Executive Functioning in the Dystrophinopathies and the Relation to Underlying Mutation Position
| العنوان: | Executive Functioning in the Dystrophinopathies and the Relation to Underlying Mutation Position |
|---|---|
| المؤلفون: | Jacqueline Montes, Veronica J. Hinton, Robert J. Fee |
| المصدر: | Journal of the International Neuropsychological Society. 25:146-155 |
| بيانات النشر: | Cambridge University Press (CUP), 2018. |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | Male, 050103 clinical psychology, Adolescent, Intelligence, NIH Toolbox, Muscular Dystrophies, Dystrophin, Executive Function, 03 medical and health sciences, 0302 clinical medicine, Borderline intellectual functioning, Memory span, Humans, Cognitive Dysfunction, 0501 psychology and cognitive sciences, Effects of sleep deprivation on cognitive performance, Child, Working memory, General Neuroscience, 05 social sciences, Cognitive flexibility, Executive functions, Psychiatry and Mental health, Clinical Psychology, Behavior Rating Inventory of Executive Function, Memory, Short-Term, Child, Preschool, Neurology (clinical), Psychology, Psychomotor Performance, 030217 neurology & neurosurgery, Clinical psychology |
| الوصف: | Objectives: The aim of this study was to investigate executive skills in children with dystrophinopathy and to examine the association between executive functions and dystrophin gene mutation position. Methods: Fifty boys with dystrophinopathy (mean age, 11 years 0 months; ages range, 5 to 17 years) completed measures of intellectual functioning (IF), working memory and executive functioning [including Digit Span (working memory) and measures from the NIH Toolbox (selective attention/inhibitory control, set shifting, working memory, and processing speed)]. Parents completed the Behavior Rating Inventory of Executive Function (BRIEF). Mutation positions were categorized into three groups (upstream exon 30, 31–62, and downstream exon 63). Paired-samples t tests compared performance on executive measures to IF, and a one-way (three-group) multivariate analysis of covariance compared cognitive performance with mutation location controlling for motor functioning. Results: Mean performance on all executive measures was significantly lower than IF. Parents were also more likely to rate their child with dystrophinopathy as having clinically significant executive difficulties on the Shift, Emotional Control, and Behavior Regulation indices of the BRIEF. Mutation analyses resulted in small groups limiting power to detect subtle differences. Those with a downstream mutation position had significantly poorer performance on IF and Total Digit Span, but not on other measures of executive function including behavior. Conclusions: Individuals with dystrophinopathy have executive skill deficits, but they are not generally associated with more distal mutations. (JINS, 2019, 25, 146–155) |
| تدمد: | 1469-7661 1355-6177 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8c3a761b09c27515495535cbd9322741Test https://doi.org/10.1017/s1355617718000942Test |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....8c3a761b09c27515495535cbd9322741 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14697661 13556177 |
|---|