Jain Puneet, Arya Ravindra, Jaryal Ashok, Gulati Sheffali
Department of Pediatrics, Division of Pediatric Neurology, All India Institute of Medical Sciences, New Delhi, India.
J Child Neurol. 2013 Apr;28(4):527-30. doi: 10.1177/0883073812446484. Epub 2012 May 25.
Harlequin syndrome, Harlequin sign, Holmes-Adie syndrome, and Ross syndrome lie on a spectrum of partial dysautonomias affecting facial sudomotor, vasomotor, and pupillary responses. These syndromes have imprecise clinical boundaries and overlap syndromes are known. We report a 9-year-old girl who presented with anhidrosis over the right half of her face and the left side of her body, with compensatory hyperhidrosis on the contralateral side. She was noted to have bilateral tonic pupils and normal muscle stretch reflexes with other features suggestive of autonomic dysfunction. Investigations to rule out secondary causes were noncontributory. Her clinical presentation can be categorized as partial overlap between Harlequin syndrome and Holmes-Adie syndrome.
丑角综合征、丑角征、霍姆斯-阿迪综合征和罗斯综合征属于一组影响面部汗腺运动、血管运动和瞳孔反应的部分自主神经功能障碍。这些综合征的临床界限不明确，已知存在重叠综合征。我们报告一名9岁女孩，其右侧面部和左侧身体无汗，对侧代偿性多汗。她被发现双侧瞳孔强直，肌肉牵张反射正常，还有其他提示自主神经功能障碍的特征。排除继发原因的检查无异常发现。她的临床表现可归类为丑角综合征和霍姆斯-阿迪综合征之间的部分重叠。
