Harlequin syndrome - one face of many etiologies
Gunnar Wasner*, Rainer Maag, Janne Ludwig, Andreas Binder, Jörn Schattschneider, Robert Stingele and Ralf Baron
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 
1.2 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.
1.2 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.
Nature Clinical Practice Neurology (2005) 1, 54-59
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