Could the motor unit number index be an early prognostic biomarker for amyotrophic lateral sclerosis?

Autor: Grapperon AM; APHM, Timone University Hospital, Referral Center for Neuromuscular Diseases and ALS, ERN Euro-NMD Center, Marseille, France. Electronic address: aude-marie.grapperon@ap-hm.fr., Harlay V; APHM, Timone University Hospital, Referral Center for Neuromuscular Diseases and ALS, ERN Euro-NMD Center, Marseille, France., Boucekine M; Aix Marseille University, Center for Studies and Research on Health Services and Quality of Life, Marseille, France., Devos D; Department of Medical Pharmacology, Expert Center of ALS Lille University, INSERM UMRS_1772, Lille University Hospital, LICEND COEN Centre, LilNCog - Lille Neuroscience & Cognition, 59000, ACT4ALS-MND Network, France., Rolland AS; Department of Medical Pharmacology, Expert Center of ALS Lille University, INSERM UMRS_1772, Lille University Hospital, LICEND COEN Centre, LilNCog - Lille Neuroscience & Cognition, 59000, ACT4ALS-MND Network, France., Desnuelle C; Côte d'Azur University, Medical Faculty of Nice, Department of Neurology, Nice, France., Delmont E; APHM, Timone University Hospital, Referral Center for Neuromuscular Diseases and ALS, ERN Euro-NMD Center, Marseille, France., Verschueren A; APHM, Timone University Hospital, Referral Center for Neuromuscular Diseases and ALS, ERN Euro-NMD Center, Marseille, France., Attarian S; APHM, Timone University Hospital, Referral Center for Neuromuscular Diseases and ALS, ERN Euro-NMD Center, Marseille, France; Aix Marseille University, Inserm, GMGF, Marseille, France.
Jazyk: angličtina
Zdroj: Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology [Clin Neurophysiol] 2024 Jul; Vol. 163, pp. 47-55. Date of Electronic Publication: 2024 Apr 26.
DOI: 10.1016/j.clinph.2024.04.013
Abstrakt: Objective: To evaluate the associations between motor unit number index (MUNIX) and disease progression and prognosis in amyotrophic lateral sclerosis (ALS) in a large-scale longitudinal study.
Methods: MUNIX was performed at the patient's first visit, at 3, 6, and 12 months in 4 muscles. MUNIX data from the patients were compared with those from 38 age-matched healthy controls. Clinical data included the revised ALS functional rating scale (ALSFRS-R), the forced vital capacity (FVC), and the survival of the patients.
Results: Eighty-two patients were included at baseline, 62 were evaluated at three months, 48 at six months, and 33 at twelve months. MUNIX score was lower in ALS patients compared to controls. At baseline, MUNIX was correlated with ALSFRS-R and FVC. Motor unit size index (MUSIX) was correlated with patient survival. Longitudinal analyses showed that MUNIX decline was greater than ALSFRS-R decline at each evaluation. A baseline MUNIX score greater than 378 predicted survival over the 12-month period with a sensitivity of 82% and a specificity of 56%.
Conclusions: This longitudinal study suggests that MUNIX could be an early quantitative marker of disease progression and prognosis in ALS.
Significance: MUNIX might be considered as potential indicator for monitoring disease progression.
(Copyright © 2024. Published by Elsevier B.V.)
Databáze: MEDLINE
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