Shapiro syndrome

Disease Overview

Shapiro syndrome is a rare neurological disease characterized by recurrent episodes of excessive sweating and hypothermia along with agenesis of the corpus callosum.[1375][11653][11654] The duration and frequency of the episodes vary from person to person, with some episodes lasting hours to weeks and occurring from hours to years; the reason for the variations in the episodes is not yet known. The cause of the condition is currently unknown; however, suggested possible mechanisms include hypothalamic dysfunction, neurochemical abnormalities, inflammatory processes, and epileptic activity.[1375] Treatment aims to address the symptoms present in each individual and may include re-warming with a warm blanket and the use of medications with varying success.[1375][11654]

Synonyms

• Shapiro's syndrome
• Recurrent spontaneous hypothermia with hypoplasia of the corpus callosum
• Spontaneous periodic hypothermia syndrome
• Spontaneous recurrent hypothermia syndrome
• Spontaneous periodic hypothermia