The patient, a 41-year-old woman, presented to the rheumatology clinic. She reported that, for the past 3 years, she experienced unilateral facial flushing and coldness of the ipsilateral hand after vigorous exercise. Results of the neurologic assessment, including tests for autonomic function, were normal. Findings of magnetic resonance imaging of the patient's brain, neck, thoracic outlet, and chest were also normal. The patient mentioned that her son experienced similar symptoms, although he had not been formally assessed. A diagnosis of Harlequin syndrome was made in light of the patient's characteristic symptoms, which included unilateral flushing and hyperhidrosis after exercise (Wasner G, Maag R, Ludwig J, Binder A, Schattschneider J, Stingele R, et al. Harlequin syndrome-one face of many etiologies. Nat Clin Pract Neurol 2005;l:54-9). Harlequin syndrome results from disruption of sympathetic fibers at or distal to the sympathetic ganglia at T2-T3, with proximally located lesions associated with greater autonomic dysfunction. To date, 91 patients with Harlequin syndrome have been reported, and in 64% of these no cause was identified. In the remaining patients, mediastinal tumors, paravertebral thoracic blocks, and neck-mass resection accounted for the majority of the cases.
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