Episodic spontaneous hypothermia with hyperhidrosis syndrome (ESHH) is a rare syndrome characterized by periodic hypothermia and hyperhidrosis. This occurs in the absence of agenesis of the corpus callosum, thus differentiating it from Shapiro syndrome 1.
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Epidemiology
Episodic spontaneous hypothermia with hyperhidrosis syndrome is a very rare condition and has typically been described in the pediatric population 1-3.
Clinical presentation
The presentation is one of periodic hypothermia and hyperhidrosis, without warning and often lasting hours before spontaneous resolution back to baseline 1-3.
Pathology
The pathogenesis has not been fully elucidated, however, it is thought that there may be a dysfunction of central serotonergic pathways 1-3.
Radiographic features
Neuroimaging is typically normal 1-3.
Treatment and prognosis
Management is guided by case report-level evidence, with one notably effective agent being cyproheptadine 1-3.
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