J Korean Med Sci 2005; 20: 329-30 ISSN 1011-8934 Copyright The Korean Academy of Medical Sciences Harlequin Syndrome with Crossed Sympathetic Deficit of the Face and Arm 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 parasym- pathetic neurons in the ciliary ganglia. We report a 43-yr-old woman who present- ed 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 auto- nomic dysfunction, which was observed in Holmes-Adie, Ross, and Guillain-Barre′ syndrome. Key Words : Harlequin Syndrome; Hypohidrosis, Autonomic Neuropathy; Hereditary Sensory and Autonom- ic Neuropathies So Young Moon, Dong-In Shin, Seong-Ho Park, Ji Soo Kim Department of Neurology, College of Medicine, Seoul National University, Seoul National University Bundang Hospital, Seongnam, Korea Received : 11 December 2003 Accepted : 20 April 2004 Address for correspondence Ji Soo Kim, M.D. Department of Neurology, College of Medicine, Seoul National University, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam 463-707, Korea Tel : +82.31-787-7463, Fax : +82.31-719-6828 E-mail : jisookim@snu.ac.kr *This study was supported by a grant (R05-2001-000- 00616-0) from the Korea Science & Engineering Foun- dation. INTRODUCTION Harlequin syndrome is characterized by unilateral facial flushing and sweating, which are predominantly induced by heat or exercise (1). Harlequin syndrome is mostly idiopath- ic (1), but may occur in association with brainstem infarc- tion, superior mediastinal neurinoma, internal jugular vein catheterization (1-3). The sympathetic deficit is in the non- flushing side and the healthy side shows normal or excessive flushing and sweating. Usually the sympathetic deficit is con- fined to the face. Rarely, the autonomic deficit involves the arm or the parasympathetic neurons in the ciliary ganglia (4, 5). We report a patient with unilateral facial flushing and sweating, and ipsilateral arm coldness, which were mainly induced by exertion. CASE REPORT A 43-yr-old woman presented with a 3-yr-history of facial flushing and sweating in the right side, which were mainly induced by exercise. She had also noticed an increased cold- ness of the right arm during such episodes. The symptoms were more prominent in the winter, but did not aggravate during the preceding three years. Her family and medical his- tory were unremarkable. Findings of the physical and neuro- 329 logical examinations at rest were normal. However, after a 30-min-running, she showed flushing and sweating only on the right side of the face (Fig. 1). During this episode, the left side of her face remained dry with impaired sweating. Pupils were equal. Blood pressure, pulse rate, and skin color did not differ among the four extremities during the episode. Using a digital skin thermometer, skin temperature measured 2℃ colder on the right arm than on the left arm, and 2.9℃ cold- er on the left face than on the right face. Response to the Val- salva maneuver and sympathetic skin response were normal. The immersion of one hand in ice water for 1 min (cold pres- sure test) revealed a normal response in both arms. The pupils did not constrict with 0.125% pilocarpine eyedrops. Com- puted tomography of the chest was normal. DISCUSSION The name harlequin syndrome was first coined by Lance and Drummond in 1988 when they described five cases of unilateral facial flushing and sweating, which were induced by exercise in four (1). They proposed that the torsional occlu- sion of the anterior radicular artery at the third thoracic seg- ment caused the syndrome, and that the lesion might involve both pre- or postganglionic cervical sympathetic fibers and parasympathetic neurons of the ciliary ganglion.