{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,10]],"date-time":"2026-07-10T23:10:32Z","timestamp":1783725032175,"version":"3.55.0"},"reference-count":39,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2022,7,15]],"date-time":"2022-07-15T00:00:00Z","timestamp":1657843200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/medicina58070938","type":"journal-article","created":{"date-parts":[[2022,7,17]],"date-time":"2022-07-17T22:57:06Z","timestamp":1658098626000},"page":"938","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Mini-Review on the Harlequin Syndrome\u2014A Rare Dysautonomic Manifestation Requiring Attention"],"prefix":"10.3390","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9844-4010","authenticated-orcid":false,"given":"Ioannis","family":"Mavroudis","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Leeds Teaching Hospitals, NHS Trust, Leeds LS2 9JT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ioana-Miruna","family":"Balmus","sequence":"additional","affiliation":[{"name":"Department of Exact Sciences and Natural Sciences, Institute of Interdisciplinary Research, \u201cAlexandru Ioan Cuza\u201d University of Iasi, Alexandru Lapusneanu Street, No. 26, 700057 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alin","family":"Ciobica","sequence":"additional","affiliation":[{"name":"Department of Biology, Faculty of Biology, \u201cAlexandru Ioan Cuza\u201d University of Iasi, 700506 Iasi, Romania"},{"name":"Academy of Romanian Scientists, Splaiul Independentei Nr. 54, Sector 5, 050094 Bucuresti, Romania"},{"name":"Center of Biomedical Research, Romanian Academy, B dul Carol I, No 8, 700505 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5676-0864","authenticated-orcid":false,"given":"Alina-Costina","family":"Luca","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 16 Universitatii Street, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rumana","family":"Chowdhury","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Leeds Teaching Hospitals, NHS Trust, Leeds LS2 9JT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alin-Constantin","family":"Iordache","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 16 Universitatii Street, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6454-9092","authenticated-orcid":false,"given":"Dragos Lucian","family":"Gorgan","sequence":"additional","affiliation":[{"name":"Department of Biology, Faculty of Biology, \u201cAlexandru Ioan Cuza\u201d University of Iasi, 700506 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iulian","family":"Radu","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 16 Universitatii Street, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"5342593","DOI":"10.1155\/2017\/5342593","article-title":"Idiopathic Harlequin Syndrome Manifesting during Exercise: A Case Report and Review of the Literature","volume":"2017","author":"Algahtani","year":"2017","journal-title":"Case Rep. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1136\/jnnp.51.5.635","article-title":"Harlequin syndrome: The sudden onset of unilateral flushing and sweating","volume":"51","author":"Lance","year":"1988","journal-title":"J. Neurol. Neurosurg. 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