Kristin Caspers Conway1, Katherine D Mathews, Pangaja Paramsothy, Joyce Oleszek, Christina Trout, Ying Zhang, Paul A Romitti. 1. *Department of Epidemiology, College of Public Health, The University of Iowa, Iowa City, IA; †Departments of Pediatrics and Neurology, Carver College of Medicine, The University of Iowa, Iowa City, IA; ‡National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA; §Department of Physical Medicine and Rehabilitation, University of Colorado School of Medicine and Children's Hospital Colorado, Denver, CO; ‖Department of Biostatistics, College of Public Health, Fairbanks School of Public Health and School of Medicine, Indiana University, Indianapolis, IN.
Abstract
OBJECTIVE: To describe the occurrence of selected neurobehavioral concerns among males with a dystrophinopathy and to explore the associations with corticosteroid or supportive device use. METHODS: Medical record abstraction of neurobehavioral concerns was conducted for 857 affected males from 765 families, born since 1982 and followed through 2011, and enrolled in the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network. Cumulative probabilities for attention-deficit hyperactivity disorder (ADHD), behavior problems, and depressed mood were calculated from Kaplan-Meier estimates for the subsample of oldest affected males (n = 765). Hazard ratios (HRs) and 95% confidence intervals (95% CIs) for corticosteroid and supportive device use were estimated from Cox regression models with time-dependent covariates. RESULTS: Of the 857 affected males, 375 (44%) had at least 1 of the 3 selected neurobehavioral concerns; a similar percentage (45%) was found among the 765 oldest affected males. The estimated cumulative probabilities among these oldest affected males were 23% for ADHD, 43% for behavior problems, and 51% for depressed mood. Corticosteroid (HR = 2.35, 95% CI = 1.75-3.16) and mobility device (HR = 1.53, 95% CI = 1.06-2.21) use were associated with behavior problems. Use of a mobility device (HR = 3.53, 95% CI = 2.13-5.85), but not corticosteroids, was associated with depressed mood. ADHD was not significantly associated with corticosteroid or mobility device use. Respiratory assist device use was not examined due to low numbers of users before onset of neurobehavioral concerns. CONCLUSION: Selected neurobehavioral concerns were common among males with a dystrophinopathy. Reported associations highlight the importance of increased monitoring of neurobehavioral concerns as interventions are implemented and disease progresses.
OBJECTIVE: To describe the occurrence of selected neurobehavioral concerns among males with a dystrophinopathy and to explore the associations with corticosteroid or supportive device use. METHODS: Medical record abstraction of neurobehavioral concerns was conducted for 857 affected males from 765 families, born since 1982 and followed through 2011, and enrolled in the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network. Cumulative probabilities for attention-deficit hyperactivity disorder (ADHD), behavior problems, and depressed mood were calculated from Kaplan-Meier estimates for the subsample of oldest affected males (n = 765). Hazard ratios (HRs) and 95% confidence intervals (95% CIs) for corticosteroid and supportive device use were estimated from Cox regression models with time-dependent covariates. RESULTS: Of the 857 affected males, 375 (44%) had at least 1 of the 3 selected neurobehavioral concerns; a similar percentage (45%) was found among the 765 oldest affected males. The estimated cumulative probabilities among these oldest affected males were 23% for ADHD, 43% for behavior problems, and 51% for depressed mood. Corticosteroid (HR = 2.35, 95% CI = 1.75-3.16) and mobility device (HR = 1.53, 95% CI = 1.06-2.21) use were associated with behavior problems. Use of a mobility device (HR = 3.53, 95% CI = 2.13-5.85), but not corticosteroids, was associated with depressed mood. ADHD was not significantly associated with corticosteroid or mobility device use. Respiratory assist device use was not examined due to low numbers of users before onset of neurobehavioral concerns. CONCLUSION: Selected neurobehavioral concerns were common among males with a dystrophinopathy. Reported associations highlight the importance of increased monitoring of neurobehavioral concerns as interventions are implemented and disease progresses.
Authors: Ruth Perou; Rebecca H Bitsko; Stephen J Blumberg; Patricia Pastor; Reem M Ghandour; Joseph C Gfroerer; Sarra L Hedden; Alex E Crosby; Susanna N Visser; Laura A Schieve; Sharyn E Parks; Jeffery E Hall; Debra Brody; Catherine M Simile; William W Thompson; Jon Baio; Shelli Avenevoli; Michael D Kogan; Larke N Huang Journal: MMWR Suppl Date: 2013-05-17
Authors: Susanna N Visser; Melissa L Danielson; Rebecca H Bitsko; Joseph R Holbrook; Michael D Kogan; Reem M Ghandour; Ruth Perou; Stephen J Blumberg Journal: J Am Acad Child Adolesc Psychiatry Date: 2013-11-21 Impact factor: 8.829
Authors: ThuyQuynh N Do; Natalie Street; Jennifer Donnelly; Melissa M Adams; Christopher Cunniff; Deborah J Fox; Richard O Weinert; Joyce Oleszek; Paul A Romitti; Christina P Westfield; Julie Bolen Journal: Birth Defects Res Date: 2018-08-02 Impact factor: 2.344
Authors: Kashika M Sahay; Tiffany Smith; Kristin M Conway; Paul A Romitti; Molly M Lamb; Jennifer Andrews; Shree Pandya; Joyce Oleszek; Christopher Cunniff; Rodolfo Valdez Journal: J Child Neurol Date: 2018-10-22 Impact factor: 1.987
Authors: Mathula Thangarajh; Jos Hendriksen; Michael P McDermott; William Martens; Kimberly A Hart; Robert C Griggs Journal: Neurology Date: 2019-10-08 Impact factor: 9.910
Authors: Mathula Thangarajh; Christopher F Spurney; Heather Gordish-Dressman; Paula R Clemens; Eric P Hoffman; Craig M McDonald; Erik K Henricson Journal: PLoS Curr Date: 2018-10-17
Authors: Kristin M Conway; Katy Eichinger; Christina Trout; Paul A Romitti; Katherine D Mathews; Shree K Pandya Journal: SAGE Open Med Date: 2019-03-02