| Site of autonomic deficit in harlequin syndrome: local autonomic failure affecting the arm and the face. | |
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MedLine Citation:
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PMID: 8250530 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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P D Drummond; J W Lance |
Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Annals of neurology Volume: 34 ISSN: 0364-5134 ISO Abbreviation: Ann. Neurol. Publication Date: 1993 Dec |
Date Detail:
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Created Date: 1994-01-04 Completed Date: 1994-01-04 Revised Date: 2008-11-21 |
Medline Journal Info:
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Nlm Unique ID: 7707449 Medline TA: Ann Neurol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 814-9 Citation Subset: IM |
Affiliation:
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Psychology Section, Murdoch University, Western Australia. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Arm / physiopathology* Autonomic Nervous System Diseases / complications*, pathology, physiopathology Body Temperature Regulation Brain / pathology Face / physiopathology* Female Flushing / etiology, physiopathology* Galvanic Skin Response Humans Magnetic Resonance Imaging Male Ocular Physiological Phenomena Pupil / physiology Spinal Cord / pathology Sweating* Taste / physiology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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