ELECTRONIC LETTER Death in adults with Prader-Willi syndrome may be correlated with maternal uniparental disomy

Prader-Willi syndrome (PWS) is a disorder comprisingsevere neonatal hypotonia, hypogonadism, gross obesity,short stature, small hands and feet, mental handicap, a characteristic facial appearance (almond shaped eyes, thin downturned upper lip, and a narrow bitemporal diameter), nasal, inarticulate speech, and a particular personality profile.1 2 Prader-Willi syndrome has a biphasic course. Initially there is severe neonatal hypotonia, difficulty in feed-ing, and failure to thrive, usually persisting for 12 months. This is followed by weight gain, and if unchecked obesity is well developed by 6 years of age.1 2 The gain in weight is primarily attributable to a hypothalamic defect resulting in an insatiable appetite and hyperphagia,3 4 but a lowered meta-bolic rate and lack of exercise owing to continuing hypotonia contribute.5 The patients have a food obsession with a rather specific food related behavioural phenotype, which includes foraging for food, stealing food, and eating inedibles.1