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Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention

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 diso...

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Autores principales: Mavroudis, Ioannis, Balmus, Ioana-Miruna, Ciobica, Alin, Luca, Alina-Costina, Chowdhury, Rumana, Iordache, Alin-Constantin, Gorgan, Dragos Lucian, Radu, Iulian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9324885/
https://www.ncbi.nlm.nih.gov/pubmed/35888657
http://dx.doi.org/10.3390/medicina58070938
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author Mavroudis, Ioannis
Balmus, Ioana-Miruna
Ciobica, Alin
Luca, Alina-Costina
Chowdhury, Rumana
Iordache, Alin-Constantin
Gorgan, Dragos Lucian
Radu, Iulian
author_facet Mavroudis, Ioannis
Balmus, Ioana-Miruna
Ciobica, Alin
Luca, Alina-Costina
Chowdhury, Rumana
Iordache, Alin-Constantin
Gorgan, Dragos Lucian
Radu, Iulian
author_sort Mavroudis, Ioannis
collection PubMed
description 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.
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spelling pubmed-93248852022-07-27 Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention Mavroudis, Ioannis Balmus, Ioana-Miruna Ciobica, Alin Luca, Alina-Costina Chowdhury, Rumana Iordache, Alin-Constantin Gorgan, Dragos Lucian Radu, Iulian Medicina (Kaunas) Review 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. MDPI 2022-07-15 /pmc/articles/PMC9324885/ /pubmed/35888657 http://dx.doi.org/10.3390/medicina58070938 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Mavroudis, Ioannis
Balmus, Ioana-Miruna
Ciobica, Alin
Luca, Alina-Costina
Chowdhury, Rumana
Iordache, Alin-Constantin
Gorgan, Dragos Lucian
Radu, Iulian
Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention
title Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention
title_full Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention
title_fullStr Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention
title_full_unstemmed Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention
title_short Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention
title_sort mini-review on the harlequin syndrome—a rare dysautonomic manifestation requiring attention
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9324885/
https://www.ncbi.nlm.nih.gov/pubmed/35888657
http://dx.doi.org/10.3390/medicina58070938
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