Shapiro syndrome with hypothalamic hypothyroidism.

Dr. Ronaldo Arkader – Department of Pediatric Intensive Care, Hospital Sabará Rua Dona Antonia de Queiros 505 01307-010 São Paulo SP Brasil. E-mail: [email protected] Shapiro syndrome, first described in 1969, is an extremely rare condition consisting of the clinical triad of recurrent hypothermia, sweating, and chills in the presence of agenesis of the corpus callosum. The periodicity of the hypothermic episodes ranges from hours to years, and episodes may last hours to weeks. Typically, but not invariably, there are associated central nervous system abnormalities affecting structures, particularly in the hypothalamus1. Central forms of hypothyroidism include secondary hypothyroidism, which is caused by TSH deficiency, and tertiary hypothyroidism, which is caused by TRH deficiency2. We describe a previously healthy boy with Shapiro syndrome and hypothalamic hypothyroidism treated with thyroxine and cyproheptadine.