Motor function linked to cognitive differences for SMA type 3 adults

In adults with spinal muscular atrophy (SMA) type 3people with greater motor impairment tend to score better on some cognitive measures but worse on others, a new study reports.

Among those with SMA, “patients with greater motor difficulties had lower performance in attention but higher performance in language, verbal fluency and memory,” the researchers wrote.

The study, “Cognitive Profiles and Clinical Factors in Type III Spinal Muscular Atrophy: A Preliminary Study”, was published in the magazine Neuromuscular Disorders

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SMA is a neuromuscular disorder characterized by the loss of motor neurons, the nerve cells responsible for coordinating muscle movements. Other neuromuscular disorders such as amyotrophic lateral sclerosis and Duchenne muscular dystrophy known to sometimes cause cognitive impairment.

SMA is generally not associated with cognitive problems, but how the disease affects cognition remains incompletely understood. In addition to SMA’s direct effects on the nervous system, an emerging study suggests that SMA may cause cognitive differences, in part because patients live with different physical limitations than their peers, so they must find different strategies to navigate daily life.

In this study, led by scientists from the University of Padova in Italy, 22 adults with SMA type 3 (ages 18-56) underwent a series of neuropsychological tests, as well as measurements of motor function.

“We used a comprehensive battery that allowed us to closely examine performance in multiple cognitive domains,” the researchers wrote.

By comparison, the study also included adults without SMA (controls). The two groups were similar in terms of age and sex, although the controls were more formally trained. The researchers explained this difference in statistical analyses, but still acknowledged it as a limitation of the study, as well as the small number of participants.

Compared to the control group, the SMA patients scored significantly lower in language skills, visual-spatial skills (telling where objects are based on sight) and executive function (the cognitive process of controlling your own behavior).

Statistical analyzes showed that, among the SMA patients, those with worse scores on assessments of motor function or disease severity tended to score lower on measures of attention, but higher on measures of language, verbal fluency and memory. Gender stratified analyzes generally showed similar trends, although more pronounced for men.

The association between motor symptoms and diminished scores in measures of attention “may indicate presence” [of] structural or functional brain changes in SMA type III,” the researchers said, as this association implies that some cognitive problems tend to worsen as other SMA symptoms worsen. However, they cautioned that no far-reaching conclusions can be drawn from this preliminary study.

“Studies with a larger sample [sizes] are needed to understand the frequency of cognitive ‘disorders’ and which factors may mediate the pathways of cognitive functions,” the researchers wrote. They also noted that future studies should assess whether cognitive problems are related to visible changes in the brain or to psychological challenges such as depression and anxiety.

The fact that poorer motor scores were associated with better scores in some areas of cognition adds to the growing body of evidence suggesting that people with SMA experience cognitive changes to compensate for physical limitations associated with the disease, the researchers said. .

“Together, our results partially support previous findings, and as a whole may point to a possible compensatory mechanism or coping strategies in SMA that may positively impact cognitive development,” the team concluded.


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