Moon So Young, Shin Dong-In, Park Seong-Ho, Kim Ji Soo
Department of Neurology, College of Medicine, Seoul National University, Seoul National University Bundang Hospital, Seongnam 463-707, Korea.
J Korean Med Sci. 2005 Apr;20(2):329-30. doi: 10.3346/jkms.2005.20.2.329.
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 syndromé.
小丑综合征的特征是单侧多汗和潮红，主要由热或运动诱发。通常，交感神经功能缺陷局限于面部。很少有自主神经功能缺陷累及手臂或睫状神经节中的副交感神经元。我们报告一名43岁女性，她表现为右侧面部潮红和出汗，主要由运动诱发。面部潮红伴有右臂相对发冷。瓦尔萨尔瓦动作、冷压和0.125%毛果芸香碱试验以及胸部计算机断层扫描均正常。左侧面部和右臂的交叉性交感神经缺陷表明病变是多灶性的。我们患者的交感神经损伤可能处于节前和节后自主神经功能障碍的范围内，这在霍姆斯-阿迪综合征、罗斯综合征和吉兰-巴雷综合征中可见。
