Autonomic and Neurovascular Medicine Unit, Imperial College London, St Mary's Hospital, 2nd Floor, Queen Elizabeth the Queen Mother Wing, Praed Street, London W2 1NY, UK. c.mathias@ imperial.ac.uk
Nat Rev Neurol. 2011 Dec 6;8(1):22-34. doi: 10.1038/nrneurol.2011.187.
Postural tachycardia syndrome (PoTS) is a poorly understood but important cause of orthostatic intolerance resulting from cardiovascular autonomic dysfunction. PoTS is distinct from the syndromes of autonomic failure usually associated with orthostatic hypotension, such as pure autonomic failure and multiple system atrophy. Individuals affected by PoTS are mainly young (aged between 15 years and 40 years) and predominantly female. The symptoms--palpitations, dizziness and occasionally syncope--mainly occur when the patient is standing upright, and are often relieved by sitting or lying flat. Common stimuli in daily life, such as modest exertion, food ingestion and heat, are now recognized to be capable of exacerbating the symptoms. Onset of the syndrome can be linked to infection, trauma, surgery or stress. PoTS can be associated with various other disorders; in particular, joint hypermobility syndrome (also known as Ehlers-Danlos syndrome hypermobility type, formerly termed Ehlers-Danlos syndrome type III). This Review describes the characteristics and neuroepidemiology of PoTS, and outlines possible pathophysiological mechanisms of this syndrome, as well as current and investigational treatments.
体位性心动过速综合征(PoTS)是一种心血管自主神经功能障碍导致的、尚未完全阐明但很重要的直立不耐受原因。PoTS 与通常与直立性低血压相关的自主神经衰竭综合征不同,如单纯自主神经衰竭和多系统萎缩。受 PoTS 影响的个体主要为年轻人(15 岁至 40 岁之间),且以女性为主。症状——心悸、头晕,偶尔晕厥——主要发生在患者直立时,常通过坐或躺平缓解。日常生活中的常见刺激,如适度运动、进食和热,现在被认为能够加重症状。该综合征的发作可与感染、外伤、手术或压力有关。PoTS 可与各种其他疾病相关;特别是,关节过度活动综合征(也称为埃勒斯-当洛斯综合征过度活动型,以前称为埃勒斯-当洛斯综合征 III 型)。本文描述了 PoTS 的特征和神经流行病学,并概述了该综合征可能的病理生理机制,以及目前和正在研究的治疗方法。