レッテト症候群行動質問票の韓国語版の開発と検証

Background/Objectives: The Rett Syndrome Behavior Questionnaire (RSBQ) is a widely used caregiver-reported instrument for assessing behavioral and neurological features of Rett syndrome (RTT). However, a validated Korean version has not been available. This study aimed to translate the RSBQ into Korean (K-RSBQ) and to evaluate its psychometric properties in a Korean RTT population. Methods: The RSBQ was translated and back-translated using standardized procedures and refined through a Delphi process. Primary caregivers of individuals with clinically diagnosed RTT completed an online survey including the K-RSBQ and the Childhood Autism Rating Scale (CARS). Test-retest reliability was assessed in a subset of caregivers who completed the questionnaire twice within one week, and inter-rater reliability was evaluated when an additional caregiver was available. Results: Sixty-six primary caregivers participated. The K-RSBQ demonstrated high internal consistency for the total score (Cronbach's α = 0.912) and moderate-to-high consistency across most subscales. Test-retest reliability for the total score was moderate (weighted κ = 0.594), while inter-rater reliability between primary and secondary caregivers was generally low. The hand behavior subscale showed low and non-significant test-retest reliability. The K-RSBQ total score exhibited a low-to-moderate correlation with the CARS total score, and the general mood subscale showed a moderate correlation with the CARS emotional response item. Caregivers reported minimal difficulty in understanding the questionnaire items. Conclusions: The K-RSBQ demonstrates acceptable internal consistency and test-retest reliability when administered to primary caregivers, with preliminary evidence supporting its construct validity. Although limitations exist regarding criterion validation and inter-rater agreement, the K-RSBQ represents a feasible and culturally adapted tool for assessing RTT-related behavioral features in Korean clinical and research settings.