Non-Invasive Spinal Cord Stimulation for Motor Rehabilitation of Patients with Spinal Muscular Atrophy Treated with Orphan Drugs

Spinal muscular atrophy (SMA) is an orphan disease characterized by the progressive degeneration of spinal alpha motor neurons. In recent years, nusinersen and several other drugs have been approved for the treatment of this disease. Transcutaneous spinal cord stimulation (tSCS) modulates spinal neu...

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Bibliographic Details
Published in:Biomedicines 2024-05, Vol.12 (6), p.1162
Main Authors: Novikov, Anton, Maldova, Maria, Shamantseva, Natalia, Shalmiev, Ivan, Shoshina, Elena, Epoyan, Natalia, Krutikova, Natalia, Moshonkina, Tatiana
Format: Article
Language:English
Subjects:
Age
Atrophy
Clinical medicine
Disease
Drug approval
Drug therapy
Drugs
Electrical stimuli
FDA approval
Genes
Locomotion
Motor ability
Motor activity
Motor neurons
Motor skill
Muscle strength
Neural networks
Neurons
nusinersen
onasemnogene abeparvovec
Orphan drugs
Pathogenesis
Physical therapy
Rehabilitation
Respiratory function
risdiplam
Spinal cord
Spinal cord injuries
spinal cord stimulation
Spinal muscular atrophy
Therapeutics, Physiological
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Summary:Spinal muscular atrophy (SMA) is an orphan disease characterized by the progressive degeneration of spinal alpha motor neurons. In recent years, nusinersen and several other drugs have been approved for the treatment of this disease. Transcutaneous spinal cord stimulation (tSCS) modulates spinal neuronal networks, resulting in changes in locomotion and posture in patients with severe spinal cord injury and stroke. We hypothesize that tSCS can activate motor neurons that are intact and restored by medication, slow the decline in motor activity, and contribute to the development of motor skills in SMA patients. Thirty-seven children and adults with SMA types 2 and 3 participated in this study. The median duration of drug treatment was over 20 months. The application of tSCS was performed during physical therapy for 20-40 min per day for ~12 days. Outcome measures were specific SMA motor scales, goniometry of contractured joints, and forced vital capacity. Significant increases in motor function, improved respiratory function, and decreased contracture were observed in both type 2 and 3 SMA participants. The magnitude of functional changes was not associated with participant age. Further studies are needed to elucidate the reasons for the beneficial effects of spinal cord electrical stimulation on SMA.
ISSN:2227-9059
2227-9059
DOI:10.3390/biomedicines12061162