Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

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

Tatiana Moshonkina
* ORCID logo ,
Anton Novikov
,
Maria Maldova
,
Ivan Shalmiev
,
Elena Shoshina
,
Natalia Epoyan
,
Natalia D. Shamantseva
ORCID logo ,
Natalia Krutikova
Version 1 : Received: 16 April 2024 / Approved: 17 April 2024 / Online: 17 April 2024 (13:30:10 CEST)

How to cite: Moshonkina, T.; Novikov, A.; Maldova, M.; Shalmiev, I.; Shoshina, E.; Epoyan, N.; Shamantseva, N.D.; Krutikova, N. Non-Invasive Spinal Cord Stimulation for Motor Rehabilitation of Patients with Spinal Muscular Atrophy Treated with Orphan Drugs. Preprints 2024, 2024041164. https://doi.org/10.20944/preprints202404.1164.v1 Moshonkina, T.; Novikov, A.; Maldova, M.; Shalmiev, I.; Shoshina, E.; Epoyan, N.; Shamantseva, N.D.; Krutikova, N. Non-Invasive Spinal Cord Stimulation for Motor Rehabilitation of Patients with Spinal Muscular Atrophy Treated with Orphan Drugs. Preprints 2024, 2024041164. https://doi.org/10.20944/preprints202404.1164.v1

Abstract

Spinal muscular atrophy (SMA) is an orphan disease characterized by 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 the study. The median duration of drug treatment was over 20 months. Application of tSCS was performed during physical therapy for 20-40 minutes per day for ~12 days. Outcome measures were specific SMA motor scales, goniometry of contractured joints, forced vital capacity. Significant increases in motor function, improved respiratory function, and decreased contracture were observed in both types 2 and 3 SMA participants. The magnitude of functional changes was not associated with participants age. Further studies are needed to elucidate the reasons for the beneficial effects of spinal cord electrical stimulation in SMA.

Keywords

spinal muscular atrophy; spinal cord stimulation; physical therapy; nusinersen; risdiplam; onasemnogene abeparvovec

Subject

Biology and Life Sciences, Neuroscience and Neurology

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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