Tascilar Nida, Tekin Nilgün Solak, Erdem Zuhal, Alpay Atilla, Emre Ufuk
Department of Neurology, Zonguldak Karaelmas University Medical Faculty, Zonguldak, Turkey.
Auton Neurosci. 2007 Dec 30;137(1-2):1-9. doi: 10.1016/j.autneu.2007.05.004. Epub 2007 Jun 13.
Harlequin sign and harlequin syndrome, which are used interchangeably in the literature, are characterized by sudden onset of hemifacial sweating and flushing, induced by exercise and heat. Hemifacial sweating and flushing with normal ocular sympathetic innervation, known as harlequin syndrome, is rarely associated with tonic pupils, parasympathetic oculomotor lesion and pre- or postganglionic sudomotor sympathetic deficit. In the literature, hemifacial sweating and flushing in patients with apparently abnormal ocular sympathetic innervation has been defined as harlequin sign. To date, a few reports of excessive hemifacial sweating and flushing in structural lesion have been documented. Herein, we report five patients with excessive hemifacial sweating and flushing, two of whom had a syrinx. In presenting the patients, we have attempted to distinguish harlequin syndrome from harlequin sign. With this in mind, Case 1 can be described as harlequin syndrome resembling Ross syndrome, Case 2 as harlequin syndrome with normal ocular sympathetic innervation, Case 3 as harlequin sign with congenital Horner syndrome, Case 4 as harlequin sign with sympathetic and parasympathetic denervation sensitivity, and Case 5 as harlequin syndrome associated with occult sympathetic denervation sensitivity. These cases are discussed together with a review of the literature.
文献中常互换使用的“丑角征”和“丑角综合征”，其特征为运动和受热诱发的半侧面部突然出汗和潮红。半侧面部出汗和潮红伴正常眼部交感神经支配，即所谓的丑角综合征，很少与强直性瞳孔、副交感神经动眼神经病变以及节前或节后汗腺运动性交感神经功能障碍相关。文献中，眼部交感神经支配明显异常的患者出现的半侧面部出汗和潮红被定义为丑角征。迄今为止，已有一些关于结构性病变导致半侧面部出汗和潮红过多的报道。在此，我们报告5例半侧面部出汗和潮红过多的患者，其中2例患有空洞。在介绍这些患者时，我们试图区分丑角综合征和丑角征。基于此，病例1可描述为类似罗斯综合征的丑角综合征，病例2为眼部交感神经支配正常的丑角综合征，病例3为伴有先天性霍纳综合征的丑角征，病例4为伴有交感和副交感神经失神经敏感性的丑角征，病例5为伴有隐匿性交感神经失神经敏感性的丑角综合征。本文结合文献回顾对这些病例进行讨论。
