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进行性多灶性白质脑病的磁共振成像表现概述

Overview of MRI findings in progressive multifocal leukoencephalopathy.

作者信息

Mori Koichiro, Kurokawa Mariko, Harada Masafumi, Nakamichi Kazuo, Arai Hideo, Takao Masaki, Takaki Yasunobu, Miura Yoshiharu

机构信息

Department of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-Ku, Tokyo, 113-8677, Japan.

Department of Radiology, The University of Tokyo, Tokyo, Japan.

出版信息

Jpn J Radiol. 2025 Jul 21. doi: 10.1007/s11604-025-01837-y.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by JC virus (JCV) infection. PML affects patients with various underlying conditions, such as HIV/AIDS, hematological malignancies, organ transplants, autoimmune diseases, or multiple sclerosis particularly those receiving disease-modifying therapies. MRI plays a crucial role in diagnosis, demonstrating characteristic findings across multiple sequences, including T2-weighted imaging (T2WI)/fluid-attenuated inversion recovery (FLAIR), T1-weighted imaging (T1WI), diffusion-weighted imaging (DWI), and susceptibility-weighted imaging (SWI). Early stage markers first appear as a cluster of punctate high-signal areas in T2WI (the "punctate pattern") and later develop into a distribution of oval-shaped lesions of varying sizes, commonly referred to as the "milky way appearance." Lesions typically show T2WI/FLAIR hyperintensity, T1WI hypointensity, and DWI hyperintensity. Recent findings highlight the significance of SWI hypointensity as a potential early marker. The prognosis varies significantly depending on the underlying condition and timing of diagnosis, with mortality rates ranging from 20 to 90%. Early detection, particularly in asymptomatic stages, significantly improves survival rates, emphasizing the importance of regular MRI screening in high-risk patients. Diagnostic challenges include low JCV DNA levels in cerebrospinal fluid (CSF), particularly in early stages and drug-associated cases, necessitating ultrasensitive PCR testing. This review provides an overview of PML's imaging characteristics, with particular emphasis on early diagnostic features using MRI, with a detailed understanding of PML's imaging characteristics across various stages and clinical subtypes, aiming to improve patient outcomes through early detection and intervention.

摘要

进行性多灶性白质脑病(PML)是由JC病毒(JCV)感染引起的一种严重的中枢神经系统脱髓鞘疾病。PML影响患有各种基础疾病的患者,如艾滋病毒/艾滋病、血液系统恶性肿瘤、器官移植、自身免疫性疾病或多发性硬化症,特别是那些接受疾病修饰疗法的患者。MRI在诊断中起着至关重要的作用,在多个序列上显示出特征性表现,包括T2加权成像(T2WI)/液体衰减反转恢复序列(FLAIR)、T1加权成像(T1WI)、扩散加权成像(DWI)和磁敏感加权成像(SWI)。早期标志物首先在T2WI上表现为一簇点状高信号区(“点状模式”),随后发展为大小不一的椭圆形病灶分布,通常称为“银河外观”。病灶通常表现为T2WI/FLAIR高信号、T1WI低信号和DWI高信号。最近的研究结果强调了SWI低信号作为潜在早期标志物的重要性。预后因基础疾病和诊断时间的不同而有很大差异,死亡率在20%至90%之间。早期检测,尤其是在无症状阶段,可显著提高生存率,强调了对高危患者进行定期MRI筛查的重要性。诊断挑战包括脑脊液(CSF)中JCV DNA水平较低,特别是在早期和药物相关病例中,需要进行超灵敏PCR检测。本综述概述了PML的影像学特征,特别强调了使用MRI的早期诊断特征,详细了解PML在各个阶段和临床亚型的影像学特征,旨在通过早期检测和干预改善患者预后。

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