Patients with Duchenne and Becker muscular dystrophies face high rates of cognitive learning challenges, according to rating scales completed by their caregivers.

A team of researchers led by Seth Sorensen, PhD, from the University of Arkansas for Medical Sciences in Little Rock assessed the neurocognitive and learning challenges that young people with Duchenne and Becker muscular dystrophies face. To do so, they used multiple psychometric parent report measures of cognitive and learning challenges. These included the Barkley Deficits in Executive Functioning Scale short parent report measure (BDEFS-CA Short Form) to assess executive functioning and the Colorado Learning Difficulties Questionnaire (CLDQ) to assess cognitive learning.

The researchers analyzed the responses of the caregivers of 36 boys with Duchenne or Becker muscular dystrophy. They found that almost half of the boys (42% to 47%) had deficits in executive functioning. The biggest area of deficit in cognitive learning was reading. This was followed by social cognition, social isolation, spatial disorganization, and, finally, mathematics.


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There was a positive correlation between the BDEFS total summary scores and the CLDQ social cognition, spatial disorganization, social isolation, and reading scores.

“[Executive functioning] ratings were moderately to strongly related to ratings of academic and social functioning, but not significantly related to mathematics skills,” the researchers said.

These findings were presented as a poster at the 2022 Muscular Dystrophy Association Clinical & Scientific Conference held in Nashville, Tennessee, and online.

Read more about the etiology of muscular dystrophy.

Duchenne and Becker muscular dystrophies are caused by a mutation in the DMD gene, which results in either no dystrophin protein being produced, as in the case of Duchenne, or a shorter version of the protein being produced, as in the case of Becker muscular dystrophy. 

The main symptoms of the diseases are muscle wasting and atrophy, which are much more severe in the case of Duchenne muscular dystrophy. The diseases are also known to affect cognitive abilities.

Reference

Bearden R, Flegal M, Haley B, Fawcett K, Veerapandiyan A, Sorensen S. Assessment of cognitive and learning challenges in patients with dystrophinopathy via parent ratings of learning and executive functioning challenges. Poster presented at: 2022 Muscular Dystrophy Association Clinical & Scientific Conference; March 13-16, 2022; Nashville, TN. Poster 162.