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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| Format: | Article |
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MDPI AG
2022-07-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/58/7/938 |
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| author | Ioannis Mavroudis Ioana-Miruna Balmus Alin Ciobica Alina-Costina Luca Rumana Chowdhury Alin-Constantin Iordache Dragos Lucian Gorgan Iulian Radu |
| author_facet | Ioannis Mavroudis Ioana-Miruna Balmus Alin Ciobica Alina-Costina Luca Rumana Chowdhury Alin-Constantin Iordache Dragos Lucian Gorgan Iulian Radu |
| author_sort | Ioannis Mavroudis |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-09T06:14:42Z |
| format | Article |
| id | doaj.art-6a9e17f727614658b7895cdbdabed572 |
| institution | Directory Open Access Journal |
| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-09T06:14:42Z |
| publishDate | 2022-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-6a9e17f727614658b7895cdbdabed5722023-12-03T11:54:54ZengMDPI AGMedicina1010-660X1648-91442022-07-0158793810.3390/medicina58070938Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring AttentionIoannis Mavroudis0Ioana-Miruna Balmus1Alin Ciobica2Alina-Costina Luca3Rumana Chowdhury4Alin-Constantin Iordache5Dragos Lucian Gorgan6Iulian Radu7Department of Neuroscience, Leeds Teaching Hospitals, NHS Trust, Leeds LS2 9JT, UKDepartment of Exact Sciences and Natural Sciences, Institute of Interdisciplinary Research, “Alexandru Ioan Cuza” University of Iasi, Alexandru Lapusneanu Street, No. 26, 700057 Iasi, RomaniaDepartment of Biology, Faculty of Biology, “Alexandru Ioan Cuza” University of Iasi, 700506 Iasi, RomaniaFaculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 16 Universitatii Street, 700115 Iasi, RomaniaDepartment of Neuroscience, Leeds Teaching Hospitals, NHS Trust, Leeds LS2 9JT, UKFaculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 16 Universitatii Street, 700115 Iasi, RomaniaDepartment of Biology, Faculty of Biology, “Alexandru Ioan Cuza” University of Iasi, 700506 Iasi, RomaniaFaculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 16 Universitatii Street, 700115 Iasi, RomaniaHarlequin 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.https://www.mdpi.com/1648-9144/58/7/938Harlequin syndromeautonomic nervous system impairmentunilateralface flushingsweatinganhidrosis |
| spellingShingle | Ioannis Mavroudis Ioana-Miruna Balmus Alin Ciobica Alina-Costina Luca Rumana Chowdhury Alin-Constantin Iordache Dragos Lucian Gorgan Iulian Radu Mini-Review on the Harlequin Syndrome—A Rare Dysautonomic Manifestation Requiring Attention Medicina Harlequin syndrome autonomic nervous system impairment unilateral face flushing sweating anhidrosis |
| 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 | Harlequin syndrome autonomic nervous system impairment unilateral face flushing sweating anhidrosis |
| url | https://www.mdpi.com/1648-9144/58/7/938 |
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