Rett Syndrome Behaviour Questionnaire in Children and Adults With Rett Syndrome: Psychometric Characterization and Revised Factor Structure
Author:
Oberman Lindsay M.1, Leonard Helen2, Downs Jenny3, Cianfaglione Rina4, Stahlhut Michelle5, Larsen Jane L.6, Madden Katherine V.7, Kaufmann Walter E.8
Affiliation:
1. Lindsay M. Oberman, Center for Neuroscience and Regenerative Medicine, Henry M. Jackson Foundation for the Advancement of Military Medicine, USA 2. Helen Leonard, Telethon Kids Institute, The University of Western Australia, Perth, Western Australia, Australia 3. Jenny Downs, Telethon Kids Institute, The University of Western Australia and School of Physiotherapy and Exercise Science, Curtin University, Perth, Western Australia, Australia 4. Rina Cianfaglione, University of Southampton 5. Michelle Stahlhut, Center for Rett Syndrome, Rigshospitalet, Copenhagen, Denmark 6. Jane L. Larsen, Center for Rett Syndrome, Rigshospitalet, Copenhagen, Denmark 7. Katherine V. Madden, Anavex Life Sciences Corp., USA 8. Walter E. Kaufmann, Anavex Life Sciences Corp. and Emory University School of Medicine, USA
Abstract
Abstract
Rett syndrome (RTT) is a severe neurodevelopmental disorder associated with multiple neurobehavioral abnormalities. The Rett Syndrome Behaviour Questionnaire (RSBQ) was developed for pediatric RTT observational studies. Because its application has expanded to adult and interventional studies, we evaluated the RSBQ's psychometric properties in six pediatric (n = 323) and five adult (n = 309) datasets. Total and General Mood subscale scores had good reliability. Clinical severity had no influence on RSBQ scores. Exploratory and confirmatory factor analyses yielded 6 pediatric and 7 adult clinically relevant and psychometrically strong factors including the original Breathing Problems and Fear/Anxiety subscales and the novel Emotional and Disruptive Behavior subscale composed of items from the original General Mood and Nighttime Behaviours subscales. The present findings support additional evaluations and improvements of an important RTT behavioral measure.
Publisher
American Association on Intellectual and Developmental Disabilities (AAIDD)
Subject
Psychiatry and Mental health,Neurology (clinical),Arts and Humanities (miscellaneous),Developmental and Educational Psychology,Neuropsychology and Physiological Psychology,General Medicine,Pediatrics, Perinatology and Child Health
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