J Korean Med Sci.  2005 Apr;20(2):329-330. 10.3346/jkms.2005.20.2.329.

Harlequin Syndrome with Crossed Sympathetic Deficit of the Face and Arm

• Moon SY
• Shin DI
• Park SH
• Kim JS

• KMID: 1095214
• DOI: http://doi.org/10.3346/jkms.2005.20.2.329

Abstract

Harlequin syndrome is characterized by unilateral hyperhidrosis and flushing, which are predominantly induced by heat or exercise. Usually, the sympathetic deficits confine to the face. Rarely, the autonomic deficits involve the arm or the parasympathetic neurons in the ciliary ganglia. We report a 43-yr-old woman who presented with facial flushing and sweating in the right side, which were mainly induced by exercise. The facial flushing accompanied relative coldness in the right arm. Valsalva maneuver, cold pressure and 0.125% pilocarpine test, and computed tomography of the chest were normal. The crossed sympathetic deficit in the left face and right arm suggested that the lesions were multifocal. The sympathetic impairment in our patient may lie on a spectrum of pre- and postganglionic autonomic dysfunction, which was observed in Holmes-Adie, Ross, and Guillain-Barre syndrome.

Keyword

Harlequin Syndrome; Hypohidrosis, Autonomic Neuropathy; Hereditary Sensory and Autonom-ic Neuropathies

MeSH Terms

Adult
Arm/innervation/*physiopathology
Autonomic Nervous System Diseases/*physiopathology
Face/innervation/*physiopathology
Female
Flushing/*physiopathology
Humans
Research Support, Non-U.S. Gov't
Sweating/*physiology
Syndrome

Figure

• 

  Fig. 1 Photograph of the patient, taken after a 30-min-run, shows facial flushing only in the right side. During this episode, the left side of her face remained dry without sweating.

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