Journal of Diagnostics Concepts & Practice ›› 2026, Vol. 25 ›› Issue (02): 131-140.doi: 10.16150/j.1671-2870.2026.02.003
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ZHANG Bei1, GUAN Ai2,*, ZHANG Xiaoyu2, LI Wanlin2, TANG Liufeng2, ZHAO Zhenbo2, WANG Hualong1(
)
Received:2025-12-29
Revised:2026-03-17
Accepted:2026-03-18
Online:2026-04-25
Published:2026-04-25
Contact:
WANG Hualong
E-mail:wanghualong@renji.com
CLC Number:
ZHANG Bei, GUAN Ai, ZHANG Xiaoyu, LI Wanlin, TANG Liufeng, ZHAO Zhenbo, WANG Hualong. Features and advances in diagnosis and treatment of atypical Parkinson syndromes[J]. Journal of Diagnostics Concepts & Practice, 2026, 25(02): 131-140.
Table 1
Comparison of key characteristics of subtypes of APS
| 亚型 | 主要病理蛋白 | 核心临床特征 | 影像学特征(代表性) | 诊断标准要点 | 治疗要点 |
|---|---|---|---|---|---|
| MSA | α突触核蛋白(少突胶质细胞;主要累及纹状体-黑质、小脑-脑桥-橄榄及自主神经中枢) | 早期自主神经功能障碍(直立性低血压、泌尿生殖功能障碍),帕金森样症状或小脑共济失调 | MRI:桥脑“十字征”、壳核边缘征。FDG-PET:纹状体/小脑低代谢 | 四级诊断:神经病理确诊、临床确诊、临床很可能、前驱可能。临床确诊需满足散发、进展性、成年起病(>30岁)的基本特征,同时具有核心临床表现,至少存在2项支持性临床表现(包括运动特征如快速进展、姿势不稳、吞咽困难、巴宾斯基征等,以及非运动特征如喘鸣、手足发冷、病理性哭笑等),至少存在1项MRI标志,不存在排除性的临床表现 | 以自主神经症状管理为核心,运动症状对左旋多巴反应有限,重视康复支持 |
| DLB | α突触核蛋白(神经元;以大脑皮层和边缘系统为主) | 波动性认知障碍、视幻觉、帕金森样症状、快速眼动睡眠行为障碍 | DAT-SPECT/PET:纹状体摄取下降。FDG-PET:枕叶低代谢伴“扣带回岛征”。¹²³I-MIBG:心肌摄取降低 | 二级诊断:很可能DLB,可能的DLB。很可能的DLB需满足≥2项核心特征,或1项核心+1项指示性生物标志物。可能的DLB需仅具有1个核心特征,或者1个或多个提示性生物标志物阳性 | 首选胆碱酯酶抑制剂改善认知与精神症状,慎用抗精神病药物 |
| PSP | 4R-tau(以中脑、基底节等深部结构为主) | 垂直性核上性眼肌麻痹,早期姿势不稳和跌倒,中轴性强直 | MRI:中脑萎缩,“蜂鸟征”,中脑-脑桥比值降低。FDG-PET:额叶、丘脑低代谢 | 四级诊断:确诊PSP,很可能PSP,可能PSP,提示PSP。基于眼动障碍、姿势不稳、运动不能、认知障碍四大功能域的组合进行诊断,并纳入了8个临床亚型 | 以对症和康复治疗为主,抗帕金森药物尝试,重在防跌倒管理 |
| CBD | 4R-tau(主要累及额顶叶皮层及皮层下结构,呈不对称分布) | 非对称性运动障碍,显著皮层征(失用、异己肢、皮质感觉障碍) | MRI:额-顶叶不对称萎缩。FDG-PET:对应皮层低代谢 | 三级诊断:病理确诊CBD、很可能CBD、可能CBD 核心在于区分病理CBD与临床CBS。诊断基于四大临床表型:经典型CBS、额叶行为空间综合征、非流利性原发性进行性失语及进行性核上性麻痹综合征 | 对症治疗为主,肉毒毒素缓解局灶性肌张力障碍,强调多学科康复 |
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