Differential impact on motor unit characteristics across severities of adult spinal muscular atrophy.
Kristina Marie KellyJordan MizellLadan BigdeliSamuel PaulMarco Antonio TellezAmy BartlettSarah HeintzmanJerold Everett ReynoldsGary Brent SterlingKiran Francis RajneeshStephen James KolbBakri ElsheikhWilliam David ArnoldPublished in: Annals of clinical and translational neurology (2023)
dEMG provided reliable, noninvasive measure of MUAP amplitude size and firing rate and revealed divergent patterns across disease severity in adults with SMA. Firing rate enhancement, as seen in milder SMA, may provide a therapeutic avenue for improving function in more severe SMA, where firing rates appear preserved. MUAP amplitude size and firing rate, quantified with dEMG, may be promising monitoring biomarker candidates for noninvasive assessment of SMA.