Abstract

ObjectiveTo evaluate how the motor unit number index (MUNIX) is related to high-density motor unit number estimation (HD-MUNE) in healthy controls and patients with amyotrophic lateral sclerosis (ALS). MethodsBoth MUNIX and HD-MUNE were performed on the thenar muscles in 18 ALS patients and 24 healthy controls. Patients were measured at baseline, within 2weeks, and after 4 and 8months. Clinical evaluation included Medical Research Council (MRC) scale and the ALS functional rating scale (ALSFRS). ResultsThere was a significant positive correlation between MUNE and MUNIX values in ALS patients (r=0.49 at baseline; r=0.56 at 4months; r=0.56 at 8months, all p<0.05), but not in healthy controls. After 8months, both MUNE and MUNIX values of the ALS patients decreased significantly more compared to MRC scale, ALS functional rating scale (ALSFRS) and compound muscle action potential (CMAP) (p<0.05). There was no significant difference in relative decline of MUNIX and HD-MUNE values. ConclusionsIn ALS patients, MUNIX and HD-MUNE are significantly correlated. MUNIX has an almost equivalent potential in detecting motor neuron loss compared to HD-MUNE. SignificanceMUNIX could serve as a reliable and sensitive marker for monitoring disease progression in ALS.

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