Cognitive abilities stable over time in Becker MD boys, study finds

But changes seen over its year in executive function, and worth monitoring

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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Cognitive function in boys with Becker muscular dystrophy (BMD) appear to be stable over time, although these children may struggle with working memory and executive function, a small study reports.

Based on its findings, the researchers are calling for boys with BMD to have access to routine cognitive and psychiatric monitoring so that, if issues develop, they can be detected and addressed.

The study, “Evolution of neuropsychological and behavioral profile in a cohort of Becker muscular dystrophy pediatric patients in a longitudinal study,” which was published in Neuromuscular Disorders.

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Disease progression with BMD largely seen only in adults: Study

Cognitive function studied with Duchenne, but less so with Becker MD

BMD is caused by mutations in the gene that provides instructions for making dystrophin. In BMD, dystrophin is present but at low levels or is dysfunctional, which differentiates Becker MD from the more severe Duchenne muscular dystrophy (DMD), where the protein is absent entirely.

Prior research has suggested that DMD patients without obvious cognitive problems still may struggle with executive function, the ability to regulate and direct one’s thoughts and actions. DMD studies also suggest that even when deficits are present, executive function and other cognitive abilities tend to be stable over time.

Studies into how cognitive processes may change over time in people with Becker muscular dystrophy, however, are limited.

In this study, 17 boys with BMD, ages 5 to 18, underwent a detailed battery of cognitive and psychiatric tests, then underwent the same tests again one year later. These tests included assessments of executive memory, language, behavioral issues, adaptive functioning — the ability to cope with he needs of daily life and their degree of adaptation — as well as intellectual functioning.

The boys’ parents also completed parts of these assessments. The researchers, all in Italy, compared the scores to normative values for the general population, and also assessed how scores changed over time.

“In this study, we described for the first time the longitudinal trend of the neuropsychological and behavioral profile in a pediatric population affected by BMD,” the scientists wrote.

A normal cognitive profile seen, but decline possible in some areas

Results showed that most scores were more or less within normal ranges, and generally without much change over time.

“The longitudinal evaluation showed that the cognitive profile of these patients remains stable over time,” the researchers wrote, noting that this finding is similar to what’s been previously reported for people with DMD.

However, the scientists noted that measures of executive function and working memory (the ability to keep information at the forefront of the mind) tended to be poorer after one year than initial measures.

They stressed that this was a small study lasting for a year, so it’s not entirely clear how meaningful the measured differences are or if such changes are just normal variations for growing boys. Still, findings suggest that some BMD children may start to experience “learning disorders” that require extra support.

“While our findings are not conclusive … [or] demonstrative of a neuropsychological diagnosis, we believe that it should be recommended to routinely monitor the cognitive and neuropsychological profile during the development of these children in order to early detect any difficulties,” the scientists concluded.

Doing so would “engage these children as soon as possible within a multimodal rehabilitation program aimed at treating and preventing learning difficulties, as well as improving their general quality of life and also preventing any emotional distress,” they added.