Assessment of motor unit firing by high‐density surface electromyography detects motor neuronal hyperexcitable state in amyotrophic lateral sclerosis

Introduction/Aims In amyotrophic lateral sclerosis (ALS), the impact of motor neuron dysfunction on the motor unit (MU) firing pattern remains to be elucidated. The aim of this study was to clarify the characteristics of the MU firing rate and its association with clinical factors in ALS patients using high‐density surface electromyography (HDSEMG) and MU decomposition analysis. Methods Nineteen ALS patients and 20 controls prospectively underwent HDSEMG recording of the vastus lateralis muscle during ramp‐up (30% of maximum voluntary contraction) and sustained (10% of maximal voluntary contraction for 60 seconds) contractions on performing isometric knee extension. After decomposition analysis, instantaneous firing rates (IFRs) of individually identified MUs were calculated. Comparison of IFRs and clinical variables between ALS patients and controls and analysis of the correlation between individual mean IFR and clinical variables in ALS patients were performed. Results The number of identified MUs was lower in ALS patients than in controls (P = .017). Mean IFRs of MUs (i.e., mean MU firing rates) were higher in ALS patients than in controls at some force levels on ramp‐up contraction (P