%0 Journal Article
%A Bjelica, Bogdan
%A Wohnrade, Camilla
%A Osmanovic, Alma
%A Schreiber-Katz, Olivia
%A Koerner, Sonja
%A Kollewe, Katja
%A Haeckl, Sebastian
%A Freigang, Maren
%A Cordts, Isabell
%A Becker, Benedikt
%A Vogt, Charlotte
%A Muhandes, Mohamad Tareq
%A Günther, René
%A Deschauer, Marcus
%A Koch, Jan C
%A Tuerk, Matthias
%A Regensburger, Martin
%A Neuwirth, Christoph
%A Petri, Susanne
%T Compound Muscle Action Potential (CMAP) Amplitude Trajectories and Pattern in Adults with 5q-Spinal Muscular Atrophy Receiving Nusinersen Therapy: A Multicenter, Binational Observational Study.
%J European journal of neurology
%V 32
%N 11
%@ 1351-5101
%C Oxford [u.a.]
%I Wiley-Blackwell
%M DZNE-2025-01223
%P e70405
%D 2025
%X This study aimed to evaluate changes in compound muscle action potential (CMAP) amplitude in adults with spinal muscular atrophy (SMA) undergoing nusinersen treatment and its association with motor function improvements.This multicenter study assessed median, ulnar, and peroneal CMAP over a follow-up of up to 4.5 years using linear mixed models. Motor function was measured using the Revised Upper Limb Module (RULM) and the Hammersmith Functional Motor Scale Expanded (HFMSE). Correlations between CMAP and motor function scores were analyzed.Seventy-eight patients (27 ambulatory, 51 non-ambulatory) were included. Baseline ulnar CMAP ≥ 2.0 mV distinguished SMA type 3 from type 2 with 91.3
%K Humans
%K Female
%K Oligonucleotides: therapeutic use
%K Oligonucleotides: pharmacology
%K Male
%K Adult
%K Action Potentials: drug effects
%K Action Potentials: physiology
%K Middle Aged
%K Ulnar Nerve: physiopathology
%K Muscular Atrophy, Spinal: drug therapy
%K Muscular Atrophy, Spinal: physiopathology
%K Young Adult
%K Median Nerve: physiopathology
%K Spinal Muscular Atrophies of Childhood: drug therapy
%K Spinal Muscular Atrophies of Childhood: physiopathology
%K Peroneal Nerve: physiopathology
%K biomarker (Other)
%K compound muscle action potential (CMAP) (Other)
%K neurophysiology (Other)
%K nusinersen (Other)
%K spinal muscular atrophy (SMA) (Other)
%K nusinersen (NLM Chemicals)
%K Oligonucleotides (NLM Chemicals)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:41174958
%2 pmc:PMC12579276
%R 10.1111/ene.70405
%U https://pub.dzne.de/record/281844