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Updated: Apr 12, 2026
In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
Published on: June 15, 2018
Electrically evoked multiplet discharges are associated with more marked clinical deterioration in motor neuron
Boudewijn T H M Sleutjes1, Ellen M Maathuis1, Pieter A van Doorn2
1Department of Clinical Neurophysiology, University Medical Centre Rotterdam, P.O. Box 2040, 3000, CA Rotterdam, The Netherlands.
Introduction:
The aim of this study was to determine whether electrically evoked multiplet discharges (MDs) are related to severity of clinical deterioration in motor neuron disease (MND).
Methods:
Stimulated high-density surface electromyographic (HDsEMG) recordings were performed in thenar muscles. Data were collected from 31 MND patients. MDs from the HDsEMG recordings were determined at baseline. ALSFRS-R scores were obtained at baseline and at a maximum of 16 weeks follow-up.
Results:
The presence of MDs was associated with progressive deterioration of ALSFRS-R score (P = 0.02) and fine motor function (FMF) (P < 0.001). Patients who had a higher number of motor units that generated MDs (r = 0.61, P < 0.001) and patients who had a higher number of MDs (as percentage of applied stimuli) (r = 0.59, P = 0.001) had a more severe decline in FMF.
Conclusions:
Electrically evoked MDs are associated with more marked clinical deterioration in patients with MND.
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