The spontaneous periodic hypothermia syndrome is usually a benign disease. It is characterized by paroxysmal episodes associating profuse sweating and hypothermia of variable duration and frequency, occurring in the absence of any detectable infectious or endocrine cause. The handicap is above all functional when the episodes recur daily. Biological and morphological examinations are normal, outside the framework of Shapiro's syndrome, which, by definition, associates the spontaneous periodic hypothermia syndrome and agenesis of the corpus callosum. This disease generally has a spontaneously favorable outcome in several weeks or months. The pathophysiological hypotheses, not confirmed to date, point to a paroxysmal dysregulation of the thermoregulator centers, particularly diencephalic. The different therapeutic approaches attempted have been unsuccessful or of doubtful efficacy because of the small number of patients. Less than 30 cases have been reported in the literature. With the goal of improving our understanding of this disease, it seemed important to us to collect data on all the known patients to attempt to discern similarities or certain characteristics which might help reveal a pathophysiological mechanism and, as a consequence, a therapeutic strategy.
Last update: June 2003