Recurrent Hypothermia and Autonomic Dysfunction Secondary to Shapiro Syndrome.

A 44-year-old man presented with recurrent hypothermia, diaphoresis and hypertension. Extensive investigation for infectious, inflammatory, metabolic and endocrine aetiologies was negative. MR scan of the brain demonstrated no lesions but revealed callosal dysgenesis, consistent with Shapiro syndrome. We describe the pathophysiologic basis of central thermoregulation and an approach to the assessment and management of patients presenting with disorders thereof, including the importance of considering central thermoregulatory failure after systemic disease has been carefully excluded.