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@ARTICLE{Schneider:893880,
      author       = {Schneider, Christian and Wassermann, Meike K. and Grether,
                      Nicolai B. and Fink, Gereon R. and Wunderlich, Gilbert and
                      Lehmann, Helmar C.},
      title        = {{M}otor unit number estimation in adult patients with
                      spinal muscular atrophy treated with nusinersen},
      journal      = {European journal of neurology},
      volume       = {28},
      number       = {9},
      issn         = {1351-5101},
      address      = {Oxford},
      publisher    = {Blackwell Science},
      reportid     = {FZJ-2021-02892},
      pages        = {3022-3029},
      year         = {2021},
      abstract     = {Background and purposeThe aim was to assess the
                      organization and short-term changes of motor units in adult
                      patients with spinal muscular atrophy (SMA) treated with
                      nusinersen.MethodsIn this single-centre cross-sectional and
                      longitudinal study 15 adult patients with SMA type 3 were
                      assessed and compared to 15 age-matched healthy controls and
                      nine patients with amyotrophic lateral sclerosis. Moreover,
                      10 patients with SMA were followed up after 4–8 months.
                      All patients were investigated clinically and by the motor
                      unit number estimation method MScanFit of the abductor
                      pollicis brevis muscle.ResultsThe number of motor units (p <
                      0.001) was significantly lower in patients with SMA compared
                      to healthy controls at study entry. Mean unit amplitude,
                      median amplitude and largest unit (p < 0.001) were
                      significantly increased in patients with SMA. Patients with
                      amyotrophic lateral sclerosis showed a significant reduction
                      of compound muscle action potential (p = 0.005) and number
                      of motor units (p = 0.03) compared to patients with SMA,
                      accompanied by a larger median amplitude (p = 0.03). A
                      prospective analysis identified patients with the ability to
                      walk to improve the number of motor units (p = 0.046)
                      accompanied by a decreased median amplitude (p = 0.03).
                      Electrophysiological measures showed a moderate to strong
                      correlation with clinical scores.ConclusionPatients with SMA
                      show loss of motor units in distal muscles. MScanFit
                      variables indicate that compound muscle action potential
                      amplitudes are maintained by collateral sprouting.
                      Prospective analyses suggest that milder affected adult
                      patients with SMA preferentially benefit from nusinersen
                      treatment through recovery of smaller motor units.
                      Correlations with clinical scores underline the potential of
                      MScanFit as a surrogate marker.},
      cin          = {INM-3},
      ddc          = {610},
      cid          = {I:(DE-Juel1)INM-3-20090406},
      pnm          = {5251 - Multilevel Brain Organization and Variability
                      (POF4-525)},
      pid          = {G:(DE-HGF)POF4-5251},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:34216082},
      UT           = {WOS:000675075100001},
      doi          = {10.1111/ene.15005},
      url          = {https://juser.fz-juelich.de/record/893880},
}