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000281844 037__ $$aDZNE-2025-01223
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000281844 1001_ $$00000-0002-1783-703X$$aBjelica, Bogdan$$b0
000281844 245__ $$aCompound Muscle Action Potential (CMAP) Amplitude Trajectories and Pattern in Adults with 5q-Spinal Muscular Atrophy Receiving Nusinersen Therapy: A Multicenter, Binational Observational Study.
000281844 260__ $$aOxford [u.a.]$$bWiley-Blackwell$$c2025
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000281844 520__ $$aThis study aimed to evaluate changes in compound muscle action potential (CMAP) amplitude in adults with spinal muscular atrophy (SMA) undergoing nusinersen treatment and its association with motor function improvements.This multicenter study assessed median, ulnar, and peroneal CMAP over a follow-up of up to 4.5 years using linear mixed models. Motor function was measured using the Revised Upper Limb Module (RULM) and the Hammersmith Functional Motor Scale Expanded (HFMSE). Correlations between CMAP and motor function scores were analyzed.Seventy-eight patients (27 ambulatory, 51 non-ambulatory) were included. Baseline ulnar CMAP ≥ 2.0 mV distinguished SMA type 3 from type 2 with 91.3% sensitivity and 88.9% specificity (AUC 0.96, 95% CI 0.92-1.0), while baseline median nerve CMAP ≥ 6.5 mV showed 91.7% sensitivity and 77.3% specificity (AUC 0.84, 95% CI 0.72-0.96). No significant changes over time were observed in median, ulnar, and peroneal CMAP amplitudes (p > 0.05). CMAP trajectories did not differ between SMA types 2 and 3 (p > 0.05). No significant difference in the change in RULM or HFMSE at any time point was observed between SMA patients with baseline median nerve CMAP < 5 mV and those with CMAP of ≥ 5 mV (p > 0.05). No significant correlations were found between changes in median nerve CMAP and HFMSE or RULM (p > 0.05).CMAP amplitudes remained stable during nusinersen treatment, with no differences in trajectories between SMA types 2 and 3. Our findings suggest that while CMAP amplitude correlates with disease severity, it may not serve as a sensitive biomarker of treatment response in adult SMA patients.
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000281844 650_7 $$2Other$$abiomarker
000281844 650_7 $$2Other$$acompound muscle action potential (CMAP)
000281844 650_7 $$2Other$$aneurophysiology
000281844 650_7 $$2Other$$anusinersen
000281844 650_7 $$2Other$$aspinal muscular atrophy (SMA)
000281844 650_7 $$05Z9SP3X666$$2NLM Chemicals$$anusinersen
000281844 650_7 $$2NLM Chemicals$$aOligonucleotides
000281844 650_2 $$2MeSH$$aHumans
000281844 650_2 $$2MeSH$$aFemale
000281844 650_2 $$2MeSH$$aOligonucleotides: therapeutic use
000281844 650_2 $$2MeSH$$aOligonucleotides: pharmacology
000281844 650_2 $$2MeSH$$aMale
000281844 650_2 $$2MeSH$$aAdult
000281844 650_2 $$2MeSH$$aAction Potentials: drug effects
000281844 650_2 $$2MeSH$$aAction Potentials: physiology
000281844 650_2 $$2MeSH$$aMiddle Aged
000281844 650_2 $$2MeSH$$aUlnar Nerve: physiopathology
000281844 650_2 $$2MeSH$$aMuscular Atrophy, Spinal: drug therapy
000281844 650_2 $$2MeSH$$aMuscular Atrophy, Spinal: physiopathology
000281844 650_2 $$2MeSH$$aYoung Adult
000281844 650_2 $$2MeSH$$aMedian Nerve: physiopathology
000281844 650_2 $$2MeSH$$aSpinal Muscular Atrophies of Childhood: drug therapy
000281844 650_2 $$2MeSH$$aSpinal Muscular Atrophies of Childhood: physiopathology
000281844 650_2 $$2MeSH$$aPeroneal Nerve: physiopathology
000281844 7001_ $$00000-0003-3035-4638$$aWohnrade, Camilla$$b1
000281844 7001_ $$00000-0002-6012-8423$$aOsmanovic, Alma$$b2
000281844 7001_ $$00000-0001-5621-4487$$aSchreiber-Katz, Olivia$$b3
000281844 7001_ $$aKoerner, Sonja$$b4
000281844 7001_ $$aKollewe, Katja$$b5
000281844 7001_ $$00000-0002-9806-0495$$aHaeckl, Sebastian$$b6
000281844 7001_ $$00000-0002-0941-6075$$aFreigang, Maren$$b7
000281844 7001_ $$00000-0002-2078-1997$$aCordts, Isabell$$b8
000281844 7001_ $$00009-0001-9459-4991$$aBecker, Benedikt$$b9
000281844 7001_ $$00009-0000-8347-0259$$aVogt, Charlotte$$b10
000281844 7001_ $$00009-0000-4166-2746$$aMuhandes, Mohamad Tareq$$b11
000281844 7001_ $$0P:(DE-2719)2811849$$aGünther, René$$b12
000281844 7001_ $$00000-0001-6116-6790$$aDeschauer, Marcus$$b13
000281844 7001_ $$aKoch, Jan C$$b14
000281844 7001_ $$00000-0001-9812-3794$$aTuerk, Matthias$$b15
000281844 7001_ $$00000-0002-2172-7386$$aRegensburger, Martin$$b16
000281844 7001_ $$aNeuwirth, Christoph$$b17
000281844 7001_ $$00000-0002-9783-8584$$aPetri, Susanne$$b18
000281844 773__ $$0PERI:(DE-600)2020241-6$$a10.1111/ene.70405$$gVol. 32, no. 11, p. e70405$$n11$$pe70405$$tEuropean journal of neurology$$v32$$x1351-5101$$y2025
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