Department of Neuroscience, Leeds Teaching Hospitals, NHS Trust, Leeds LS2 9JT, UK.
Department of Exact Sciences and Natural Sciences, Institute of Interdisciplinary Research, "Alexandru Ioan Cuza" University of Iasi, Alexandru Lapusneanu Street, No. 26, 700057 Iasi, Romania.
Medicina (Kaunas). 2022 Jul 15;58(7):938. doi: 10.3390/medicina58070938.
Harlequin syndrome (HS) is a rare autonomic disorder. The causes and risk factors of the disease are not fully understood. Some cases of HS are associated with traumatic injuries, tumors, or vascular impairments of the head. Symptoms of HS can also occur in some autoimmune disorders, ophthalmic disorders, sleep disorders, and with certain organic lesions. In this context, a thorough review of the pathophysiology of HS in relation to neurological, ophthalmological, and dermatological conditions is necessary. In this mini-review, we aim to review the pathophysiological changes and underlying mechanisms in primary and secondary HS. Additionally, we discuss possible management approaches for patients with HS in light of the discussed pathological mechanisms. The main symptoms of HS that are correlated with autonomic nervous system impairments include sudden unilateral flushing of the face, neck, chest, and rarely arm, with concurrent contralateral anhidrosis. Despite reported co-occurring syndromes (such as cluster headaches), several studies have shown that HS could frequently overlap with other syndromes that are disruptive to the idiopathic nerve pathways. HS usually does not require any medical treatment. In some severe cases, symptomatic treatments could be needed. However, total symptomatic relief may not be achieved in many cases of HS. We therefore suggest an approach to comprehensive management of HS, which may lead to better long-term control of HS.
亨廷顿舞蹈症（HS）是一种罕见的自主神经障碍。其病因和危险因素尚未完全阐明。某些 HS 病例与头部的创伤性损伤、肿瘤或血管损伤有关。HS 的症状也可能发生在一些自身免疫性疾病、眼部疾病、睡眠障碍和某些器质性病变中。在这种情况下，有必要深入研究 HS 与神经、眼科和皮肤病相关的病理生理学。在本篇小型综述中，我们旨在综述原发性和继发性 HS 的病理生理变化及其潜在机制。此外，我们根据所讨论的病理机制讨论了 HS 患者的可能治疗方法。HS 与自主神经系统损伤相关的主要症状包括突然单侧面部、颈部、胸部，偶尔手臂潮红，同时对侧无汗。尽管有报道称存在共发综合征（如丛集性头痛），但几项研究表明，HS 可能经常与其他破坏特发性神经通路的综合征重叠。HS 通常不需要任何治疗。在一些严重的情况下，可能需要对症治疗。然而，HS 的许多病例可能无法完全缓解症状。因此，我们建议采用综合管理 HS 的方法，这可能有助于更好地长期控制 HS。
