Evidence map›Paper›PMID 41772190›Full record

Trial reportJournal of neurology2026

Electrophysiological assessment of motor unit loss in adult spinal muscular atrophy types III and IV: a multicenter national study comparing MUNIX, CMAP, and MUSIX.

Eva Sole-Cruz, Emmanuelle Salort-Campana, Timothee Lenglet, Etienne Fortanier, Yann Pereon, Edouard Berling, Sadia Beloribi-Djefaflia, Françoise Bouhour, Pascal Cintas, Emilien Delmont and 14 more

Registry-linked trialAbstract readClinical TrialComparative StudyMulticenter Study
PubMed Publisher
In one paragraph

Trial report in Journal of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04690998 (Outcome Measures and Biomarkers in a Cohort of Spinal Muscular Atrophy Type III/ IV Patients), which is not on this map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT04690998 naactive not recruitingnot on this map

Outcome Measures and Biomarkers in a Cohort of Spinal Muscular Atrophy Type III/ IV Patients

TypeinterventionalSponsorAssistance Publique Hopitaux De MarseilleRan2021 to 2025Enrolled104ConditionsSpinal Muscular AtrophyArmsBlood Samples, MRI
3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

24 authors.

Eva Sole-CruzCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.
Emmanuelle Salort-CampanaCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.
Timothee LengletCentre de référence de pathologie neuromusculaire Paris-Est, groupe hospitalier Pitié-Salpêtrière, institut de Myologie, AP-HP, Paris, France.
Etienne FortanierCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.
Yann PereonCentre de Référence Maladies Neuromusculaires AOC, Filnemus, Euro-NMD, Hôtel-Dieu, CHU de Nantes, Nantes, France.
Edouard BerlingService de Neurologie, Centre de référence des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, FHU Phenix, Garches, France.
Sadia Beloribi-DjefafliaCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.
Françoise BouhourService d'Electroneuromyographie et Pathologies Neuromusculaires, centre de référence des maladies neuromusculaires PACA-Réunion-Rhône Alpes, Hôpital Neurologique P. Wertheimer, Hospices Civils de Lyon, Lyon, France.
Pascal CintasService de Neurologie, Centre de référence des Maladies Neuromusculaires, CHU de Toulouse Purpan, Toulouse, France.
Emilien DelmontCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.
Michelle CavalliService de Neurologie: Système nerveux périphérique, Muscle et SLA, Hôpital Pasteur 2, CHU de Nice, Nice, France.
Andra EzaruService de Neurologie: Système nerveux périphérique, Muscle et SLA, Hôpital Pasteur 2, CHU de Nice, Nice, France.
Elisa De La CruzService de Neurologie, CHU Gui de Chauliac, Montpellier, France.
Sara FernandesCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.
Anne-Laure KaminskyService de Neurologie, Centre Référent des Maladies Neuromusculaires Rares, CHU de Saint Etienne, Saint-Etienne, France.
Ludivine KoutonCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.
Armelle MagotCentre de Référence Maladies Neuromusculaires AOC, Filnemus, Euro-NMD, Hôtel-Dieu, CHU de Nantes, Nantes, France.
Aleksandra Nadaj-PaklezaService de Neurologie, Centre de référence des maladies neuromusculaires Nord/Est/Ile-de-France, CHU de Strasbourg, 67000, Strasbourg, France.
Céline TardService de Neurologie, U1172, Centre de Référence des Maladies Neuromusculaires Nord/Est/Ile-de-France, CHU de Lille, Lille, France.
Antoine PegatService d'Electroneuromyographie et Pathologies Neuromusculaires, centre de référence des maladies neuromusculaires PACA-Réunion-Rhône Alpes, Hôpital Neurologique P. Wertheimer, Hospices Civils de Lyon, Lyon, France.
Guillaume NicolasService de Neurologie, Centre de référence des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, FHU Phenix, Garches, France.
Tanya StojkovicCentre de référence de pathologie neuromusculaire Paris-Est, groupe hospitalier Pitié-Salpêtrière, institut de Myologie, AP-HP, Paris, France.
Jean-Yves HogrelCentre de référence de pathologie neuromusculaire Paris-Est, groupe hospitalier Pitié-Salpêtrière, institut de Myologie, AP-HP, Paris, France.
Shahram AttarianCentre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France. shahram.attarian@ap-hm.fr.ORCID http://orcid.org/0000-0002-7211-4694

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundSpinal muscular atrophy (SMA) types III and IV are the most common late-onset forms, and they progress slowly, making the identification of sensitive biomarkers critical. The Motor Unit Number Index (MUNIX) estimates motor unit loss and may complement traditional electrophysiological measurements such as Compound Muscle Action Potential amplitudes (CMAP). However, their respective performances have never been directly compared in adult SMA.

methodsIn a French multicenter study (NCT04690998), 71 adult patients with SMA and 24 healthy controls underwent clinical and electrophysiological evaluation. MUNIX, CMAP, and Motor Unit Size Index (MUSIX) were recorded in four muscles, and sum scores (SumMUNIX, SumCMAP, SumMUSIX) were calculated. Reliability was assessed using intraclass correlation coefficients (ICCs), and associations with functional outcomes were explored.

resultsMUNIX and CMAP effectively distinguished SMA patients from controls, showing strong test - retest reliability. MUNIX showed the highest discriminative performance (AUC = 0.92), while CMAP demonstrated the strongest and most consistent associations with clinical severity. In multivariate analyses, only CMAP remained independently associated with all functional and strength measures, whereas MUNIX and MUSIX lost significance.

conclusionMUNIX demonstrated the highest discriminative performance among biomarkers for differentiating SMA from controls, indicating early motor unit loss even when CMAP values were within normal limits. However, disease burden and functional impairment were better reflected by CMAP, probably due to it integrating both motor unit loss and reinnervation. The complementary nature of these profiles supports their combined use (concurrent application), and longitudinal studies are warranted to assess their responsiveness in adult SMA as well as their appropriateness for clinical trial settings.

Indexed as

Action PotentialsMotor NeuronsMuscle, SkeletalRecruitment, NeurophysiologicalSpinal Muscular Atrophies of ChildhoodAdultAgedElectromyographyFemaleFranceHumansMaleMiddle AgedReproducibility of ResultsYoung AdultBiomarkersCompound muscle action potentialElectromyographyMUNIXSpinal muscular atrophy

Identifiers

What Socratic holds

Textmetadata
Read underepoch 390

Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.