The neurocognitive profile of adults with Becker muscular dystrophy in the Netherlands

Z. Koeks, D.M.J. Hellebrekers, N.M. Van de Velde, I. Alleman, P. Spitali, H.A. Van Duyvenvoorde, J.J.G.M. Verschuuren, J.G.M. Hendriksen, E.H. Niks*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Background: In Becker muscular dystrophy evidence for neurocognitive and behavioral comorbidity is evolving. More insight into the extend of these problems is of great importance for early detection and remediation in clinical practice.Objective: In this study we aimed to describe the neurocognitive and behavioral features of a Dutch adult cohort of BMD patients, and to evaluate correlations to motor function outcomes.Methods: 28 adult BMD patients were included. Intelligence, executive functioning, verbal memory and reaction times were assessed cross-sectionally. Additionally, patients completed questionnaires on behavioral and emotional symptoms, psychosocial and executive functions. Results were compared to normative data and correlated with disease severity as measured by the 10-meter run/walk test and Performance of the Upper Limb version 1.2.Results: 15 patients (53.6%) had a high educational level despite frequent grade repeating (48.3%) during primary or secondary school. Neuropsychological testing revealed that intellectual abilities, verbal memory, processing speed and executive functioning were statistically significant below average, but still within normal range. Regarding outcomes of the behavioral questionnaires, no significant differences were reported compared to the norm population. No relevant correlations with disease severity were found.Conclusions: This cohort of adult BMD patients exhibits minor cognitive impairments and no significant behavioral problems. The lower outcomes on processing speed and verbal memory, combined with the relatively high prevalence of grade repeating during primary and secondary school, implies that these minor impairments played a role in childhood. However, the on average high educational levels suggests that they grow out of their cognitive impairments with ageing.
Original languageEnglish
Pages (from-to)543-553
Number of pages11
JournalJournal of neuromuscular diseases
Volume9
Issue number4
DOIs
Publication statusPublished - 2022

Keywords

  • Muscular dystrophy
  • dystrophin
  • cognition
  • cognitive dysfunction
  • intellectual disability
  • behavior
  • NEUROPSYCHOLOGICAL PROFILE
  • MEMORY
  • BOYS
  • CHILDREN
  • MALES
  • PERFORMANCE
  • SCALE
  • ADHD

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