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Intellect and behaviour in Duchenne muscular dystrophy

D Leibowitz, V Dubowitz

    Developmental Medicine and Child Neurology
    |October 1, 1981
    PubMed
    Summary
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    Intellectual and emotional difficulties are common in boys with Duchenne muscular dystrophy. These cognitive and behavioral issues, including impaired reading, are not progressive and do not impact all children with the condition.

    Area of Science:

    • Neurology
    • Developmental Pediatrics
    • Child Psychology

    Background:

    • Duchenne muscular dystrophy (DMD) is a progressive genetic disorder primarily affecting males.
    • Understanding the non-motor aspects of DMD, such as cognitive and emotional functioning, is crucial for comprehensive care.
    • Previous research suggests potential links between DMD and intellectual or behavioral challenges.

    Purpose of the Study:

    • To investigate the intellectual, educational, and emotional functioning in a cohort of boys with Duchenne muscular dystrophy.
    • To determine if cognitive and emotional impairments associated with DMD are progressive or age-dependent.
    • To provide a clearer picture of the cognitive profile and behavioral patterns in children with DMD.

    Main Methods:

    • A cohort of 57 boys diagnosed with Duchenne muscular dystrophy, aged 3–13 years, participated in the study.

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  • Standardized intelligence tests were administered to assess cognitive abilities.
  • Educational assessment included a reading test, and emotional functioning was evaluated using the Rutter Behaviour Questionnaires.

    • Main Results:

    • Boys with DMD scored approximately 1 standard deviation below the mean on intelligence tests, with a tendency towards better performance on non-verbal tasks.
    • Reading abilities varied, averaging 1.5 standard deviations below the mean, with a significant number exhibiting very low reading levels.
    • Both Rutter Behaviour Questionnaires indicated a high prevalence of emotional disorders among the participants.

    Conclusions:

    • Intellectual impairment, particularly verbal deficits, is associated with Duchenne muscular dystrophy but appears to be non-progressive.
    • A significant proportion of boys with DMD experience emotional disturbances, highlighting the need for psychological support.
    • The findings underscore the importance of assessing cognitive and emotional well-being in the management of Duchenne muscular dystrophy.