SHAPIROS SYNDROME - AGENESIS OF CORPUS-CA LLOSUM, RECURRENT HYPOTHERMIA, AND HYPERHYDROSIS

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