Document Detail

Harlequin syndrome: the sudden onset of unilateral flushing and sweating.
MedLine Citation:
PMID:  3155385     Owner:  NLM     Status:  MEDLINE    
Facial flushing and sweating were investigated in five patients who complained of the sudden onset of unilateral facial flushing in hot weather or when exercising vigorously. One patient probably suffered a brainstem infarct at the time that the unilateral flush was first noticed, and was left with a subtle Horner's syndrome on the side opposite to the flush. The other four had no other neurological symptoms and no ocular signs of Horner's syndrome. Thermal and emotional flushing and sweating were found to be impaired on the non-flushing side of the forehead in all five patients whereas gustatory sweating and flushing were increased on that side in four of the five patients, a combination of signs indicating a deficit of the second sympathetic neuron at the level of the third thoracic segment. CT and MRI of this area failed to disclose a structural lesion but latency from stimulation of the motor cortex and thoracic spinal cord to the third intercostal muscle was delayed on the non-flushing side in one patient. The complaint of unilateral flushing and sweating was abolished in one patient by ipsilateral stellate ganglionectomy. The unilateral facial flushing and sweating induced by heat in all five patients was thus a normal or excessive response by an intact sympathetic pathway, the other side failing to respond because of a sympathetic deficit. The onset in the four cases of peripheral origin followed strenuous exertion, which suggested that an anterior radicular artery may have become occluded at the third thoracic segment during torsion of the thoracic spine.
J W Lance; P D Drummond; S C Gandevia; J G Morris
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of neurology, neurosurgery, and psychiatry     Volume:  51     ISSN:  0022-3050     ISO Abbreviation:  J. Neurol. Neurosurg. Psychiatr.     Publication Date:  1988 May 
Date Detail:
Created Date:  1988-09-15     Completed Date:  1988-09-15     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  2985191R     Medline TA:  J Neurol Neurosurg Psychiatry     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  635-42     Citation Subset:  IM    
Department of Neurology, Prince Henry Hospital, Sydney, Australia.
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MeSH Terms
Autonomic Nervous System Diseases / physiopathology*
Brain / physiopathology
Dominance, Cerebral / physiology*
Flushing / physiopathology*
Ganglia, Sympathetic / physiopathology
Hot Temperature / adverse effects
Middle Aged
Motor Cortex / physiopathology
Muscles / innervation
Physical Exertion
Reflex, Pupillary
Spinal Cord / physiopathology
Comment In:
J Neurol Neurosurg Psychiatry. 1991 Aug;54(8):744   [PMID:  1809267 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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