Hypothalamic disease and dysfunction is known to be associated with obesity but can also be associated with wasting syndromes. The relative prevalence of these is unknown as are the etiologic determinants for whether a hypothalamic disease causes one or the other.
A systematic review of the electronic health records of a large multispecialty health care system was done to evaluate cases of hypothalamic dysfunction syndrome (HDS) over a 10 yr period (Jan 2009-Jan 2019) and to detail the identified etiologies of the hypothalamic disease, the weight trajectory over the period of observation and prevalence of wasting syndromes vs obesity in the identified cohort.
131 adult patients with HDS were identified over the 10 yr period.Prader Will syndrome was the most common etiology (58; 44%) followed by craniopharyngioma(55; 41%). Both of these conditions had high prevalence of obesity and morbid obesity (50-79%) with a gradual tendency to progressive weight gain over the observation period. Of the 18 subjects who did not have either of these conditions 9 (50%) had BMI (>25) with tendency to gradual weight loss over the observation period consistent with a wasting syndrome. Only 2 (11%) of these patients were obese.
The HDS is an uncommon consequence of hypothalamic disease with myriad causes. Craniopharyngioma and Prada Willi syndrome are the most common causes of hypothalamic obesity and are often associated with morbid obesity. Most patients with HDS without either of these etiologies in contrast are associated with a wasting syndrome. Better understanding of the disparate causes of obesity versus wasting syndrome in HDS is needed and may inform future therapeutic strategies for management of both hypothalamic and non hypothalamic obesity and wasting syndromes.