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进行性核上性麻痹:过去和现在的方面。

The Progressive Supranuclear Palsy: Past and Present Aspects.

机构信息

3rd Age Day Care Center IASIS, Glyfada, Greece.

Institute of Child Health, Research Center, "Aghia Sophia" Children's Hospital, Athens, Greece.

出版信息

Clin Gerontol. 2020 Mar-Apr;43(2):155-180. doi: 10.1080/07317115.2019.1694115. Epub 2019 Nov 22.

Abstract

: Recently, new criteria for sensitive and specific clinical diagnosis of progressive supranuclear palsy (PSP) have been addressed while distinct clinical phenotypes of the disorder have been increasingly described in the literature. This study aimed to describe past and present aspects of the disease as well as to highlight the cognitive and behavioral profile of PSP patients in relation to the underlying pathology, genetics and treatment procedures.: A Medline and Scopus search was performed to identify articles published on this topic. Articles published solely in English were considered.: The most common clinical characteristics of PSP included early postural instability and falls, vertical supranuclear gaze palsy, parkinsonism with poor response to levodopa and pseudobulbar palsy. Frontal dysfunction and verbal fluency deficits were the most distinct cognitive impairments in PSP while memory, visuospatial and social cognition could also be affected. Apathy and impulsivity were also present in PSP patients and had significant impact on relatives and caregivers.: PSP is a neurodegenerative disorder with prominent tau neuropathology. Movement, motivation and communication impairments in patients with PSP may limit participation in everyday living activities. Comprehensive neuropsychological assessments are of significant importance for PSP cognitive evaluation. Pharmacologic and non-pharmacologic approaches could be applied in order to relieve patients and improve quality of life.: Executive dysfunction is the most notable cognitive impairment and dominates the neuropsychological profile of patients with PSP.

摘要

: 最近,人们提出了用于敏感且特异性临床诊断进行性核上性麻痹(PSP)的新标准,同时该疾病的不同临床表型也在文献中被越来越多地描述。本研究旨在描述该疾病的过去和现在,并强调 PSP 患者的认知和行为特征与潜在病理、遗传学和治疗程序的关系。: 进行了 Medline 和 Scopus 检索,以确定有关该主题的已发表文章。仅发表英文文章的被认为符合标准。: PSP 的最常见临床特征包括早期姿势不稳和跌倒、垂直核上性眼球运动障碍、对左旋多巴反应不佳的帕金森病和假性延髓性麻痹。额功能障碍和言语流畅性缺陷是 PSP 中最明显的认知障碍,而记忆、视空间和社会认知也可能受到影响。淡漠和冲动也存在于 PSP 患者中,并对亲属和护理人员有重大影响。: PSP 是一种具有明显 tau 神经病理学的神经退行性疾病。PSP 患者的运动、动机和沟通障碍可能会限制他们参与日常生活活动。全面的神经心理学评估对 PSP 的认知评估具有重要意义。可以应用药物和非药物方法来缓解患者的症状并提高生活质量。: 执行功能障碍是最显著的认知障碍,主导 PSP 患者的神经心理学特征。

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