Jellinger Kurt A
Institute of Clinical Neurobiology, Alberichgasse 5/13, A-1150, Vienna, Austria.
J Neural Transm (Vienna). 2025 Jun 5. doi: 10.1007/s00702-025-02960-w.
Vascular parkinsonism (VP), resulting from cerebrovascular disease, is a rare disorder with a characteristic motor and non-motor clinical profile distinct from sporadic/idiopathic Parkinson disease (PD) and other parkinsonian disorders. It accounts for 3-6% of all parkinsonian syndromes and may overlap with other parkinsonisms. Clinical features of VP are heterogenous and include bilateral rigidity with lower body predominance, bradykinesia, postural instability, shuffling gait, falls, corticospinal symptoms and cognitive impairment, tremor being rare or absent. An international working group recommended three VP subtypes: (1) the acute or subacute poststroke type: asymmetric parkinsonism due to involvement of the nigrostriatal system and response to dopaminergic drugs, (2) the more frequent insidious onset subtype due to ischemic deep white matter lesions and/or lacunar infarcts presents with progressive, symmetrical parkinsonism, prominent postural instability, gait impairment, corticospinal, pseudobulbar, urinary and cognitive symptoms, and poor levodopa response; (3) mixed VP/PD and other neurodegenerative parkinsonisms showing overlaps between these forms, with upper and lower body rigidity, resting tremor, dementia and positive levodopa response. Neuroimaging shows brain atrophy, widespread deep white matter lesions, lacunar infarcts and rare direct damage to nigrostriatal areas. Advanced MRI techniques and dopamine transporter imaging may be useful in the differentiation of VP with PD and other neurodegenerative parkinsonian syndromes. Neuropathology of VP reveals multiple subcortical ischemic lesions due to small vessel disease in basal ganglia and deep white matter, less often lesions of striatum, and substantia nigra involving cortico-basal ganglia-cortical and other neuronal circuits. Lewy pathology is usually absent. New molecular biomarkers will help to differentiate VP from other parkinsonian syndromes. The response of VP to different therapeutic strategies is modest. Further studies are warranted to explore the role of vascular lesions in the pathogenesis of VP and to demonstrate the efficacy of new therapy options.
血管性帕金森综合征（VP）由脑血管疾病引起，是一种罕见的疾病，其运动和非运动临床特征有别于散发性/特发性帕金森病（PD）及其他帕金森综合征。它占所有帕金森综合征的3%至6%，可能与其他帕金森综合征重叠。VP的临床特征具有异质性，包括以下表现：以双下肢为主的双侧强直、运动迟缓、姿势不稳、拖步、跌倒、皮质脊髓症状和认知障碍，震颤少见或无震颤。一个国际工作组推荐了三种VP亚型：（1）急性或亚急性卒中后型：由于黑质纹状体系统受累导致不对称性帕金森综合征，对多巴胺能药物有反应；（2）更常见的隐匿性起病亚型，由缺血性深部白质病变和/或腔隙性梗死引起，表现为进行性、对称性帕金森综合征，突出的姿势不稳、步态障碍、皮质脊髓、假性球麻痹、泌尿和认知症状，对左旋多巴反应不佳；（3）混合型VP/PD及其他神经退行性帕金森综合征，表现为这些类型之间的重叠，有上下身强直、静止性震颤、痴呆和左旋多巴反应阳性。神经影像学显示脑萎缩、广泛的深部白质病变、腔隙性梗死以及黑质纹状体区域罕见的直接损害。先进的磁共振成像技术和多巴胺转运体成像可能有助于鉴别VP与PD及其他神经退行性帕金森综合征。VP的神经病理学显示，基底节和深部白质的小血管疾病导致多个皮质下缺血性病变，纹状体和黑质病变较少见，涉及皮质-基底节-皮质及其他神经回路。通常不存在路易体病理改变。新的分子生物标志物将有助于鉴别VP与其他帕金森综合征。VP对不同治疗策略的反应一般。有必要进一步研究以探讨血管病变在VP发病机制中的作用，并证明新治疗方案的疗效。
