Lenka Abhishek, Beach Paul
Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA.
Mov Disord Clin Pract. 2025 Aug 7. doi: 10.1002/mdc3.70268.
Autonomic dysfunction is a common feature of synucleinopathies such as Parkinson's disease (PD), dementia with Lewy bodies (DLB), multiple system atrophy (MSA), and pure autonomic failure (PAF). Orthostatic hypotension (OH) and supine hypertension (SH) are hallmark manifestations of cardiovascular autonomic dysfunction that are increasingly recognized as contributors to long-term adverse clinical outcomes such as accelerated cognitive decline, motor symptom exacerbation, and end-organ damage. Several atypical presentations of OH, including coat-hanger pain, cognitive fluctuation, postural instability, fatigue, exertional dyspnea, and falls, commonly overlap with motor and non-motor features of synucleinopathies. This clinical overlap highlights the need for heightened awareness of the diverse and often subtle manifestations of OH in affected individuals. Although the general management of OH has been addressed in prior literature, a dedicated framework tailored to the pathophysiological and clinical nuances specific to synucleinopathies remains lacking. SH, despite its high prevalence and significant prognostic implications, has received minimal attention in existing reviews and is often overlooked in clinical practice.
Given the growing recognition of cardiovascular autonomic dysfunction as a potentially modifiable contributor to disease progression and patient quality of life, a focused updated review is warranted to inform clinical decision-making and guide future research in this evolving area.
This narrative review comprehensively discusses the approach to OH and SH in the context of synucleinopathies.
The key topics addressed are the pathophysiology of OH and SH in synucleinopathies, diagnostic criteria and types of OH, diagnostic criteria of SH, and management strategies (non-pharmacological, pharmacological, and novel/experimental).
自主神经功能障碍是帕金森病（PD）、路易体痴呆（DLB）、多系统萎缩（MSA）和单纯自主神经功能衰竭（PAF）等突触核蛋白病的常见特征。直立性低血压（OH）和卧位高血压（SH）是心血管自主神经功能障碍的标志性表现，越来越被认为是导致认知加速衰退、运动症状加重和终末器官损害等长期不良临床结局的因素。OH的几种非典型表现，包括衣架样疼痛、认知波动、姿势不稳、疲劳、劳力性呼吸困难和跌倒，通常与突触核蛋白病的运动和非运动特征重叠。这种临床重叠凸显了提高对受影响个体中OH多样且往往细微表现的认识的必要性。尽管先前的文献已经讨论了OH 的一般管理，但仍缺乏一个专门针对突触核蛋白病的病理生理和临床细微差别的框架。SH尽管患病率高且具有重要的预后意义，但在现有综述中受到的关注极少，在临床实践中也常常被忽视。
鉴于心血管自主神经功能障碍作为疾病进展和患者生活质量的潜在可调节因素日益受到认可，有必要进行重点更新综述，为临床决策提供信息，并指导这一不断发展领域的未来研究。
本叙述性综述全面讨论了突触核蛋白病背景下OH和SH的处理方法。
所涉及的关键主题是突触核蛋白病中OH和SH的病理生理学、OH的诊断标准和类型、SH的诊断标准以及管理策略（非药物、药物和新型/实验性）。
