Tomc S A, Williamson N K, Pauli R M
Department of Medical Genetics, University of Wisconsin, Madison.
Am J Med Genet. 1990 Jul;36(3):345-52. doi: 10.1002/ajmg.1320360321.
Anecdotal reports suggest that children with Williams syndrome are loquacious, affectionate, charming, open, and gentle. The temperament, or behavioral style, of individuals with Williams syndrome was assessed using standard temperament scales of parental response. Children with Williams syndrome appear, on average, to display higher activity, lower rhythmicity, greater approach, lower adaptability, greater intensity, more negative mood, less persistence, greater distractibility, and lower threshold to arousal than will average children. Older children with Williams syndrome will likely be viewed as "difficult." Despite this overrepresentation in the "difficult" cluster, on average, this population differs from the usual population of "difficult" children in being approaching, rather than displaying a tendency to withdraw. Parents of a child with Williams syndrome should be apprised of potentially negative temperament characteristics, relieved of the burden of responsibility for having a difficult child who "should" be easy, and counselled, when appropriate, regarding the efficacy of modification of parent-child interactions with children having the difficult temperament.
