Home > Publications Database > Compound Muscle Action Potential (CMAP) Amplitude Trajectories and Pattern in Adults with 5q-Spinal Muscular Atrophy Receiving Nusinersen Therapy: A Multicenter, Binational Observational Study. > print

001     281844
005     20251117102906.0
024 7 _ |a 10.1111/ene.70405
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024 7 _ |a 1351-5101
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024 7 _ |a 1468-1331
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037 _ _ |a DZNE-2025-01223
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Bjelica, Bogdan
|0 0000-0002-1783-703X
|b 0
245 _ _ |a Compound Muscle Action Potential (CMAP) Amplitude Trajectories and Pattern in Adults with 5q-Spinal Muscular Atrophy Receiving Nusinersen Therapy: A Multicenter, Binational Observational Study.
260 _ _ |a Oxford [u.a.]
|c 2025
|b Wiley-Blackwell
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520 _ _ |a 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.
536 _ _ |a 353 - Clinical and Health Care Research (POF4-353)
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650 _ 7 |a biomarker
|2 Other
650 _ 7 |a compound muscle action potential (CMAP)
|2 Other
650 _ 7 |a neurophysiology
|2 Other
650 _ 7 |a nusinersen
|2 Other
650 _ 7 |a spinal muscular atrophy (SMA)
|2 Other
650 _ 7 |a nusinersen
|0 5Z9SP3X666
|2 NLM Chemicals
650 _ 7 |a Oligonucleotides
|2 NLM Chemicals
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Oligonucleotides: therapeutic use
|2 MeSH
650 _ 2 |a Oligonucleotides: pharmacology
|2 MeSH
650 _ 2 |a Male
|2 MeSH
650 _ 2 |a Adult
|2 MeSH
650 _ 2 |a Action Potentials: drug effects
|2 MeSH
650 _ 2 |a Action Potentials: physiology
|2 MeSH
650 _ 2 |a Middle Aged
|2 MeSH
650 _ 2 |a Ulnar Nerve: physiopathology
|2 MeSH
650 _ 2 |a Muscular Atrophy, Spinal: drug therapy
|2 MeSH
650 _ 2 |a Muscular Atrophy, Spinal: physiopathology
|2 MeSH
650 _ 2 |a Young Adult
|2 MeSH
650 _ 2 |a Median Nerve: physiopathology
|2 MeSH
650 _ 2 |a Spinal Muscular Atrophies of Childhood: drug therapy
|2 MeSH
650 _ 2 |a Spinal Muscular Atrophies of Childhood: physiopathology
|2 MeSH
650 _ 2 |a Peroneal Nerve: physiopathology
|2 MeSH
700 1 _ |a Wohnrade, Camilla
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700 1 _ |a Osmanovic, Alma
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700 1 _ |a Schreiber-Katz, Olivia
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700 1 _ |a Koerner, Sonja
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700 1 _ |a Haeckl, Sebastian
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700 1 _ |a Freigang, Maren
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700 1 _ |a Cordts, Isabell
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700 1 _ |a Becker, Benedikt
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700 1 _ |a Vogt, Charlotte
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700 1 _ |a Muhandes, Mohamad Tareq
|0 0009-0000-4166-2746
|b 11
700 1 _ |a Günther, René
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700 1 _ |a Deschauer, Marcus
|0 0000-0001-6116-6790
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700 1 _ |a Koch, Jan C
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700 1 _ |a Tuerk, Matthias
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700 1 _ |a Regensburger, Martin
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700 1 _ |a Neuwirth, Christoph
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700 1 _ |a Petri, Susanne
|0 0000-0002-9783-8584
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773 _ _ |a 10.1111/ene.70405
|g Vol. 32, no. 11, p. e70405
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|t European journal of neurology
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