Hypothalamic obesity is usually induced by tumoral or genetic alterations such as craniopharyngioma or Prader-Willi syndrome, respectively. However, few case s have been reported without recognized etiology, this syndrome is also called idiopathic hypothalamic syndrome. Objectives. -To improve definition a nd frequency of complications associated with this syndrome. Population and Meth ods. -A retrospective cohort study was performed in French endocrine paediatric departments and was associated with a literature review. Results. -We report f ive cases of idiopathic hypothalamic syndrome. This syndrome is correlated with a high mortality (one of our five cases, 25%in the literature) by neurovegetati ve dysfunction (breathing or thermal alteration). Obesity began before six years old because of compulsive eating and resulted in social behaviour disorders. Ab normal endocrine secretions were characterized by early hyperprolactinemia, perm anent but later somatotrope deficiency and 80%of thyreotrope deficiency. Pubert y abnormalities included hypogonadotropic hypogonadism as well as precocious (on e of our cases, three cases including literature) or normal puberty. Neurogenic hypernatremia and water and electrolytic disorders were also responsible of acut e neurological alterations. Conclusion. -This largest study ever reported of id iopathic hypothalamic syndrome emphasizes the need of amultidisciplinary coordin ation to provide the best care of these patients.
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