% IMPORTANT: The following is UTF-8 encoded.  This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.

@ARTICLE{Bjelica:281844,
      author       = {Bjelica, Bogdan and Wohnrade, Camilla and Osmanovic, Alma
                      and Schreiber-Katz, Olivia and Koerner, Sonja and Kollewe,
                      Katja and Haeckl, Sebastian and Freigang, Maren and Cordts,
                      Isabell and Becker, Benedikt and Vogt, Charlotte and
                      Muhandes, Mohamad Tareq and Günther, René and Deschauer,
                      Marcus and Koch, Jan C and Tuerk, Matthias and Regensburger,
                      Martin and Neuwirth, Christoph and Petri, Susanne},
      title        = {{C}ompound {M}uscle {A}ction {P}otential ({CMAP})
                      {A}mplitude {T}rajectories and {P}attern in {A}dults with
                      5q-{S}pinal {M}uscular {A}trophy {R}eceiving {N}usinersen
                      {T}herapy: {A} {M}ulticenter, {B}inational {O}bservational
                      {S}tudy.},
      journal      = {European journal of neurology},
      volume       = {32},
      number       = {11},
      issn         = {1351-5101},
      address      = {Oxford [u.a.]},
      publisher    = {Wiley-Blackwell},
      reportid     = {DZNE-2025-01223},
      pages        = {e70405},
      year         = {2025},
      abstract     = {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
                      $91.3\%$ sensitivity and $88.9\%$ specificity (AUC 0.96,
                      $95\%$ CI 0.92-1.0), while baseline median nerve CMAP ≥
                      6.5 mV showed $91.7\%$ sensitivity and $77.3\%$ specificity
                      (AUC 0.84, $95\%$ CI 0.72-0.96). No significant changes over
                      time were observed in median, ulnar, and peroneal CMAP
                      amplitudes (p > 0.05). CMAP trajectories did not differ
                      between SMA types 2 and 3 (p > 0.05). No significant
                      difference in the change in RULM or HFMSE at any time point
                      was observed between SMA patients with baseline median nerve
                      CMAP < 5 mV and those with CMAP of ≥ 5 mV (p > 0.05). No
                      significant correlations were found between changes in
                      median nerve CMAP and HFMSE or RULM (p > 0.05).CMAP
                      amplitudes remained stable during nusinersen treatment, with
                      no differences in trajectories between SMA types 2 and 3.
                      Our findings suggest that while CMAP amplitude correlates
                      with disease severity, it may not serve as a sensitive
                      biomarker of treatment response in adult SMA patients.},
      keywords     = {Humans / Female / Oligonucleotides: therapeutic use /
                      Oligonucleotides: pharmacology / Male / Adult / Action
                      Potentials: drug effects / Action Potentials: physiology /
                      Middle Aged / Ulnar Nerve: physiopathology / Muscular
                      Atrophy, Spinal: drug therapy / Muscular Atrophy, Spinal:
                      physiopathology / Young Adult / Median Nerve:
                      physiopathology / Spinal Muscular Atrophies of Childhood:
                      drug therapy / Spinal Muscular Atrophies of Childhood:
                      physiopathology / Peroneal Nerve: physiopathology /
                      biomarker (Other) / compound muscle action potential (CMAP)
                      (Other) / neurophysiology (Other) / nusinersen (Other) /
                      spinal muscular atrophy (SMA) (Other) / nusinersen (NLM
                      Chemicals) / Oligonucleotides (NLM Chemicals)},
      cin          = {AG Falkenburger},
      ddc          = {610},
      cid          = {I:(DE-2719)1710012},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41174958},
      pmc          = {pmc:PMC12579276},
      doi          = {10.1111/ene.70405},
      url          = {https://pub.dzne.de/record/281844},
}