2022, Number 3
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Folia 2022; 16 (3)
Harlequin syndrome in childhood
Alemán MT, González VE, González MM
Language: Spanish
References: 10
Page:
PDF size: 298.30 Kb.
ABSTRACT
Harlequin syndrome is a rare disorder of the autonomic nervous system, which consists
of the sudden appearance of bouts of flushing and hemifacial diaphoresis that is usually
due to heat, emotion or physical exertion. The absence of unilateral facial redness and
sweating is due to ipsilateral dysfunction of the sympathetic cholinergic vasodilator and sudomotor pathway, at the central hypothalamic or spinal cord level from T1 to T4
(innervation of the face), preganglionic or postganglionic. Compensatory neuronal
hyperactivity on the healthy side is thought to accentuate the cutaneous signs that identify
this syndrome. A case of Harlequin syndrome is reported in a 4-year-old boy, diagnosed
in Ciego de Ávila province. It is an infrequent entity in our setting, either due to
underdiagnosis or the scarcity of cases reported in the scientific literature.
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