THE SWORD OF DAMOCLES - THE PSYCHOSOCIAL IMPACT OF FAMILIAL SPINOCEREBELLAR ATAXIA IN SOUTH-AFRICA

A survey was conducted on 30 unaffected individuals from a family with autosomal dominant late onset spinocerebellar ataxia in South Africa, The psychological impact of the disorder on individual lives, risk aw areness, attitudes towards affected kin and reproduction were evaluate d, Respondents employed various psychological strategies to deal with the threat of developing the disorder, In a comparison of ''assigned'' risk with ''perceived'' risk, 80% of unaffected persons reported inco rrect perceptions of personal risk status. The disorder had little imp act on attitudes concerning reproduction; the majority of individuals at risk wanted more children. These issues need to be addressed in the genetic and predictive testing service for familial ataxia in South A frica. Am, J. Med. Genet, 74:270-274, 1997. (C) 1997 Wiley-Liss, Inc.