Preliminary reliability and validity of a battery for assessing functional skills in children with Sturge-Weber syndrome

Purpose The purpose of this study was to evaluate inter-rater reliability and validity of a proposed functional outcome battery for clinical trials in children with Sturge-Weber syndrome (SWS). Methods Ten children were evaluated twice on the same day using a series of functional outcome measures selected for sensitivity to the range of age and function of children with SWS: Modified Rankin Scale, Pediatric Evaluation of Disability Index, Modified House Functional Classification, and a modified version of the Erhardt Developmental Prehension Assessment. Inter-rater reliability was calculated, and criterion validity was explored through correlations with the Sturge-Weber Syndrome-Neurological Rating Score (SWS-NRS). Results Inter-rater reliability was high across all measures. Correlations were identified between the SWS-NRS and the study measures. Conclusions The proposed battery of functional outcome measures captures child’s functioning at the levels of impairment, activity and participation and is robust to evaluation by different raters and across sessions on the same day. This battery is expected to be sensitive to treatment-related changes in qualitative patterns of hand use, functional skills, and/or change in independence in daily living.