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 .

Epidemiology

Episodic spontaneous hypothermia with hyperhidrosis syndrome is a very rare condition and has typically been described in the pediatric population .

Clinical presentation

The presentation is one of periodic hypothermia and hyperhidrosis, without warning and often lasting hours before spontaneous resolution back to baseline .

Pathology​

The pathogenesis has not been fully elucidated, however, it is thought that there may be a dysfunction of central serotonergic pathways .

Radiographic features

Neuroimaging is typically normal .

Treatment and prognosis

Management is guided by case report-level evidence, with one notably effective agent being cyproheptadine .

Differential diagnosis

• Shapiro syndrome

Siehe auch:

• Dysgenesie des Corpus callosum