| Pupillographic findings in 39 consecutive cases of harlequin syndrome. | |
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MedLine Citation:
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PMID: 18769278 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Harlequin syndrome is a curious phenomenon in which one half of the face fails to flush during thermal or emotional stress as a result of damage to vasodilator sympathetic fibers. Anecdotal reports suggest that some of these patients have abnormal pupils. In this study we set out to systematically investigate autonomic pupil disturbances in an unselected cohort of patients with harlequin syndrome. METHODS: A consecutive series of 39 patients with harlequin syndrome who were referred to a tertiary autonomic function laboratory underwent slit-lamp examinations, testing of deep tendon reflexes, infrared video pupillography and, where needed, additional pharmacologic pupillary testing. Results were compared with a meta-analysis of all previously reported cases of harlequin syndrome (n = 39) identified from a literature search. RESULTS: In 65% of patients, no underlying causative medical disturbance could be identified. In 64% of patients, there were abnormal pupils, most commonly Horner syndrome, which was always present ipsilateral to the side of the face with impaired facial sweating and flushing. The lesion was postganglionic in 9 of 10 patients tested pharmacologically. Five (13%) patients had tonic pupils, most of whom also had tendon areflexia but no other neurologic findings, a pattern consistent with Holmes-Adie syndrome. In 2 of these patients, tonic and Horner pupils coexisted. Normal pupils were present in 36% of patients. These results are similar to those for the 39 previously reported patients with harlequin syndrome. CONCLUSIONS: The frequent coexistence of harlequin and Horner syndromes without other neurologic deficits suggests pathologic changes affecting the superior cervical ganglion. Because either syndrome may occur alone, damage is apparently selective. Among the patients with harlequin syndrome who also have tonic pupils and tendon areflexia (Holmes-Adie syndrome), we postulate a ganglionopathy affecting not merely the (sympathetic) superior cervical ganglion, but also the (parasympathetic) ciliary and dorsal root ganglia. Because we found that more than 10% of patients had an undisclosed mass lesion in the chest or neck or a generalized autonomic neuropathy, we recommend a targeted evaluation in selected patients with harlequin syndrome. |
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Authors:
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Fion Bremner; Stephen Smith |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Volume: 28 ISSN: 1536-5166 ISO Abbreviation: J Neuroophthalmol Publication Date: 2008 Sep |
Date Detail:
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Created Date: 2008-09-04 Completed Date: 2009-01-08 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9431308 Medline TA: J Neuroophthalmol Country: United States |
Other Details:
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Languages: eng Pagination: 171-7 Citation Subset: IM |
Affiliation:
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Department of Neuro-Ophthalmology, National Hospital for Neurology and Neurosurgery, London, United Kingdom. fion.bremner@uclh.nhs.uk |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aged Autonomic Nervous System Diseases / complications, diagnosis, etiology, physiopathology* Child Cohort Studies Female Flushing / etiology, physiopathology Ganglia, Parasympathetic / physiopathology Ganglia, Spinal / physiopathology Ganglia, Sympathetic / physiopathology Horner Syndrome / diagnosis, etiology, physiopathology Humans Hypohidrosis / etiology, physiopathology Male Middle Aged Peripheral Nervous System Diseases / complications, physiopathology Pupil Disorders / diagnosis, etiology, physiopathology* Sympathetic Fibers, Postganglionic / physiopathology Syndrome |
| Comments/Corrections | |
Comment In:
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J Neuroophthalmol. 2008 Sep;28(3):169-70
[PMID:
18769277
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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