Specific Mental Retardation Disorders and Problem Behaviours

Abstract

Problem behaviours occur frequently among mentally retarded persons in general. In particular mental retardation disorders, such behaviours may be relatively non-specific or may include features which are highly characteristic of the disorder concerned. These considerations are exemplified in this paper with reference to five disorders (Bourneville disease, phenylketonuria, Lesch-Nyhan syndrome, Rett syndrome and Prader-Willi syndrome), each of which has an established or probable genetic basis. Although psychosocial influences certainly contribute to the overall behavioural outcome, underlying biological aberrations in such disorders as those considered are crucial factors in the genesis of clinical manifestations which occur. Increasing scope for elucidating the nature of these aberrations and how they produce their adverse effects have enhanced the prospects for more basic therapeutic and primary preventive strategies than have heretofore been available.