Behavioural Issues

Comparisons between children with Williams Syndrome and control group children matched for age, sex, social class, and verbal intelligence provide support for a cognitive and behavioural phenotype in Williams Syndrome. Children with the syndrome showed higher rates of behavioural and emotional difficulties when compared with the control group children, particularly in terms of concentration difficulties, excessive anxiety, and poor relationships with peers; and they also had significantly poorer visuo-spatial and motor skills. However, the Williams Syndrome children were not uniformly poor in all areas of nonverbal abilities. Their visual recall skills were as good as those of the control group children, and their performance was superior to that of the control group children on a task of face recognition and on tasks requiring recall of verbal material.

 

Journal of Clinical and Experimental Neuropsychology Volume 13, Issue 2, 1991

Certain personality characteristics are especially common in children with Williams Syndrome. These characteristics include: an outgoing social nature, an exuberant enthusiasm, a sense of the dramatic, overfriendliness, a short attention span, extra sensitivity to sounds (hyperacusis), and anxiety – especially about upcoming events.

Children with Williams Syndrome are often particularly appealing. Many of the associated characteristics are rather desirable (bright eyes, very broad engaging smile, enthusiastic manner, socially engaging and conversational, strong sensitivity to the emotions of others, cute upturned nose, excellent memory for people met infrequently or long ago, very expressive of own emotions – especially happy excitement). It is important to keep in mind that these are indeed “real” characteristics of the child, and not just ‘syndromal’. That is, it is important to capitalize on, and enjoy the very real charismatic appeal of many children with Williams syndrome, and not dismiss these behaviors as simply, “Williams-isms.”

Some behavioral characteristics associated with Williams Syndrome can pose challenges in classrooms and workplaces. There are effective strategies for minimizing the difficulties and helping the child cope. These characteristics and strategies are outlined below.

Short attention span and distractibility

Attention difficulties often lead to associated difficulties such as impulsivity which can result in the child not following directions well, getting out of their seat, etc.

  • flexibility in requirements for time spent working
  • frequent ‘breaks’ in work time
  • a “high success,” high motivation curriculum
  • minimal distractions; auditory as well as visual
  • rewards for attending behaviors and, when possible, redirection around ‘off task’ behaviors or ignoring same
  • allowing some degree of choice for the child in terms of activity
  • small groups
  • consultation with a behaviorist familiar with positive behavior management approaches
  • difficulty modulating emotions
  • extreme excitement when happy
  • tearfulness in response to apparently mild distress
  • terror in response to apparently mildly frightening events
  • Decide when this is a problem. For example, expressing enthusiastic excitement, albeit impulsively or without raising a hand, may be beneficial to the motivation of the class as a whole, whereas frequent tears and a high degree of anxiety is problematic for the child with Williams syndrome as well as the other children.
  • Help the child to develop increasingly effective internal controls to modulate emotions while adapting the environment to minimize situations of extreme anxiety and frustration.
  • Anticipate beginning buildup of frustration. Help the child to remove himself from the frustrating situation and find a different activity before the frustration escalates
  • Minimize unexpected changes in schedule, plans, etc.
  • Use stories and role play/pretend play to act out various anxiety provoking situations with the child
  • Heightened sensitivity to sounds (hyperacusis)