Harlequin syndrome

WikiDoc Resources for Harlequin syndrome
Articles
Most recent articles on Harlequin syndrome Most cited articles on Harlequin syndrome Review articles on Harlequin syndrome Articles on Harlequin syndrome in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Harlequin syndrome Images of Harlequin syndrome Photos of Harlequin syndrome Podcasts & MP3s on Harlequin syndrome Videos on Harlequin syndrome
Evidence Based Medicine
Cochrane Collaboration on Harlequin syndrome Bandolier on Harlequin syndrome TRIP on Harlequin syndrome
Clinical Trials
Ongoing Trials on Harlequin syndrome at Clinical Trials.gov Trial results on Harlequin syndrome Clinical Trials on Harlequin syndrome at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Harlequin syndrome NICE Guidance on Harlequin syndrome NHS PRODIGY Guidance FDA on Harlequin syndrome CDC on Harlequin syndrome
Books
Books on Harlequin syndrome
News
Harlequin syndrome in the news Be alerted to news on Harlequin syndrome News trends on Harlequin syndrome
Commentary
Blogs on Harlequin syndrome
Definitions
Definitions of Harlequin syndrome
Patient Resources / Community
Patient resources on Harlequin syndrome Discussion groups on Harlequin syndrome Patient Handouts on Harlequin syndrome Directions to Hospitals Treating Harlequin syndrome Risk calculators and risk factors for Harlequin syndrome
Healthcare Provider Resources
Symptoms of Harlequin syndrome Causes & Risk Factors for Harlequin syndrome Diagnostic studies for Harlequin syndrome Treatment of Harlequin syndrome
Continuing Medical Education (CME)
CME Programs on Harlequin syndrome
International
Harlequin syndrome en Espanol Harlequin syndrome en Francais
Business
Harlequin syndrome in the Marketplace Patents on Harlequin syndrome
Experimental / Informatics
List of terms related to Harlequin syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Harlequin syndrome is a condition characterized by asymmetric sweating and flushing on the upper thoracic region of the chest, the neck, and the face. It is caused by sustaining an injury to the sympathetic nervous system, the part of the nervous system that reacts to stress and flight-or-fight circumstances. Listed as a rare disease, Harlequin syndrome affects fewer than 200,000 people in the United States.

It can also be the outcome of a unilateral endoscopic thoracic sympathectomy (ETS) or endoscopic sympathetic blockade (ESB) surgery, see endoscopic thoracic sympathectomy.

Historical Perspective

The name for the syndrome is credited to Lance and Drummond who were inspired by resemblance patient’s half-flushed faces bore to colorful Harlequin masks.

Causes

One possible cause of Harlequin syndrome is a lesion to the preganglionic or postganglionic cervical sympathetic fibers and parasympathetic neurons of the ciliary ganglion. It is also believed that torsion of the thoracic spine can cause blockage of the anterior radicular artery leading to harlequin syndrome. The sympathetic deficit on the denervated side causes the flushing of the opposite side to appear more pronounced. It is unclear whether or not the response of the undamaged side was normal or excessive, but it is believed it could be a result of the body attempting to compensate for the damaged side and maintain homeostasis.

Symptoms

The ‘Harlequin Sign’ is unilateral flushing and sweating of the face and neck usually after exposure to heat or strenuous exertion. Horner’s syndrome, another problem associated with the sympathetic nervous system, is often seen in conjunction with harlequin syndrome.

Treatment

Harlequin syndrome is not fatal so treatment is not normally necessary.

References

Horner's Syndrome