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Advancing disease monitoring of amyotrophic lateral sclerosis with the compound muscle action potential scan
- Source :
- Sleutjes, B T H M, Bystrup Jacobsen, A, Tankisi, H, Gorkem Sirin, N, Emre Oge, A, Henderson, R D, van Doorn, P A, van den Berg, L H & van Eijk, R P A 2021, ' Advancing disease monitoring of amyotrophic lateral sclerosis with the compound muscle action potential scan ', Clinical Neurophysiology, vol. 132, no. 12, pp. 3152-3159 . https://doi.org/10.1016/j.clinph.2021.09.014, Clinical Neurophysiology, 132(12), 3152-3159. Elsevier Ireland Ltd
- Publication Year :
- 2021
- Publisher :
- Elsevier BV, 2021.
-
Abstract
- Objective To determine which compound muscle action potential (CMAP) scan-derived electrophysiological markers are most sensitive for monitoring disease progression in amyotrophic lateral sclerosis (ALS), and whether they hold value for clinical trials. Methods We used four independent patient cohorts to assess longitudinal patterns of a comprehensive set of electrophysiological markers including their association with the ALS functional rating scale (ALSFRS-R). Results were translated to trial sample size requirements. Results In 65 patients, 225 thenar CMAP scan recordings were obtained. Electrophysiological markers showed extensive variation in their longitudinal trajectories. Expressed as standard deviations per month, motor unit number estimation (MUNE) values declined by 0.09 (CI 0.07–0.12), D50, a measure that quantifies CMAP scan discontinuities, declined by 0.09 (CI 0.06–0.13) and maximum CMAP by 0.05 (CI 0.03–0.08). ALSFRS-R declined fastest (0.12, CI 0.08 – 0.15), however the between-patient variability was larger compared to electrophysiological markers, resulting in larger sample sizes. MUNE reduced the sample size by 19.1% (n = 388 vs n = 314) for a 6-month study compared to the ALSFRS-R. Conclusions CMAP scan-derived markers show promise in monitoring disease progression in ALS patients, where MUNE may be its most suitable derivate. Significance MUNE may increase clinical trial efficiency compared to clinical endpoints.
- Subjects :
- Adult
Male
medicine.medical_specialty
Action Potentials
Motor unit sizes
Clinical trials
Rating scale
Physiology (medical)
Internal medicine
Clinical endpoint
medicine
Humans
Motor unit number estimation
Amyotrophic lateral sclerosis
Muscle, Skeletal
Aged
Clinical Trials as Topic
Electromyography
business.industry
Amyotrophic Lateral Sclerosis
Middle Aged
Compound muscle action potential scan
medicine.disease
Sensory Systems
Compound muscle action potential
Clinical trial
Neurology
Sample size determination
Electrophysiological markers
Cardiology
Female
Neurology (clinical)
Monitoring disease progression
business
Subjects
Details
- ISSN :
- 13882457
- Volume :
- 132
- Database :
- OpenAIRE
- Journal :
- Clinical Neurophysiology
- Accession number :
- edsair.doi.dedup.....40178af5a0dbaacefd34fafd53e0debc