ABSTRACT
Obesity is a feature of several congenital syndromes associated with a learning disability including Prader-Willi syndrome (PWS), Bardet-Biedl syndrome, Cohen syndrome, Albright hereditary osteodystrophy, Borjeson-Forssman-Lehmann syndrome, and some rarer disorders (Introduction) (1). Of these, PWS is the most common form of syndromal obesity, with an estimated incidence of 1:25,000 (2) and prevalence of 1:52,000 (3,4). In this chapter we describe the clinical phenotype and the genetic basis and review underlying mechanisms and behavior patterns that influence the hyperphagia and obesity found in PWS.
