进行性多灶性白质脑病临床诊治研究进展

Advances in Clinical Diagnosis and Treatment of Progressive Multifocal Leukoencephalopathy

摘要: 进行性多灶性白质脑病是一种由JC病毒感染少突胶质细胞引发的罕见的神经脱髓鞘疾病，主要发生于免疫功能严重受损的个体。随着免疫抑制剂广泛应用及HIV/AIDS流行，近年来其发病率显著上升。目前，该疾病的发病机制、诊断及治疗方面仍面临诸多挑战，如早期诊断困难、缺乏特效疗法等。本文系统综述进行性多灶性白质脑病的流行病学、发病机制、临床表现、诊断策略及治疗进展。重点探讨免疫重建综合征、新型生物标志物、影像学技术革新以及靶向与免疫疗法的最新研究成果，以期为临床诊疗提供全面参考，并展望未来研究方向。

Abstract: Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating neurological disorder caused by JC virus infection of oligodendrocytes, predominantly occurring in individuals with severe immunosuppression. With the widespread use of immunosuppressants and the prevalence of HIV/AIDS, the incidence of PML has increased significantly in recent years. Currently, the pathogenesis, diagnosis, and treatment of PML pose substantial challenges, including difficulties in early detection and the absence of specific therapeutic agents. This article systematically reviews the epidemiology, pathogenesis, clinical manifestations, diagnostic strategies, and therapeutic advances in PML. It focuses specifically on recent research developments concerning immune reconstitution syndrome, novel biomarkers, advances in imaging modalities, as well as targeted and immunotherapeutic approaches. This review aims to provide a comprehensive reference for clinical management and to outline directions for future research.