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Farzad Fatehi, Aude-Marie Grapperon, Davood Fathi, Emilien Delmont, Shahram Attarian
The need for a valid biomarker for assessing disease progression and for use in clinical trials on amyotrophic lateral sclerosis (ALS) has stimulated the study of methods that could measure the number of motor units. Motor unit number index (MUNIX) is a newly developed neurophysiological technique that was demonstrated to have a good correlation with the number of motor units in a given muscle, even though it does not necessarily accurately express the actual number of viable motor neurons. Several studies demonstrated the technique is reproducible and capable of following motor neuron loss in patients with ALS and peripheral polyneuropathies...
October 1, 2018: Neurophysiologie Clinique, Clinical Neurophysiology
Giorgia Querin, Timothée Lenglet, Rabab Debs, Tanya Stojkovic, Anthony Behin, François Salachas, Nadine Le Forestier, Maria Del Mar Amador, Lucette Lacomblez, Vincent Meininger, Gaelle Bruneteau, Pascal Laforêt, Sophie Blancho, Véronique Marchand-Pauvert, Peter Bede, Jean-Yves Hogrel, Pierre-François Pradat
OBJECTIVE: Objective of this study is the comprehensive characterisation of motor unit (MU) loss in type III and IV Spinal Muscular Atrophy (SMA) using motor unit number index (MUNIX), and evaluation of compensatory mechanisms based on MU size indices (MUSIX). METHODS: Nineteen type III and IV SMA patients and 16 gender- and age-matched healthy controls were recruited. Neuromuscular performance was evaluated by muscle strength testing and functional scales. Compound motor action potential (CMAP), MUNIX and MUSIX were studied in the abductor pollicis brevis (APB), abductor digiti minimi (ADM), deltoid, tibialis anterior and trapezius muscles...
September 13, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Serkan Uslu, Tunca Nüzket, Hilmi Uysal
Objective: The understanding of the spasticity mechanism is still a problem in the literature, as its definition can be made on the basis of more than one parameter. Therefore, we studied alpha motor neuron excitability, dynamic changes based on force production, and patellar tendon (T) reflex in spasticity and healthy control groups. Methods: Alpha motor neuron excitability, force production, and patellar T reflex were evaluated through three different test protocols...
2018: Clinical Neurophysiology Practice
Mana Higashihara, Parvathi Menon, Mehdi van den Bos, Nimeshan Geevasinga, Steve Vucic
INTRODUCTION: Reproducibility of the multiple point stimulation motor unit number estimation (MPS-MUNE) technique was compared to the recently developed motor unit number index (MUNIX) technique. METHODS: MPS-MUNE and MUNIX were performed on 15 healthy subjects at three different time points by the same examiner. Reproducibility was analyzed using intraclass correlation coefficient (ICC) and coefficient variation (CV). RESULTS: ICC values for MUNIX and MPS-MUNE were excellent across three tests (0...
September 8, 2018: Muscle & Nerve
Anna M Wirth, Andrei Khomenko, Dobri Baldaranov, Ines Kobor, Ohnmar Hsam, Thomas Grimm, Siw Johannesen, Tim-Henrik Bruun, Wilhelm Schulte-Mattler, Mark W Greenlee, Ulrich Bogdahn
Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative process affecting upper and lower motor neurons as well as non-motor systems. In this study, precentral and postcentral cortical thinning detected by structural magnetic resonance imaging (MRI) were combined with clinical (ALS-specific functional rating scale revised, ALSFRS-R) and neurophysiological (motor unit number index, MUNIX) biomarkers in both cross-sectional and longitudinal analyses. Methods: The unicenter sample included 20 limb-onset classical ALS patients compared to 30 age-related healthy controls...
2018: Frontiers in Neurology
Anna Bystrup Jacobsen, Hugh Bostock, Hatice Tankisi
INTRODUCTION: The objective of this study was to evaluate a recently developed motor unit number estimation (MUNE) method, MScanFit MUNE (MScan), as a measure of disease progression in amyotrophic lateral sclerosis (ALS) compared to compound muscle action potential (CMAP) amplitude and two traditional MUNE methods. METHODS: ALS patients were evaluated clinically by ALS Functional Rating Scale-Revised (ALSFRS-R). MScan, multiple point stimulation MUNE (MPS) and motor unit number index (MUNIX) were performed in the abductor pollicis brevis (APB) muscle at baseline (27 patients), 4 months (23 patients) and 8 months (16 patients)...
