Harlequin syndrome
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• What is Harlequin syndrome?
  
• What symptoms are associated with Harlequin syndrome?
• What causes Harlequin syndrome?
• Is there treatment for Harlequin syndrome?

• What is Harlequin syndrome?
   (Back to Top)

• Harlequin syndrome was first coined by Lance and Drummond in 1988 when they described five cases of unilateral (on one side) flushing and sweating.^[1] The asymmetrical facial sweating and flushing described with this condition has been named the 'Harlequin Sign'.
  
• Last Reviewed: 9/4/2008

• What symptoms are associated with Harlequin syndrome? (Back to Top)

• The Harlequin sign is sometimes associated with warmth and anhidrosis (lack of sweating) of the arm and leg on the opposite side. This may be induced by exercise.  Attacks of cluster headache may be accompanied by tearing and nasal discharge, forehead sweating, and abnormal contraction of the pupils and drooping of the upper eyelid. The cause of these phenomena has no widely accepted explanation.^[2]
  
• Last Reviewed: 9/4/2008

• What causes Harlequin syndrome? (Back to Top)

• The suggested etiology (cause) of Harlequin syndrome is a lesion involving both preganglionic or postganglionic cervical sympathetic fibers and parasympathetic neurons (a bundle of nerve fibers that runs from the base of the skull to the the tailbone) of the ciliary ganglion.^[2]
  
• Last Reviewed: 9/4/2008

• Is there treatment for Harlequin syndrome? (Back to Top)

• In individuals who have the Harlequin sign with no known explanation, no treatment is usually required, and the condition is benign.^[3]
  
• Last Reviewed: 9/4/2008

References  (Back)

1. Corbett M, Abernethy DA. Harlequin syndrome. J Neurol Neurosurg Psychiatry. 1999;:. Available at: http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1736279&blobtype=pdf. September 4, 2008.
2. Nasr C. . Flushing. Cleveland Clinic Website. 2004 Available at: http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/endocrinology/flushing/flushing.htm. Accessed September 4, 2008.
3. Fallon KE, May JJ.. Harlequin syndrome in two athletes. Br J Sports Med. 2005;:. Available at: http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1725016&blobtype=pdf. September 4, 2008.