Drummond P D, Lance J W
Psychology Section, Murdoch University, Western Australia.
Ann Neurol. 1993 Dec;34(6):814-9. doi: 10.1002/ana.410340610.
The site of autonomic deficit was investigated in 4 patients with loss of thermoregulatory flushing and sweating on one side of the face but no associated miosis (harlequin syndrome). In 2 patients the sudomotor deficit included the hand. Signs of postganglionic sympathetic deficit included pathological lacrimal sweating (2 patients) and supersensitivity to 1% phenylephrine eyedrops (3 patients). One or both pupils constricted excessively to 0.0625% pilocarpine eyedrops (3 patients), consistent with ocular parasympathetic deficit. These findings suggest that pre- or postganglionic cervical sympathetic fibers, and parasympathetic neurons in the ciliary ganglia, are compromised in harlequin syndrome. The finding of cholinergic supersensitivity in the iris muscles of patients with harlequin syndrome indicates a relationship with Holmes-Adie syndrome, Ross's syndrome, and the persistent autonomic deficit occasionally associated with Guillain-Barré syndrome.
对4例面部一侧失去体温调节性潮红和出汗但无相关瞳孔缩小（丑角综合征）的患者进行了自主神经功能缺损部位的研究。在2例患者中，汗腺运动功能缺损包括手部。节后交感神经功能缺损的体征包括病理性泪汗分泌（2例患者）和对1%去氧肾上腺素滴眼液超敏（3例患者）。一只或两只瞳孔对0.0625%毛果芸香碱滴眼液过度收缩（3例患者），符合眼部副交感神经功能缺损。这些发现表明，丑角综合征患者的节前或节后颈交感神经纤维以及睫状神经节中的副交感神经元受到损害。丑角综合征患者虹膜肌肉中胆碱能超敏的发现表明其与霍姆斯-阿迪综合征、罗斯综合征以及偶尔与吉兰-巴雷综合征相关的持续性自主神经功能缺损有关。
