Assessment of psychosocial adjustment and reduced initiative in children with myotonic dystrophy type 1: a pilot study on the reliability and clinical utility of a short parent-report questionnaire

We describe the reliability and clinical utility of the personal adjustment and role skills scale III (PARS-III) for screening of psychosocial adjustment in children with myotonic dystrophy type 1 (DM1). Data of 36 pediatric DM1 patients were included from the Dutch MYODRAFT patient registry. Reliability was assessed using Cronbach's alpha. Associations between PARS-III scores and estimates of brain-related comorbidity and parent-reported physical disease burden were explored as possible factors affecting psychosocial adjustment. PARS-III data of children with DM1 in this study were compared to PARS-III data from children with Duchenne muscular dystrophy in order to describe specificity to the pediatric DM1 population. The PARS-III showed adequate internal consistency. PARS-III total scores correlated with parent-reported symptoms of autism spectrum disorder and attention deficit/hyperactivity disorder and parent-reported physical disease burden. No statistically significant associations were found with intelligence. Parents reported most problems in reduced social activity and reduced initiative. Reduced initiative was not associated with attention deficit/hyperactivity disorder, autism spectrum disorder or reported physical disease burden. Parents of children with DM1 reported more problems in initiative compared to parents of children with Duchenne muscular dystrophy. We conclude that the PARS-III is a reliable instrument for screening psychosocial adjustment in general and deficits in initiative in particular. More research is needed on this clinically relevant symptom as a possible brain-related comorbidity of children with DM1.