TY  - JOUR
AU  - Bjelica, Bogdan
AU  - Wohnrade, Camilla
AU  - Osmanovic, Alma
AU  - Schreiber-Katz, Olivia
AU  - Koerner, Sonja
AU  - Kollewe, Katja
AU  - Haeckl, Sebastian
AU  - Freigang, Maren
AU  - Cordts, Isabell
AU  - Becker, Benedikt
AU  - Vogt, Charlotte
AU  - Muhandes, Mohamad Tareq
AU  - Günther, René
AU  - Deschauer, Marcus
AU  - Koch, Jan C
AU  - Tuerk, Matthias
AU  - Regensburger, Martin
AU  - Neuwirth, Christoph
AU  - Petri, Susanne
TI  - Compound Muscle Action Potential (CMAP) Amplitude Trajectories and Pattern in Adults with 5q-Spinal Muscular Atrophy Receiving Nusinersen Therapy: A Multicenter, Binational Observational Study.
JO  - European journal of neurology
VL  - 32
IS  - 11
SN  - 1351-5101
CY  - Oxford [u.a.]
PB  - Wiley-Blackwell
M1  - DZNE-2025-01223
SP  - e70405
PY  - 2025
AB  - 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
KW  - Humans
KW  - Female
KW  - Oligonucleotides: therapeutic use
KW  - Oligonucleotides: pharmacology
KW  - Male
KW  - Adult
KW  - Action Potentials: drug effects
KW  - Action Potentials: physiology
KW  - Middle Aged
KW  - Ulnar Nerve: physiopathology
KW  - Muscular Atrophy, Spinal: drug therapy
KW  - Muscular Atrophy, Spinal: physiopathology
KW  - Young Adult
KW  - Median Nerve: physiopathology
KW  - Spinal Muscular Atrophies of Childhood: drug therapy
KW  - Spinal Muscular Atrophies of Childhood: physiopathology
KW  - Peroneal Nerve: physiopathology
KW  - biomarker (Other)
KW  - compound muscle action potential (CMAP) (Other)
KW  - neurophysiology (Other)
KW  - nusinersen (Other)
KW  - spinal muscular atrophy (SMA) (Other)
KW  - nusinersen (NLM Chemicals)
KW  - Oligonucleotides (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:41174958
C2  - pmc:PMC12579276
DO  - DOI:10.1111/ene.70405
UR  - https://pub.dzne.de/record/281844
ER  -