Harlequin syndrome--one face of many etiologies.
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.


METHODS
Neurological examination, autonomic testing, duplex ultrasonography, scintigraphy and MRI.


METHODS
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.


RESULTS
Explanation of pathophysiology and benign nature of the condition.
Wasner
Gunnar
G
Prince of Wales Medical Research Institute, University of New South Wales, Randwick, Sydney, NSW, Australia. g.wasner@neurologie.uni-kiel.de
Maag
Rainer
R
Ludwig
Janne
J
Binder
Andreas
A
Schattschneider
Jörn
J
Stingele
Robert
R
Baron
Ralf
R
D001342:Autonomic Nervous System Diseases / Q000209:etiology*; D005260:Female; D005483:Flushing; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009408:Nerve Compression Syndromes / Q000150:complications; D009460:Neurologic Examination; D011877:Radionuclide Imaging; D017783:Superior Cervical Ganglion / Q000473:pathology*; D013546:Sweating / Q000502:physiology*; D013577:Syndrome; D018616:Ultrasonography, Doppler, Duplex