Spinal Cord Stimulation for the Treatment of Motor Deficits in People With Spinal Muscular Atrophy - Upper Limb

Eligibility Criteria

• 1. Subject has a diagnosis of 5q-autosomal recessive SMA confirmed by determination of a genetic deletion in the SMN1 gene (5q12.2-q13.3).
• 2. Subject is diagnosed as being non-ambulatory SMA based on the following criteria:
• 3. Subject is ≥16 years of age and \< 65 years of age.
• 4. Subject is able to sit independently.
• 5. A minimum score of 1 for Entry Item "A" of the Revised Upper Limb Module (RULM) scale for SMA: "Can use hands to hold pencil or pick up a coin/token or drive a powered chair, use phone key pad"
• 6. Subject (and subject's parent or legal guardian if subject is a minor) is willing and able to comply with scheduled visits and study procedures
• 7. Participants must have started SMN inducing therapies (Spinraza or risdisplam) at least 6 months prior to enrollment. (They must have either gotten their first injection at at least 6 months prior, or they started daily intake of risdisplam at least 6 months prior to the study)
• 1. Subject has deformation of the spinal canal preventing lead implantation as judged by the study neurosurgeon
• 2. Subject has size of spinal canal that is insufficient for lead implantation as judged by the study neurosurgeon
• 3. Subject has moderate or severe joint contractures that would affect ability to perform study measures, determined by the study physician
• 4. Subject has severe behavioral or cognitive problems that preclude participation in the study, in the opinion of the investigator
• 5. Subject has previous or ongoing medical condition, medical history, physical findings or laboratory abnormalities that could affect safety of anesthesia or the procedures, make it unlikely that intervention or follow-up will be correctly completed or impair the assessment of study results, in the opinion of the investigator
• 6. Female subjects are pregnant or breastfeeding, as established by self-report.
• 7. Subject has severe claustrophobia
• 8. Subject is on anticoagulant, anti-spasticity or anti-seizure medication within 4 weeks of lead implantation or requires these medications during the treatment phase of the study
• 9. Subject has medical implant that precludes magnetic resonance imaging
• 10. Subject has a deconditioned respiratory system, per the discretion of the physician investigator.
• 11. Subjects with renal insufficiency at the discretion of the physician investigator.
• 12. Subjects requiring any form of sedation for MRI will be excluded.