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Site of autonomic deficit in Harlequin syndrome: local autonomic failure affecting the arm and the face
Journal article   Peer reviewed

Site of autonomic deficit in Harlequin syndrome: local autonomic failure affecting the arm and the face

P.D. Drummond and J.W. Lance
Annals of Neurology, Vol.34(6), pp.814-819
1993
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Abstract

The site of autonomic deficit was investigated in 4 patients with loss of thermoregulatory flushing and sweating on one side of the face but no associated miosis (harlequin syndrome). In 2 patients the sudomotor deficit included the hand. Signs of postganglionic sympathetic deficit included pathological lacrimal sweating (2 patients) and supersensitivity to 1% phenylephrine eyedrops (3 patients). One or both pupils constricted excessively to 0.0625% pilocarpine eyedrops (3 patients), consistent with ocular parasympathetic deficit. These findings suggest that pre- or postganglionic cervical sympathetic fibers, and parasympathetic neurons in the ciliary ganglia, are compromised in harlequin syndrome. The finding of cholinergic supersensitivity in the iris muscles of patients with harlequin syndrome indicates a relationship with Holmes-Adie syndrome, Ross's syndrome, and the persistent autonomic deficit occasionally associated with Guillain-Barré syndrome.

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Collaboration types
Domestic collaboration
Citation topics
1 Clinical & Life Sciences
1.105 Strokes
1.105.1677 Cervical Artery Dissection
Web Of Science research areas
Clinical Neurology
Neurosciences
ESI research areas
Neuroscience & Behavior
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