Pathomechanisms of neuropsychiatric disturbances in atypical parkinsonian disorders: a current view.

IF 3.2 4区 医学 Q2 CLINICAL NEUROLOGY
Journal of Neural Transmission Pub Date : 2025-04-01 Epub Date: 2025-02-15 DOI:10.1007/s00702-025-02890-7
Kurt A Jellinger
{"title":"Pathomechanisms of neuropsychiatric disturbances in atypical parkinsonian disorders: a current view.","authors":"Kurt A Jellinger","doi":"10.1007/s00702-025-02890-7","DOIUrl":null,"url":null,"abstract":"<p><p>Multiple system atrophy (MSA), corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) are the most common atypical parkinsonisms. These adult-onset and lethal neurodegenerative disorders of unknown etiology are clinically characterized by varying combinations of autonomic, levodopa-poorly responsive parkinsonsm, motor, non-motor, cerebellar syndromes, behavioral, cognitive and other neuropsychiatric disorders. Although their pathological hallmarks are different-MSA α-synucleinopathy, CBD and PSP 4-repeat (4R) tauopathies-their neuropsychiatric disturbances include anxiety, depression, agitations, attention-executive dysfunctions, less often compulsive and REM sleep behavior disorders (RBD), which may contribute to disease progression and reduced quality of life (QoL) of patients and caregivers. The present paper reviews the prevalence and type of neuropsychiatric profile in these atypical parkinsonian syndromes, their neuroimaging, and pathogenic backgrounds based on extensive literature research. MSA patients show anxiety, apathy (depression), initial RBD, attentional and executive dysfunction; PSP patients present with apathy, depression, disinhibition, and to a lesser extent, anxiety and agitation; CBD patients are featured by executive and visuospatial dysfunctions, irritability, alien limb phenomena, sleep and language disorders. Neuropsychiatric disorders in these syndromes are often similar, due to disruption of prefronto-subcortical (limbic) and striato-thalamo-cortical circuitries or default mode and attention network disorder. This supports the concept that they are brain network disorders due to complex pathogenic mechanisms related to the basic proteinopathies that are still poorly understood. Psychotic symptoms, hallucinations and delusions are rare. Neuropsychiatric changes in these disorders are often premature and anticipate motor dysfunctions; their assessment and further elucidation of their pathogenesis are warranted as a basis for early diagnosis and adequate treatment of these debilitating comorbidities.</p>","PeriodicalId":16579,"journal":{"name":"Journal of Neural Transmission","volume":" ","pages":"495-518"},"PeriodicalIF":3.2000,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Neural Transmission","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00702-025-02890-7","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/2/15 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0

Abstract

Multiple system atrophy (MSA), corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) are the most common atypical parkinsonisms. These adult-onset and lethal neurodegenerative disorders of unknown etiology are clinically characterized by varying combinations of autonomic, levodopa-poorly responsive parkinsonsm, motor, non-motor, cerebellar syndromes, behavioral, cognitive and other neuropsychiatric disorders. Although their pathological hallmarks are different-MSA α-synucleinopathy, CBD and PSP 4-repeat (4R) tauopathies-their neuropsychiatric disturbances include anxiety, depression, agitations, attention-executive dysfunctions, less often compulsive and REM sleep behavior disorders (RBD), which may contribute to disease progression and reduced quality of life (QoL) of patients and caregivers. The present paper reviews the prevalence and type of neuropsychiatric profile in these atypical parkinsonian syndromes, their neuroimaging, and pathogenic backgrounds based on extensive literature research. MSA patients show anxiety, apathy (depression), initial RBD, attentional and executive dysfunction; PSP patients present with apathy, depression, disinhibition, and to a lesser extent, anxiety and agitation; CBD patients are featured by executive and visuospatial dysfunctions, irritability, alien limb phenomena, sleep and language disorders. Neuropsychiatric disorders in these syndromes are often similar, due to disruption of prefronto-subcortical (limbic) and striato-thalamo-cortical circuitries or default mode and attention network disorder. This supports the concept that they are brain network disorders due to complex pathogenic mechanisms related to the basic proteinopathies that are still poorly understood. Psychotic symptoms, hallucinations and delusions are rare. Neuropsychiatric changes in these disorders are often premature and anticipate motor dysfunctions; their assessment and further elucidation of their pathogenesis are warranted as a basis for early diagnosis and adequate treatment of these debilitating comorbidities.

非典型帕金森病中神经精神障碍的病理机制:当前观点。
多系统萎缩(MSA)、皮质基底变性(CBD)和进行性核上性麻痹(PSP)是最常见的非典型帕金森病。这些病因不明的成人发病和致死性神经退行性疾病的临床特征是自主神经、左旋多巴反应不良的帕金森症、运动、非运动、小脑综合征、行为、认知和其他神经精神疾病的不同组合。尽管他们的病理特征不同——msa α-突触核蛋白病、CBD和PSP 4-重复(4R) tau病——但他们的神经精神障碍包括焦虑、抑郁、躁动、注意力执行功能障碍、较少的强迫和快速眼动睡眠行为障碍(RBD),这可能导致疾病进展和患者及护理人员的生活质量(QoL)下降。本文在大量文献研究的基础上,综述了这些非典型帕金森综合征的患病率和神经精神特征类型、神经影像学和发病背景。MSA患者表现为焦虑、冷漠(抑郁)、首发RBD、注意力和执行功能障碍;PSP患者表现为冷漠、抑郁、去抑制,并在较小程度上表现为焦虑和躁动;CBD患者的特点是执行和视觉空间功能障碍、易怒、异肢现象、睡眠和语言障碍。由于前额叶-皮层下(边缘)和纹状体-丘脑-皮层回路或默认模式和注意网络障碍的破坏,这些综合征中的神经精神障碍通常是相似的。这支持了这一概念,即它们是由于与基本蛋白质病变相关的复杂致病机制而导致的大脑网络疾病,而这些机制目前仍知之甚少。精神症状、幻觉和妄想很少见。这些疾病的神经精神变化往往是过早的,并预示着运动功能障碍;它们的评估和进一步阐明其发病机制是早期诊断和适当治疗这些衰弱性合并症的基础。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
Journal of Neural Transmission
Journal of Neural Transmission 医学-临床神经学
CiteScore
7.20
自引率
3.00%
发文量
112
审稿时长
2 months
期刊介绍: The investigation of basic mechanisms involved in the pathogenesis of neurological and psychiatric disorders has undoubtedly deepened our knowledge of these types of disorders. The impact of basic neurosciences on the understanding of the pathophysiology of the brain will further increase due to important developments such as the emergence of more specific psychoactive compounds and new technologies. The Journal of Neural Transmission aims to establish an interface between basic sciences and clinical neurology and psychiatry. It intends to put a special emphasis on translational publications of the newest developments in the field from all disciplines of the neural sciences that relate to a better understanding and treatment of neurological and psychiatric disorders.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信