Department of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, 955 Main Street, Buffalo, NY, 14203, USA.
J Neurol. 2022 Feb;269(2):725-732. doi: 10.1007/s00415-021-10502-z. Epub 2021 Mar 7.
Postural orthostatic tachycardia syndrome (POTS), a disorder of the autonomic nervous system characterized by a rise in heart rate of at least 30 bpm from supine to standing position, has been traditionally viewed as a dysfunction of the peripheral nervous system. However, recent studies and evidence from overlapping conditions suggest that in addition to being considered a disorder of the peripheral nervous system, POTS should be viewed also as a central nervous system (CNS) disorder given (1) significant CNS symptom burden in patients with POTS; (2) structural and functional differences found on neuroimaging in patients with POTS and other forms of orthostatic intolerance; (3) evidence of cerebral hypoperfusion and possible alteration in cerebrospinal fluid volume, and (4) positive response to medications targeting the CNS and non-pharmacologic CNS therapies. This review outlines existing evidence of POTS as a CNS disorder and proposes a hypothetical model combining key mechanisms in the pathophysiology of POTS. Redefining POTS as a CNS disorder can lead to new possibilities in pharmacotherapy and non-pharmacologic therapeutic interventions in patents affected by this disabling syndrome.
体位性心动过速综合征（POTS）是一种自主神经系统紊乱，其特征是从仰卧位到站立位时心率至少上升 30 次/分钟。传统上，它被认为是外周神经系统的功能障碍。然而，最近的研究和重叠病症的证据表明，除了被认为是外周神经系统紊乱之外，POTS 还应被视为中枢神经系统（CNS）紊乱，原因如下：（1）POTS 患者存在显著的 CNS 症状负担；（2）在患有 POTS 和其他形式的直立不耐受的患者中发现的神经影像学结构和功能差异；（3）脑灌注不足的证据和脑脊液体积可能改变；（4）针对 CNS 的药物治疗和非药物性 CNS 治疗有积极反应。本综述概述了 POTS 作为 CNS 紊乱的现有证据，并提出了一个假设模型，该模型结合了 POTS 病理生理学中的关键机制。将 POTS 重新定义为 CNS 紊乱，可以为受这种致残性综合征影响的患者的药物治疗和非药物治疗干预提供新的可能性。
