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Electrically evoked multiplet discharges are associated with more marked clinical deterioration in motor neuron disease
Authors:Boudewijn T.H.M. Sleutjes MSc  PhDEng  Ellen M. Maathuis MD  Pieter A. van Doorn MD  PhD  Joleen H. Blok PhD  Gerhard H. Visser MD  PhD
Affiliation:1. Department of Clinical Neurophysiology, University Medical Centre Rotterdam, CA Rotterdam, The Netherlands;2. Department of Neurology, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands;3. Department of Clinical Physics, Máxima Medical Centre Veldhoven, Veldhoven, The Netherlands;4. Department of Clinical Neurophysiology, SEIN Heemstede, Heemstede, The Netherlands
Abstract: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. Muscle Nerve 53: 222–226, 2016
Keywords:disease progression  ectopic motor unit activity  EMG  MND  multiplet discharges
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