GENETIC AND BEHAVIORAL ASPECTS OF PRADER-WILLI-SYNDROME - A REVIEW WITH A TRANSLATION OF THE ORIGINAL PAPER

An English translation of the 1956 paper in which Prader, Labhart and Willi originally described the syndrome which now bears their name is followed by a discussion of the advances in understanding regarding ge netic and behavioural aspects which have occurred during the interveni ng years. Prader-Willi syndrome is associated with an absence of a pat ernal contribution to a defined area of the long arm of chromosome 15, and with maladaptive behaviours which are more common than among peop le of the same age and sex, who have equivalent cognitive impairments. The syndrome is associated with an excessive appetite which may lead to the consumption of unusual items (such as frozen or discarded food) and to extreme obesity. The association between genetic and behaviour al abnormalities offers an opportunity to explore the mediating effect of environmental variables on specific maladaptive behaviours.