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

Author:

Koeks Zaıda1,Hellebrekers Danique M.J.23,van de Velde Nienke M.14,Alleman Iris5,Spitali Pietro6,van Duyvenvoorde Hermine A.7,Verschuuren Jan J.G.M.14,Hendriksen Jos G.M.234,Niks Erik H.14

Affiliation:

1. Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands

2. Centre for Neurological Learning Disabilities, Kempenhaeghe, Heeze, the Netherlands

3. School for Mental Health and Neuroscience>, Maastricht University, Maastricht, The Netherlands

4. Duchenne Center Netherlands

5. Department of Orthopaedics, Rehabilitation and Physical Therapy, Leiden University Medical Center, Leiden, The Netherlands

6. Department of Human Genetics, Leiden University Medical Center, Leiden, the Netherlands

7. Department of Clinical Genetics, Leiden University Medical Center, Leiden the Netherlands

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.

Publisher

IOS Press

Subject

Neurology (clinical),Neurology

Reference30 articles.

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