Combination of Nutritional Treatment and Troglitazone Has Beneficial Effects on Glycemic Control in a Diabetic Patient with Prader-Willi Syndrome

Abstract

Prader-Willi syndrome (PWS) is a disorder characterized by obesity, hypogonadism, hypotonia, and mental retardation. Hyperphagia associated with PWS is severe and chronic, and is responsible for obesity and type-2 diabetes mellitus (DM). We report a case of PWS with DM and agenesis of the corpus callosum. On admission to our hospital, the female patient with PWS was 20 years old and her body mass index was 50.8 kg/m². She had overt DM (HbA₁c 11.6%) with hyperinsulinemia. Magnetic resonance neuroimaging (brain MRI) showed agenesis of the corpus callosum. This case is the first report of PWS with agenesis of the corpus callosum. Nutritional treatment was transiently effective during her hospital stay, but after discharge, she gained weight and her glycemic control got worse (HbA₁c 11.1%). Troglitazone was administered because she was insulinresistant, as judged by homeostasis model assessment (HOMA 10.13). Troglitazone had beneficial effects on glycemic control by ameliorating impaired insulin sensitivity (HOMA 4.88), but it was very important to control body weight in order to realize this improvement.