Idiopathic hypothalamic syndrome:: retrospective study and literature review

Hypothalamic obesity is usually induced by tumoral or genetic alterations such as craniopharyngioma or Prader-Willi syndrome, respectively. However, few cases have been reported without recognized etiology, this syndrome is also called idiopathic hypothalamic syndrome. Objectives. - To improve definition and frequency of complications associated with this syndrome. Population and Methods. - A retrospective cohort study was performed in French endocrine paediatric departments and was associated with a literature review. Results. - We report five cases of idiopathic hypothalamic syndrome. This syndrome is correlated with a high mortality (one of our five cases, 25% in the literature) by neurovegetative dysfunction (breathing or thermal alteration). Obesity began before six years old because of compulsive eating and resulted in social behaviour disorders. Abnormal endocrine secretions were characterized by early hyperprolactinemia, permanent but later somatotrope deficiency and 80% of thyreotrope deficiency. Puberty abnormalities included hypogonadotropic hypogonadism as well as precocious (one of our cases, three cases including literature) or normal puberty. Neurogenic hypernatremia and water and electrolytic disorders were also responsible of acute neurological alterations. Conclusion. - This largest study ever reported of idiopathic hypothalamic syndrome emphasizes the need of a multidisciplinary coordination to provide the best care of these patients. (c) 2005 Publie par Elsevier SAS.