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

Λεπτομέρειες βιβλιογραφικής εγγραφής
Τίτλος:	Electrophysiological assessment of motor unit loss in adult spinal muscular atrophy types III and IV: a multicenter national study comparing MUNIX, CMAP, and MUSIX.
Συγγραφείς:	Sole-Cruz E; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France., Salort-Campana E; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France.; EURO-NMD: European Reference Network for Rare Neuromuscular Diseases, Paris, France.; Filière de Santé FILNEMUS, Paris, France., Lenglet T; Centre de référence de pathologie neuromusculaire Paris-Est, groupe hospitalier Pitié-Salpêtrière, institut de Myologie, AP-HP, Paris, France., Fortanier E; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France., Pereon Y; Centre de Référence Maladies Neuromusculaires AOC, Filnemus, Euro-NMD, Hôtel-Dieu, CHU de Nantes, Nantes, France., Berling E; Service de Neurologie, Centre de référence des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, FHU Phenix, Garches, France., Beloribi-Djefaflia S; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France., Bouhour F; Service 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., Cintas P; Service de Neurologie, Centre de référence des Maladies Neuromusculaires, CHU de Toulouse Purpan, Toulouse, France., Delmont E; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France., Cavalli M; Service de Neurologie: Système nerveux périphérique, Muscle et SLA, Hôpital Pasteur 2, CHU de Nice, Nice, France., Ezaru A; Service de Neurologie: Système nerveux périphérique, Muscle et SLA, Hôpital Pasteur 2, CHU de Nice, Nice, France., De La Cruz E; Service de Neurologie, CHU Gui de Chauliac, Montpellier, France., Fernandes S; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France., Kaminsky AL; Service de Neurologie, Centre Référent des Maladies Neuromusculaires Rares, CHU de Saint Etienne, Saint-Etienne, France., Kouton L; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France., Magot A; Centre de Référence Maladies Neuromusculaires AOC, Filnemus, Euro-NMD, Hôtel-Dieu, CHU de Nantes, Nantes, France., Nadaj-Pakleza A; Service de Neurologie, Centre de référence des maladies neuromusculaires Nord/Est/Ile-de-France, CHU de Strasbourg, 67000, Strasbourg, France., Tard C; Service de Neurologie, U1172, Centre de Référence des Maladies Neuromusculaires Nord/Est/Ile-de-France, CHU de Lille, Lille, France., Pegat A; Service 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., Nicolas G; Service de Neurologie, Centre de référence des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, FHU Phenix, Garches, France., Stojkovic T; Centre de référence de pathologie neuromusculaire Paris-Est, groupe hospitalier Pitié-Salpêtrière, institut de Myologie, AP-HP, Paris, France., Hogrel JY; Centre de référence de pathologie neuromusculaire Paris-Est, groupe hospitalier Pitié-Salpêtrière, institut de Myologie, AP-HP, Paris, France., Attarian S; Centre de référence des maladies neuromusculaires PACA Réunion Rhône Alpes, AP-HM, Marseille, France. shahram.attarian@ap-hm.fr.; EURO-NMD: European Reference Network for Rare Neuromuscular Diseases, Paris, France. shahram.attarian@ap-hm.fr.; Filière de Santé FILNEMUS, Paris, France. shahram.attarian@ap-hm.fr.
Πηγή:	Journal of neurology [J Neurol] 2026 Mar 02; Vol. 273 (3). Date of Electronic Publication: 2026 Mar 02.
Τύπος έκδοσης:	Journal Article; Multicenter Study; Comparative Study
Γλώσσα:	English
Στοιχεία περιοδικού:	Publisher: Springer-Verlag Country of Publication: Germany NLM ID: 0423161 Publication Model: Electronic Cited Medium: Internet ISSN: 1432-1459 (Electronic) Linking ISSN: 03405354 NLM ISO Abbreviation: J Neurol Subsets: MEDLINE
Imprint Name(s):	Original Publication: Berlin ; New York, Springer-Verlag
Ιατρικοί όροι (MeSH):	Spinal Muscular Atrophies of Childhood/physiopathology , Spinal Muscular Atrophies of Childhood/diagnosis , Action Potentials/physiology , Muscle, Skeletal/physiopathology , Motor Neurons/physiology , Motor Neurons/pathology , Recruitment, Neurophysiological*/physiology, Humans ; Male ; Female ; Adult ; Middle Aged ; Electromyography ; Reproducibility of Results ; France ; Aged ; Young Adult
Περίληψη:	Background: Spinal 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. Methods: In 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. Results: MUNIX 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. Conclusion: MUNIX 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. (© 2026. Springer-Verlag GmbH Germany, part of Springer Nature.)
Competing Interests:	Declarations. Conflicts of interest: Shahram Attarian received a research grant for this study and serves on advisory boards for Biogen and Roche; he also received honoraria and travel grants from these companies. Edouard Berling reports consulting fees from Biogen. Pascal Cintas received consulting fees, lecture honoraria, and research funding from Biogen. Jean-Yves Hogrel is the co-inventor of the MyoGrip and MyoPinch devices used in this study and has received honoraria for lecturing, consulting, and travel expenses from Biogen, Pfizer, Roche, LFB, Italfarmaco, and Lupin. Armelle Magot participated in an internal advisory board and received speaking invitations and congress support from Biogen. Aleksandra Nadaj-Pakleza received congress funding from Roche. Yann Péréon received honoraria from Biogen, Roche, and Novartis. Tanya Stojkovic received honoraria and travel grants from Biogen and Roche. Céline Tard reports personal fees and non-financial support from Roche and Biogen. Emmanuelle Salort-Campana received fees for participation on boards for Biogen and Roche and as a speaker in symposium for Biogen, Roche, and Novartis. Françoise Bouhour, Emilien Delmont, Guillaume Nicolas, Timothée Lenglet, Sara Fernandes, Elisa De La Cruz, Etienne Fortanier, Ludivine Kouton, Eva Sole-Cruz, Anne-Laure Kaminsky, Michelle Cavalli, Andra Ezaru, Sadia Beloribi-Djefaflia, and Antoine Pegat have no competing interests to declare that are relevant to the content of this article. Ethical approval: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The study was approved by the local ethics committee (Comité de Protection des Personnes (CPP) Sud-Ouest et Outre-Mer I (2020-A02618-31), reference number 1-20-079/20.01172.120079). Consent to participate: Written informed consent was obtained from all the participants.
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Contributed Indexing:	Keywords: Biomarkers; Compound muscle action potential; Electromyography; MUNIX; Spinal muscular atrophy
Entry Date(s):	Date Created: 20260302 Date Completed: 20260709 Latest Revision: 20260709
Update Code:	20260711
DOI:	10.1007/s00415-026-13714-3
PMID:	41772190
Βάση Δεδομένων:	MEDLINE

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ISSN:	1432-1459
DOI:	10.1007/s00415-026-13714-3