July 19, 2018: Muscle & Nerve
Paul E Barkhaus
No abstract text is available yet for this article.
August 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Mamede de Carvalho, Paul E Barkhaus, Sanjeev D Nandedkar, Michael Swash
Estimation of the number of motor units (MUNE) in specific muscles is important to monitor outcome in progressive neurogenic disorders, with potential application in clinical trials. However, in spite of recent developments to identify the most convenient technique for MUNE, all current methods have individual shortcomings. It is essential to understand the scientific concepts that support MUNE and the many methods already proposed. In particular, the core role of the compound muscle action potential (CMAP) size in the estimation process is undervalued...
August 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Christoph Neuwirth, Nathalie Braun, Kristl G Claeys, Robert Bucelli, Christina Fournier, Mark Bromberg, Susanne Petri, Stephan Goedee, Timothée Lenglet, Ron Leppanen, Antonio Canosa, Ira Goodman, Muhammad Al-Lozi, Takuya Ohkubo, Annemarie Hübers, Nazem Atassi, Agessandro Abrahao, Andreas Funke, Martin Appelfeller, Anke Tümmler, Eoin Finegan, Jonathan D Glass, Suma Babu, Shafeeq S Ladha, Olga Kwast-Rabben, Raul Juntas-Morales, Amina Coffey, Vinay Chaudhry, Tuan Vu, Chow Saephanh, Colleen Newhard, Marion Zakrzewski, Esther Rosier, Nancy Hamel, Divisha Raheja, Jesper Raaijman, Toby Ferguson, Markus Weber
OBJECTIVE: Motor Unit Number Index (MUNIX) is a quantitative neurophysiological method that reflects loss of motor neurons in Amyotrophic Lateral Sclerosis (ALS) in longitudinal studies. It has been utilized in one natural history ALS study and one drug trial (Biogen USA) after training and qualification of raters. METHODS: Prior to testing patients, evaluators had to submit test-retest data of 4 healthy volunteers. Twenty-seven centres with 36 raters measured MUNIX in 4 sets of 6 different muscles twice...
August 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Clifton L Gooch
No abstract text is available yet for this article.
September 2018: Muscle & Nerve
Joachim Bas, Emilien Delmont, Farzad Fatehi, Emmanuelle Salort-Campana, Annie Verschueren, Jean Pouget, Marie-Noëlle Lefebvre, Aude-Marie Grapperon, Shahram Attarian
OBJECTIVE: The aim of this study was to assess the usefulness of motor unit number index (MUNIX) technique in Charcot-Marie-Tooth disease and test the correlation between MUNIX and clinical impairment. METHODS: MUNIX technique was performed in the abductor pollicis brevis (APB), the abductor digiti minimi (ADM) and the tibialis anterior (TA) muscles in the nondominant side. A MUNIX sum score was calculated by adding the MUNIX of these 3 muscles. Muscle strength was measured using the MRC (medical research council) scale...
July 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Sanjeev D Nandedkar, Paul E Barkhaus, Erik V Stålberg, Christoph Neuwirth, Markus Weber
INTRODUCTION: This study proposes guidelines for motor unit number index (MUNIX) recording and analysis. METHODS: MUNIX was measured in control participants and in patients with amyotrophic lateral sclerosis. Changes in MUNIX values due to E1 electrode position, number of surface electromyography interference pattern (SIP) epochs, SIP epoch duration, force of contraction, and outlier data points were investigated. RESULTS: MUNIX depends on optimized compound muscle action potential (CMAP) amplitude...
