Quantifying behaviors of children with Sanfilippo syndrome: the Sanfilippo Behavior Rating Scale.

ABSTRACT: The Sanfilippo Behavior Rating Scale (SBRS), a 68 item questionnaire, has been developed to assess the behavioral phenotype of children with Sanfilippo syndrome and its progression over time. Fifteen scales rate orality, movement/activity, attention/self-control, emotional function including anger and fear, and social interaction. Items within scales intercorrelate; measures of internal consistency are adequate. Twelve scales are grouped into 4 abnormality clusters: Movement, Lack of fear, Social/emotional and Executive Dysfunction. A Loess age-trajectory analysis showed that Lack of Fear, Social/Emotional and Executive Dysfunction increased steadily with age; Orality and Mood/Anger/Aggression leveled off. Movement peaked around 6years, then declined as children's excessive/purposeless act

SUBMITTER: Shapiro EG 

PROVIDER: S-EPMC4390542 | biostudies-literature | 2015 Apr

REPOSITORIES: biostudies-literature