Neurobehavioral Concerns Among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network

Kristin Caspers Conway; Katherine D Mathews; Pangaja Paramsothy; Joyce Oleszek; Christina Trout; Ying Zhang; Paul A Romitti; MD STARnet

doi:10.1097/DBP.0000000000000177

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Neurobehavioral Concerns Among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network

Journal article

Peer reviewed

Neurobehavioral Concerns Among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network

Kristin Caspers Conway, Katherine D Mathews, Pangaja Paramsothy, Joyce Oleszek, Christina Trout, Ying Zhang, Paul A Romitti and MD STARnet

Journal of developmental and behavioral pediatrics, Vol.36(6), pp.455-463

07/2015

DOI: 10.1097/DBP.0000000000000177

PMCID: PMC4497929

PMID: 26020585

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Abstract

To describe the occurrence of selected neurobehavioral concerns among males with a dystrophinopathy and to explore the associations with corticosteroid or supportive device use. 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. 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. 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.

Registries

United States - epidemiology

Depression - epidemiology

Self-Help Devices - statistics & numerical data

Humans

Child, Preschool

Infant

Male

Muscular Dystrophy, Duchenne - epidemiology

Adrenal Cortex Hormones - therapeutic use

Muscular Dystrophy, Duchenne - complications

Depression - etiology

Young Adult

Problem Behavior

Adolescent

Attention Deficit Disorder with Hyperactivity - etiology

Adult

Muscular Dystrophy, Duchenne - therapy

Child

Attention Deficit Disorder with Hyperactivity - epidemiology

Details

Title: Subtitle: Neurobehavioral Concerns Among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network
Creators: Kristin Caspers Conway - University of Iowa
Katherine D Mathews - University of Iowa, Neurology
Pangaja Paramsothy
Joyce Oleszek
Christina Trout
Ying Zhang - Indiana University
Paul A Romitti - University of Iowa, Neurology
MD STARnet
Resource Type: Journal article
Publication Details: Journal of developmental and behavioral pediatrics, Vol.36(6), pp.455-463
DOI: 10.1097/DBP.0000000000000177
PMID: 26020585
PMCID: PMC4497929
NLM abbreviation: J Dev Behav Pediatr
ISSN: 0196-206X
eISSN: 1536-7312
Publisher: United States
Grant note: U01 DD000189 / NCBDD CDC HHS U01 DD001119 / NCBDD CDC HHS CC999999 / Intramural CDC HHS UL1 TR001108 / NCATS NIH HHS UL1 TR002529 / NCATS NIH HHS
Language: English
Date published: 07/2015
Academic Unit: Neurology; Stead Family Department of Pediatrics; Epidemiology; Iowa Neuroscience Institute; Biostatistics; Neurology (Pediatrics)
Record Identifier: 9983995061202771

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