Abstract
Although multiple clinical trials of various therapeutic interventions in amyotrophic lateral sclerosis (ALS) have been conducted so far, most of them lack comprehensive study design due to the absence of progression biomarkers that could provide adequate understanding of the rate and pattern of disease progression. Motor unit number estimation (MUNE) methods, motor unit number index (MUNIX) in particular, could be a promising tool for evaluating progression in ALS. This paper provides an extensive review of the main aspects of current MUNIX methodology and its emerging modifications, correlations with clinical and electrodiagnostic measures, reproducibility issues, utility in motor neuron disease diagnostics, and assessment of disease progression in clinical trials.
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