summarized his admission examination, laboratory test, imaging studies, pathology findings, process of diagnosis and treatment. Results The patient presented with depraved sision, cognitive hypofunction, behavioral abnormalities and other PML symptoms. JC virus was detected in cerebrospinal fluid （CSF）. By brain MRI, T1 FLAIR low signal, T2 WI and diffusion - weighted imaging （DWI） occurred in bilateral semi - oval center and beside lateral cerebral ventricle, and no obvious enhancement changes after enhanced MRI, without mass effect, in line with the typical PML imaging findings. After pyretolysis, fluid replacement, correction of anemia, hypoalbuminemia and other active treatments, the patient＇s condition progressed rapidly, discharged automatically and died after 1 d. Conclusion The manifestations of PML are multiple. Higher cortical dysfunction is presented in neurological system. CSF JC virus and brain MRI helps early diagnosis. Symptomatic treatment is the main method, but has poor prognosis.