Harlequin syndrome--one face of many etiologies

Nat Clin Pract Neurol. 2005 Nov;1(1):54-9. doi: 10.1038/ncpneuro0040.

Abstract

Background: A 55-year-old woman presented to hospital with a 3-month history of asymmetric facial flushing of the skin during exertion, and an 18-month history of left-sided ptosis and miosis. Detailed medical history analysis revealed that a palpable node measuring 0.8 x 1.2 x 1.2 cm (volume 1.1 ml) had been discovered 2 years previously, within the left lobe of an otherwise uncomplicated goiter that had been successfully managed for 20 years. Otherwise, the patient was healthy.

Investigations: Neurological examination, autonomic testing, duplex ultrasonography, scintigraphy and MRI.

Diagnosis: Harlequin syndrome following a lesion of the preganglionic sympathetic efferents, caused by neurovascular compression of the sympathetic chain between the stellate and superior cervical ganglion brought about by an elongated inferior thyroid artery.

Management: Explanation of pathophysiology and benign nature of the condition.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autonomic Nervous System Diseases / etiology*
  • Female
  • Flushing*
  • Humans
  • Magnetic Resonance Imaging
  • Middle Aged
  • Nerve Compression Syndromes / complications
  • Neurologic Examination
  • Radionuclide Imaging
  • Superior Cervical Ganglion / pathology*
  • Sweating / physiology*
  • Syndrome
  • Ultrasonography, Doppler, Duplex