诊断学理论与实践

诊断学理论与实践 >

2026 , Vol. 25 >Issue 02: 131 - 140

DOI: https://doi.org/10.16150/j.1671-2870.2026.02.003

专家论坛

非典型帕金森综合征的特征及诊治进展

  • 张贝 ,
  • 关爱 ,
  • 张小钰 ,
  • 李宛霖 ,
  • 唐浏丰 ,
  • 赵振博 ,
  • 王华龙
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  • 1 上海交通大学医学院附属仁济医院神经内科， 上海 200127
    2 河北医科大学第一医院神经内科， 河北 石家庄 050000
*:并列第一作者
王华龙 E-mail：wanghualong@renji.com

收稿日期: 2025-12-29

  修回日期: 2026-03-17

  录用日期: 2026-03-18

  网络出版日期: 2026-04-25

基金资助

政府资助临床医学优秀人才项目(ZF2025047);河北省自然科学基金-重点项目(H2024206494)

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Features and advances in diagnosis and treatment of atypical Parkinson syndromes

  • ZHANG Bei ,
  • GUAN Ai ,
  • ZHANG Xiaoyu ,
  • LI Wanlin ,
  • TANG Liufeng ,
  • ZHAO Zhenbo ,
  • WANG Hualong
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  • 1 Department of Neurology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China
    2 Department of Neurology, The First Hospital of Hebei Medical University, Hebei Shijiazhuang 050000, China

Received date: 2025-12-29

  Revised date: 2026-03-17

  Accepted date: 2026-03-18

  Online published: 2026-04-25

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摘要

帕金森综合征（Parkinson syndrome, PDS）是一组以运动迟缓、肌强直、静止性震颤和姿势不稳为核心表现的神经系统综合征，主要包括原发性帕金森病（Parkinson's disease, PD）、非典型帕金森综合征（atypical Parkinson syndromes, APS）以及继发性帕金森综合征等类型。非典型帕金森综合征（atypical Parkinson syndromes，APS）是一组以帕金森样运动障碍为核心表现，但在病理机制、临床特征、影像学表现、药物反应及预后方面与典型帕金森病显著不同的神经退行性疾病，主要包括多系统萎缩、路易体痴呆、进行性核上性麻痹和皮质基底节变性。近年来，随着诊断标准的不断更新以及多模态影像学和神经病理研究的深入，不同APS亚型在临床表型和疾病谱系中的定位逐渐清晰。治疗方面，APS目前仍以对症和支持治疗为主，针对α突触核蛋白和tau异常聚焦过程的疾病修饰治疗、干细胞治疗等新策略正在探索之中。本文系统综述了APS各亚型的临床及影像学特征、诊断标准演变和治疗进展，旨在为临床早期识别和规范化管理提供参考。

关键词： 非典型帕金森综合征; 多系统萎缩; 路易体痴呆; 进行性核上性麻痹; 皮质基底节变性

本文引用格式

张贝 , 关爱 , 张小钰 , 李宛霖 , 唐浏丰 , 赵振博 , 王华龙 . 非典型帕金森综合征的特征及诊治进展[J]. 诊断学理论与实践, 2026 , 25(02) : 131 -140 . DOI: 10.16150/j.1671-2870.2026.02.003

Abstract

Parkinson syndrome (PDS) is a group of neurological syndromes characterized by bradykinesia, rigidity, resting tremor, and postural instability as core manifestations, mainly including Parkinson's disease (PD), atypical Parkinson syndromes (APSs), and secondary Parkinson syndromes. APSs are a group of neurodegenerative diseases characterized by parkinsonian motor disorders as core manifestations, but they differ significantly from typical Parkinson's disease in terms of pathological mechanisms, clinical features, imaging findings, drug responses, and prognosis. They mainly include multiple system atrophy, dementia with Lewy bodies, progressive supranuclear palsy, and corticobasal degeneration. In recent years, with the continuous updating of diagnostic criteria and the in-depth research on multimodal imaging and neuropathology, the positioning of different APS subtypes in clinical phenotypes and disease spectra has gradually become clear. In terms of treatment, APSs currently still rely primarily on symptomatic and supportive therapies. New strategies, including disease-modifying therapies targeting abnormal aggregation of α-synuclein and tau protein, as well as stem cell therapy, are being explored. This article systematically reviews the clinical and imaging features, evolution of diagnostic criteria, and treatment progress of each subtype of APSs, aiming to provide a reference for early clinical identification and standardized management.

Key words： Atypical Parkinson syndromes; Multiple system atrophy; Dementia with Lewy bodies; Progressive supranuclear palsy; Corticobasal degeneration

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