Markiewicz Michał, Madetko-Alster Natalia, Alster Piotr
Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
Front Neurol. 2024 Nov 20;15:1476488. doi: 10.3389/fneur.2024.1476488. eCollection 2024.
Progressive supranuclear palsy (PSP) is an atypical form of parkinsonism characterized by tauopathy, manifesting as oculomotor dysfunction, postural instability, akinesia, and cognitive/language impairments. The diagnosis and examination of PSP can be challenging, primarily due to the unclear and underexplored pathomechanisms involved, alongside absence of effective treatments. Clinical variants of PSP is the second most common form of neurodegenerative parkinsonism after Parkinson's disease (PD). It is defined by a symmetrical akinetic-rigid syndrome (atypical parkinsonism) and vertical supranuclear gaze palsy. In contrast to PD, PSP often presents with gait instability, backward falls, and cognitive and behavioral changes at early disease stages. The classification of PSP has evolved since Richardson, Steele, and Olszewski's initial reporting of the condition in 1963, which included a cohort of nine patients. Over the years, the definition of this disorder has evolved to encapsulate a group of patients with distinct clinical variants, notably the classical Richardson syndrome (RS) and several atypical phenotypes, each with significant implications for disease progression and quality of life (QoL). The 2017 Movement Disorder Society Diagnostic Criteria by Hoglinger et al., improved the sensitivity for detecting early and variant PSP presentations and provided more specific differential diagnoses for conditions such as PD and other forms of atypical parkinsonian syndromes. Owing to the growing interest in the disease's progression, evaluating the QoL for patients with PSP has become crucial. This review emphasizes the significance of QoL evaluation and its feasibility for practical implications, serving as an initial foundation for future research focused on the well-being of individuals affected by PSP. Progressive supranuclear palsy (PSP) is an atypical form of parkinsonism characterized by tauopathy, manifesting as oculomotor dysfunction, postural instability, akinesia, and cognitive/language impairments. Diagnosing PSP is challenging owing to the lack of tools for differential examination. Additionally, the pathomechanism of this disease is not sufficiently understood, and no treatment is currently available. Owing to the growing interest in the disease's progression, evaluating the quality of life (QoL) for patients with PSP has become crucial. This review emphasizes the significance of QoL evaluation and its feasibility for practical implications, serving as an initial foundation for future research focused on the well-being of individuals affected by PSP.
进行性核上性麻痹(PSP)是一种非典型帕金森病,其特征为tau蛋白病,表现为动眼功能障碍、姿势不稳、运动不能以及认知/语言障碍。PSP的诊断和检查具有挑战性,主要原因是涉及的发病机制尚不明确且研究不足,同时也缺乏有效的治疗方法。PSP的临床变异型是仅次于帕金森病(PD)的第二常见的神经退行性帕金森病形式。它由对称性运动不能-强直综合征(非典型帕金森病)和垂直性核上性凝视麻痹所定义。与PD不同,PSP在疾病早期常表现为步态不稳、向后跌倒以及认知和行为改变。自1963年理查森、斯蒂尔和奥尔谢夫斯基首次报告该病以来,PSP的分类不断发展,当时报告的病例组有9名患者。多年来,这种疾病的定义不断演变,涵盖了一组具有不同临床变异型的患者,特别是经典的理查森综合征(RS)和几种非典型表型,每种表型对疾病进展和生活质量(QoL)都有重要影响。霍格林格等人制定的2017年运动障碍协会诊断标准提高了检测早期和变异型PSP表现的敏感性,并为PD和其他形式的非典型帕金森综合征等疾病提供了更具体的鉴别诊断。由于对该疾病进展的兴趣日益增加,评估PSP患者的生活质量变得至关重要。本综述强调了生活质量评估的重要性及其实际应用的可行性,为未来关注受PSP影响个体福祉的研究奠定了初步基础。进行性核上性麻痹(PSP)是一种非典型帕金森病,其特征为tau蛋白病,表现为动眼功能障碍、姿势不稳、运动不能以及认知/语言障碍。由于缺乏鉴别检查工具,PSP的诊断具有挑战性。此外,这种疾病的发病机制尚未得到充分了解,目前也没有治疗方法。由于对该疾病进展的兴趣日益增加,评估PSP患者的生活质量变得至关重要。本综述强调了生活质量评估的重要性及其实际应用的可行性,为未来关注受PSP影响个体福祉的研究奠定了初步基础。