Abstract
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.
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Acknowledgments
This work was supported by grants from the National Institute of Neurological Disorders and Stroke (NINDS) (National Institutes of Health [NIH] U54NS065705) (to Dr. Comi) and from Hunter’s Dream for a Cure Foundation (to Dr. Comi), Celebrate Cure Foundation (to Dr. Comi), and Faneca 66 Foundation (to Dr. Comi). The Brain Vascular Malformation Consortium (U54NS065705) is a part of the NIH Rare Disease Clinical Research Network, supported through the collaboration between the NIH Office of Rare Diseases Research at the National Center for Advancing Translational Science and the NINDS.
The authors thank Rhoda P. Erhardt, MS, OTR/L, FAOTA for her input on the use of the EDPA and review of the manuscript.
This study was approved by the Johns Hopkins Medicine Institutional Review Board and was therefore performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Parental written informed consent was obtained for each parent–child dyad.
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Teressa Garcia Reidy and Stacy J. Suskauer are co-first authors who contributed equally.
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Reidy, T.G., Suskauer, S.J., Bachur, C.D. et al. Preliminary reliability and validity of a battery for assessing functional skills in children with Sturge-Weber syndrome. Childs Nerv Syst 30, 2027–2036 (2014). https://doi.org/10.1007/s00381-014-2573-6
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DOI: https://doi.org/10.1007/s00381-014-2573-6