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非典型帕金森综合征中的不对称性——综述

Asymmetry in Atypical Parkinsonian Syndromes-A Review.

作者信息

Chunowski Patryk, Madetko-Alster Natalia, Alster Piotr

机构信息

Department of Neurology, Medical University of Warsaw, 03-242 Warsaw, Poland.

出版信息

J Clin Med. 2024 Sep 28;13(19):5798. doi: 10.3390/jcm13195798.

Abstract

Atypical parkinsonian syndromes (APSs) are a group of neurodegenerative disorders that differ from idiopathic Parkinson's disease (IPD) in their clinical presentation, underlying pathology, and response to treatment. APSs include conditions such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), and dementia with Lewy bodies (DLB). These disorders are characterized by a combination of parkinsonian features and additional symptoms, such as autonomic dysfunction, supranuclear gaze palsy, and asymmetric motor symptoms. Many hypotheses attempt to explain the causes of neurodegeneration in APSs, including interactions between environmental toxins, tau or α-synuclein pathology, oxidative stress, microglial activation, and vascular factors. While extensive research has been conducted on APSs, there is a limited understanding of the symmetry in these diseases, particularly in MSA. Neuroimaging studies have revealed metabolic, structural, and functional abnormalities that contribute to the asymmetry in APSs. The asymmetry in CBS is possibly caused by a variable reduction in striatal D2 receptor binding, as demonstrated in single-photon emission computed tomography (SPECT) examinations, which may explain the disease's asymmetric manifestation and poor response to dopaminergic therapy. In PSP, clinical dysfunction correlates with white matter tract degeneration in the superior cerebellar peduncles and corpus callosum. MSA often involves atrophy in the pons, putamen, and cerebellum, with clinical symmetry potentially depending on the symmetry of the atrophy. The aim of this review is to present the study findings on potential symmetry as a tool for determining potential neuropsychological disturbances and properly diagnosing APSs to lessen the misdiagnosis rate. A comprehensive review of the academic literature was conducted using the medical literature available in PubMed. Appropriate studies were evaluated and examined based on patient characteristics and clinical and imaging examination outcomes in the context of potential asymmetry. Among over 1000 patients whose data were collected, PSP-RS was symmetrical in approximately 84% ± 3% of cases, with S-CBD showing similar results. PSP-P was symmetrical in about 53-55% of cases, while PSP-CBS was symmetrical in fewer than half of the cases. MSA-C was symmetrical in around 40% of cases. It appears that MSA-P exhibits symmetry in about 15-35% of cases. CBS, according to the criteria, is a disease with an asymmetrical clinical presentation in 90-99% of cases. Similar results were obtained via imaging methods, but transcranial sonography produced different results. Determining neurodegeneration symmetry may help identify functional deficits and improve diagnostic accuracy. Patients with significant asymmetry in neurodegeneration may exhibit different neuropsychological symptoms based on their individual brain lateralization, impacting their cognitive functioning and quality of life.

摘要

非典型帕金森综合征(APSs)是一组神经退行性疾病,在临床表现、潜在病理和对治疗的反应方面与特发性帕金森病(IPD)不同。APSs包括多系统萎缩(MSA)、进行性核上性麻痹(PSP)、皮质基底节综合征(CBS)和路易体痴呆(DLB)等病症。这些疾病的特征是帕金森特征与其他症状的组合,如自主神经功能障碍、核上性凝视麻痹和不对称运动症状。许多假说试图解释APSs中神经退行性变的原因,包括环境毒素、tau或α-突触核蛋白病理、氧化应激、小胶质细胞激活和血管因素之间的相互作用。虽然对APSs进行了广泛研究,但对这些疾病中的对称性了解有限,尤其是在MSA中。神经影像学研究揭示了导致APSs不对称的代谢、结构和功能异常。CBS中的不对称可能是由纹状体D2受体结合的可变减少引起的,如单光子发射计算机断层扫描(SPECT)检查所示,这可能解释了该疾病的不对称表现和对多巴胺能治疗的不良反应。在PSP中,临床功能障碍与上小脑脚和胼胝体中的白质束变性相关。MSA常涉及脑桥、壳核和小脑萎缩,临床对称性可能取决于萎缩的对称性。本综述的目的是介绍关于潜在对称性的研究结果,作为确定潜在神经心理障碍和正确诊断APSs以降低误诊率的工具。使用PubMed上可用的医学文献对学术文献进行了全面综述。根据患者特征以及潜在不对称情况下的临床和影像学检查结果,对适当的研究进行了评估和审查。在收集数据的1000多名患者中,PSP-RS在约84%±3%的病例中是对称的,S-CBD显示出类似结果。PSP-P在约53-55%的病例中是对称的,而PSP-CBS在不到一半的病例中是对称的。MSA-C在约40%的病例中是对称的。MSA-P似乎在约15-35%的病例中表现出对称性。根据标准,CBS在90-99%的病例中是临床表现不对称的疾病。通过影像学方法也获得了类似结果,但经颅超声检查产生了不同结果。确定神经退行性变的对称性可能有助于识别功能缺陷并提高诊断准确性。神经退行性变存在显著不对称的患者可能根据其个体脑侧化表现出不同的神经心理症状,影响其认知功能和生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/245f/11477316/422884344318/jcm-13-05798-g001.jpg

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