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Patient with Cluster Headache and Harlequin Sign – Related or Not?

BACKGROUND: The harlequin sign or syndrome is a rare cranial autonomic condition characterized by unilateral diminished flushing and sweating of the face (and sometimes arm), in response to heat or exercise. It results from autonomic, mainly sympathetic dysfunction. Although the idiopathic form is t...

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Detalles Bibliográficos
Autor principal: Van Oosterhout, W.P.J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7496594/
https://www.ncbi.nlm.nih.gov/pubmed/32654114
http://dx.doi.org/10.1111/head.13894
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author Van Oosterhout, W.P.J.
author_facet Van Oosterhout, W.P.J.
author_sort Van Oosterhout, W.P.J.
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description BACKGROUND: The harlequin sign or syndrome is a rare cranial autonomic condition characterized by unilateral diminished flushing and sweating of the face (and sometimes arm), in response to heat or exercise. It results from autonomic, mainly sympathetic dysfunction. Although the idiopathic form is the most common, underlying structural abnormalities in the head, neck, and thorax need to be excluded. METHODS AND RESULTS: Here, we describe the first case of the combination of primary cluster headache and the harlequin syndrome in a 49‐year‐old female patient. The patient presented with a first bout of cluster headache with persisting Horner syndrome and new harlequin sign after the bout. Additional neuroimaging did not reveal underlying pathologies. This report provides an overview of cases from the literature and then discusses the association between primary headaches and the harlequin syndrome. Relevant anatomy, diagnostic tools, and therapeutic options are all considered. CONCLUSION: This case reports a rare combination of cluster headache and harlequin sign that warrants further testing to exclude pathology in the brain, neck or mediastinum. It also illustrates the underlying anatomy and physiology of the autonomic nervous system.
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spelling pubmed-74965942020-09-25 Patient with Cluster Headache and Harlequin Sign – Related or Not? Van Oosterhout, W.P.J. Headache Brief Communications BACKGROUND: The harlequin sign or syndrome is a rare cranial autonomic condition characterized by unilateral diminished flushing and sweating of the face (and sometimes arm), in response to heat or exercise. It results from autonomic, mainly sympathetic dysfunction. Although the idiopathic form is the most common, underlying structural abnormalities in the head, neck, and thorax need to be excluded. METHODS AND RESULTS: Here, we describe the first case of the combination of primary cluster headache and the harlequin syndrome in a 49‐year‐old female patient. The patient presented with a first bout of cluster headache with persisting Horner syndrome and new harlequin sign after the bout. Additional neuroimaging did not reveal underlying pathologies. This report provides an overview of cases from the literature and then discusses the association between primary headaches and the harlequin syndrome. Relevant anatomy, diagnostic tools, and therapeutic options are all considered. CONCLUSION: This case reports a rare combination of cluster headache and harlequin sign that warrants further testing to exclude pathology in the brain, neck or mediastinum. It also illustrates the underlying anatomy and physiology of the autonomic nervous system. John Wiley and Sons Inc. 2020-07-11 2020-09 /pmc/articles/PMC7496594/ /pubmed/32654114 http://dx.doi.org/10.1111/head.13894 Text en © 2020 The Authors. Headache: The Journal of Head and Face Pain published by Wiley Periodicals LLC on behalf of American Headache Society This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Brief Communications
Van Oosterhout, W.P.J.
Patient with Cluster Headache and Harlequin Sign – Related or Not?
title Patient with Cluster Headache and Harlequin Sign – Related or Not?
title_full Patient with Cluster Headache and Harlequin Sign – Related or Not?
title_fullStr Patient with Cluster Headache and Harlequin Sign – Related or Not?
title_full_unstemmed Patient with Cluster Headache and Harlequin Sign – Related or Not?
title_short Patient with Cluster Headache and Harlequin Sign – Related or Not?
title_sort patient with cluster headache and harlequin sign – related or not?
topic Brief Communications
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7496594/
https://www.ncbi.nlm.nih.gov/pubmed/32654114
http://dx.doi.org/10.1111/head.13894
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