Montigiani A, Cencetti S, Bandinelli G, Lagi A
Ospedale S. Maria Nuova di Firenze.
Ann Ital Med Int. 1998 Jul-Sep;13(3):173-5.
Asymmetrical facial sweating and flushing has been named the "Harlequin Sign". This is a rare feature, as evident from only 12 cases described up to date. The "Harlequin Sign" represents a local autonomic dysfunction due to a cervical sympathetic deficit located at the pre or postganglionic level on the non-flushing side. We observed slow onset and progression of the "Harlequin Sign" in a 19-year-old man, with preexisting slight miosis on the non-flushing side. The differential diagnosis included other forms of dysautonomia and a secondary origin of this partial Horner's syndrome. Both pupils normally reacted to light, convergence, and pilocarpine eye-drop instillation, but the affected side showed supersensitivity to phenylephrine. Deep tendon reflexes were normal, thus excluding Adie's syndrome. The absence of cholinergic supersensitivity in the iris muscles indicated normal function of the ciliary ganglion and excluded the ocular parasympathetic deficit, also evident for the coexisting Horner's syndrome. The clinical features could be explained by assuming that the lesion was located at the level of postganglionic sympathetic fibers, probably due to trans-synaptic postganglionic neuronal degeneration at the level of the stellate ganglion, thus determining the onset of the hemifacial symptoms.
面部不对称出汗和潮红被称为“丑角征”。这是一种罕见的体征，目前仅有12例相关病例报道。“丑角征”代表一种局部自主神经功能障碍，是由于非潮红侧颈交感神经节前或节后水平的缺陷所致。我们观察到一名19岁男性“丑角征”起病及进展缓慢，非潮红侧先前存在轻度瞳孔缩小。鉴别诊断包括其他形式的自主神经功能障碍以及这种部分性霍纳综合征的继发性病因。双侧瞳孔对光、集合及毛果芸香碱滴眼液滴眼均正常反应，但患侧对去氧肾上腺素表现出超敏反应。深腱反射正常，排除了艾迪综合征。虹膜肌肉无胆碱能超敏反应表明睫状神经节功能正常，排除了眼副交感神经缺陷，这在并存的霍纳综合征中也很明显。假设病变位于节后交感神经纤维水平，可能是由于星状神经节水平的跨突触节后神经元变性，从而导致半侧面部症状的出现，这样就能解释临床特征了。
