Mourad Firas, Scotto Irene, Dunning James, Giudice Andrea, Maritati Giorgio, Maselli Filippo, Kranenburg Rik, Taylor Alan, Kerry Roger, Hutting Nathan
Department of Health, LUNEX, Differdange, Luxembourg.
Luxembourg Health & Sport Sciences Research Institute Asbl, Differdange, Luxembourg.
J Man Manip Ther. 2024 Dec;32(6):646-653. doi: 10.1080/10669817.2024.2349338. Epub 2024 May 17.
Harlequin syndrome is a rare autonomic condition consisting of unilateral facial flushing and sweating induced by heat, emotion or physical activity. The affected side presents anhidrosis and midline facial pallor due to denervation of the sympathetic fibers.
This case describes a patient who reported right-side redness of the face associated with hyperhidrosis during physical activity. She had two previous major motor vehicle accidents. The patient demonstrated difficulties in the visual accommodation of the left eye, but cranial nerve assessment was unremarkable; the patient was then referred to an ophthalmologist, who excluded any autonomic dysfunction as the primary cause of convergence and visual acuity.
A left-sided sympathetic dysfunction with Harlequin sign diagnosis was made followed by a progressive compensatory adaptation of the right face. The patient was educated and reassured about the benign nature of her problem.
Knowledge of the autonomic nervous system is still limited in clinical practice. Although challenging, physiotherapists should develop the knowledge and ability needed to perform appropriate assessment of autonomic dysfunctions.
A dispositional reasoning model should be considered in differential diagnosis.
丑角综合征是一种罕见的自主神经疾病,表现为受热、情绪或体力活动诱发的单侧面部潮红和出汗。由于交感神经纤维失神经支配,患侧出现无汗和面部中线苍白。
本病例描述了一名患者,其报告在体力活动期间右侧面部发红并伴有多汗。她曾发生过两次严重的机动车事故。患者左眼视觉调节存在困难,但颅神经评估无异常;随后该患者被转诊至眼科医生处,眼科医生排除了任何自主神经功能障碍作为集合和视力的主要原因。
诊断为伴有丑角征的左侧交感神经功能障碍,随后右侧面部逐渐出现代偿性适应。对患者进行了教育,并使其对自身问题的良性性质放心。
在临床实践中,对自主神经系统的了解仍然有限。尽管具有挑战性,但物理治疗师应培养进行自主神经功能障碍适当评估所需的知识和能力。
在鉴别诊断中应考虑倾向性推理模型。