ArticleScientific reports2023
Motor unit number index (MUNIX) loss of 50% occurs in half the time of 50% functional loss according to the D50 disease progression model of ALS.
Article in Scientific reports, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 4 citations in OpenAlex.
- Electrophysiological assessment of motor unit loss in adult spinal muscular atrophy types III and IV: a multicenter national study comparing MUNIX, CMAP, and MUSIX.Journal of neurology · 2026Trial
- Troponin T and Neurofilament Light Chain Levels as Complementary Biomarkers of Disease Accumulation and Aggressiveness in Amyotrophic Lateral Sclerosis.Annals of clinical and translational neurology · 2026Article
- Quantifying motor unit loss prior to functional impairment in muscles affected by amyotrophic lateral sclerosis.Clinical neurophysiology practice · 2026Article
- Prognostic Usefulness of Motor Unit Number Index (MUNIX) in Patients Newly Diagnosed with Amyotrophic Lateral Sclerosis.Journal of clinical medicine · 2023Article
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
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Abstract
Capturing disease progression in amyotrophic lateral sclerosis (ALS) is challenging and refinement of progression markers is urgently needed. This study introduces new motor unit number index (MUNIX), motor unit size index (MUSIX) and compound muscle action potential (CMAP) parameters called M50, MUSIX200 and CMAP50. M50 and CMAP50 indicate the time in months from symptom onset an ALS patient needs to lose 50% of MUNIX or CMAP in relation to the mean values of controls. MUSIX200 represents the time in months until doubling of the mean MUSIX of controls. We used MUNIX parameters of Musculi abductor pollicis brevis (APB), abductor digiti minimi (ADM) and tibialis anterior (TA) of 222 ALS patients. Embedded in the D50 disease progression model, disease aggressiveness and accumulation were analyzed separately. M50, CMAP50 and MUSIX200 significantly differed among disease aggressiveness subgroups (p < 0.001) regardless of disease accumulation. ALS patients with a low M50 had a significantly shorter survival compared to high M50 (median 32 versus 74 months). M50 preceded the loss of global function (median of about 14 months). M50, CMAP50 and MUSIX200 characterize the disease course in ALS in a new way and may be applied as early measures of disease progression.
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