Shapiro's syndrome: Agenesis of corpus callosum, recurrent hypothermia, and hyperhydrosis

A case of spontaneously recurrent hypothermia, excessive hyperhidrosis and agenesis of the corpus callosum is reported, Despite multiple trials with various pharmacologic agents no sustained remission of symptoms could be achieved. Though this rare thermoregulatory disorder has been atrributed to hypothalamic defects or diencephalic epilepsy the exact underlying pathophysiologic mechanisms still remain to be defined. We studied the sequence of symptoms during thermoisolation; hypothermia did not occur despite excessive hyperhidrosis.