Prescribing Psychical Activity Regimens in Spinal Muscular Atrophy: Jacqueline Montes, PT, EdD

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"The caveat is that [the physical exercise] needs to be modified so that it’s doable. They can’t necessarily go for a run like you and me, and the progress towards that goal has to be slow and incremental."

Spinal muscular atrophy (SMA), an autosomal recessive neurodegenerative disease, is characterized by degeneration of spinal cord α‐motor neurons, which results in progressive proximal muscle weakness, fatigue, scoliosis, nutritional problems, respiratory complications, and severe functional limitations. Studies have shown that training may improve functional performance, muscle strength, and exercise capacity by optimizing resources and metabolic function in available muscle tissue and counteracting further muscle deterioration that occurs with inactivity.

Exercise might also have a neuroprotective effect, which could be explained by a relationship between the maturation state of the motor unit and resistance to neuronal cell death. Physical exercise training has emerged as a potential intervention for people with inherited neuromuscular disorders for which no curative treatment is as yet available, including people with SMA; however, prescribing the right “dosage” of these interventions remains a challenge.

At the 2022 Cure SMA Clinical & Scientific Annual Meeting, June 15-17, in Anaheim, California, Jacqueline Montes, PT, EdD, presented an analysis that evaluated changes in aerobic capacity and physical activity in ambulatory adults with SMA treated with nusinersen (Spinraza; Biogen) over a 6-month period. All told, there were no improvements in function, fatigue, or muscle oxygen uptake, but patients did show trends in increased percentages of predicted maximal heart rate.

Montes, associate professor of rehabilitation and regenerative medicine at Columbia University Medical Center, sat down with NeurologyLive^® to discuss the complexities in prescribing physical exercise for patients with SMA. She talked about the approach for these individuals, what previous literature has demonstrated, and the types of specific limitations patients may encounter. She also provided insight on the state of SMA drug development and the next steps in advancing the field.