title: Neurobehavioral Concerns Among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network.
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.
  
  
  CONCLUSIONS
  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.affiliation: *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.
authors.forename: Kristin
authors.initials: K
authors.lastname: Caspers Conway
authors.forename: Katherine D
authors.initials: KD
authors.lastname: Mathews
authors.forename: Pangaja
authors.initials: P
authors.lastname: Paramsothy
authors.forename: Joyce
authors.initials: J
authors.lastname: Oleszek
authors.forename: Christina
authors.initials: C
authors.lastname: Trout
authors.forename: Ying
authors.initials: Y
authors.lastname: Zhang
authors.forename: Paul A
authors.initials: PA
authors.lastname: Romitti
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D001289:Attention Deficit Disorder with Hyperactivity / Q000453:epidemiology* / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003863:Depression / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000453:epidemiology* / Q000628:therapy*; D000066553:Problem Behavior; D012042:Registries; D012656:Self-Help Devices / Q000706:statistics & numerical data*; D014481:United States / Q000453:epidemiology; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones