recurrent spontaneous hypothermia with hyperhidrosis syndrome
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 .
Episodic spontaneous hypothermia with hyperhidrosis syndrome is a very rare condition and has typically been described in the pediatric population .
The presentation is one of periodic hypothermia and hyperhidrosis, without warning and often lasting hours before spontaneous resolution back to baseline .
The pathogenesis has not been fully elucidated, however, it is thought that there may be a dysfunction of central serotonergic pathways .
Neuroimaging is typically normal .
Treatment and prognosis
Management is guided by case report-level evidence, with one notably effective agent being cyproheptadine .