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000893880 1001_ $$0P:(DE-Juel1)158077$$aSchneider, Christian$$b0
000893880 245__ $$aMotor unit number estimation in adult patients with spinal muscular atrophy treated with nusinersen
000893880 260__ $$aOxford$$bBlackwell Science$$c2021
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000893880 520__ $$aBackground 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.
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000893880 7001_ $$0P:(DE-HGF)0$$aWassermann, Meike K.$$b1
000893880 7001_ $$0P:(DE-HGF)0$$aGrether, Nicolai B.$$b2
000893880 7001_ $$0P:(DE-Juel1)131720$$aFink, Gereon R.$$b3
000893880 7001_ $$0P:(DE-HGF)0$$aWunderlich, Gilbert$$b4
000893880 7001_ $$0P:(DE-HGF)0$$aLehmann, Helmar C.$$b5$$eCorresponding author
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000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1002/mus.26304
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/j.clinph.2019.04.713
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/j.clinph.2020.01.018
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1080/146608200300079536
000893880 999C5 $$1Jacobsen AB$$2Crossref$$oJacobsen AB 2018$$y2018
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1002/mus.24945
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/j.clinph.2013.07.016
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.3109/21678421.2012.732079
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/j.clinph.2017.03.045
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/j.nmd.2007.05.009
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1177/1756286420907803
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.3233/JND-190416
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1212/WNL.0000000000009914
000893880 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1136/jnnp-2020-324254