September 2018: Muscle & Nerve
Francesc Miralles
OBJECTIVE: The motor unit number index (MUNIX) is a technique based on the surface electromyogram (sEMG) that is gaining acceptance as a method for monitoring motor neuron loss, because it is reliable and produces less discomfort than other electrodiagnostic techniques having the same intended purpose. MUNIX assumes that the relationship between the area of sEMG obtained at increasing levels of muscle activation and the values of a variable called 'ideal case motor unit count' (ICMUC), defined as the product of the ratio between area and power of the compound muscle action potential (CMAP) by that of the sEMG, is described by a decreasing power function...
June 2018: Journal of Neural Engineering
A B Jacobsen, R S Kristensen, A Witt, A G Kristensen, L Duez, S Beniczky, A Fuglsang-Frederiksen, H Tankisi
OBJECTIVE: To compare the diagnostic utility of motor unit number estimation (MUNE) methods to motor unit potential (MUP) analysis in amyotrophic lateral sclerosis (ALS). METHODS: Twenty-five patients (1 definite, 11 probable, 9 possible ALS and 4 progressive muscular atrophy) and 22 healthy controls were prospectively included. Quantitative MUP analysis and three MUNE methods; Multiple Point Stimulation MUNE (MPS), Motor Unit Number Index (MUNIX) and MScanFit MUNE (MScan) were done in abductor pollicis brevis muscle...
March 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Marcio Luiz Escorcio-Bezerra, Agessandro Abrahao, Karlo Faria Nunes, Nadia Iandoli De Oliveira Braga, Acary Souza Bulle Oliveira, Lorne Zinman, Gilberto Mastrocola Manzano
INTRODUCTION: Our objective was to determine the utility of motor unit number index (MUNIX) and neurophysiological index (NI) as surrogate biomarkers of disease progression in limbs without clinical signs of lower motor neuron (LMN) involvement from patients with slowly progressive amyotrophic lateral sclerosis (ALS). METHODS: Patients with slowly progressive ALS and at least 1 clinically unaffected limb were prospectively enrolled. Clinical signs of LMN loss and results from hand-held dynamometer (HHD), revised ALS Functional Rating Scale (ALSFRS-R), mean-MUNIX (from 3 different muscles), and NI were longitudinally recorded...
August 2018: Muscle & Nerve
Karim Benmouna, Christophe Milants, François Charles Wang
OBJECTIVE: The aim of this study was to evaluate how the motor unit number index (MUNIX) is related to the adapted multiple point stimulation (AMPS) technique. METHODS: MUNIX and AMPS technique were prospectively performed on thenar muscles in 20 consecutive patients referred to our neurophysiological laboratory with the clinical diagnosis of a possible motoneurone disorder (MND). The clinical and paraclinical assessment confirmed the diagnosis of MND in 13 out of 20 patients, amyotrophic lateral sclerosis (ALS) in 9 (with MND group)...
November 26, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Marcio Luiz Escorcio-Bezerra, Agessandro Abrahao, Denizart Santos-Neto, Nadia Iandoli de Oliveira Braga, Acary Souza Bulle Oliveira, Gilberto Mastrocola Manzano
OBJECTIVE: To assess the impact of averaging multiple MUNIX trials on the follow-up of patients with amyotrophic lateral sclerosis (ALS). METHODS: We determined the percent relative change (%RC) of MUNIX, in healthy subjects and patients with ALS, by subtracting the MUNIX value in the second visit from the first. Both the mean of a set of three MUNIX (mean-MUNIX) and the first MUNIX sample (single-MUNIX) were evaluated. Then, we studied the sensitivity to detect relative changes over time and the statistical dispersion of the %RC from these two parameters...
December 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Thomas M Jenkins, James J P Alix, Charlotte David, Eilish Pearson, D Ganesh Rao, Nigel Hoggard, Eoghan O'Brien, Kathleen Baster, Michael Bradburn, Julia Bigley, Christopher J McDermott, Iain D Wilkinson, Pamela J Shaw
OBJECTIVE: To assess clinical, electrophysiological and whole-body muscle MRI measurements of progression in patients with motor neuron disease (MND), as tools for future clinical trials, and to probe pathophysiological mechanisms in vivo. METHODS: A prospective, longitudinal, observational, clinicoelectrophysiological and radiological cohort study was performed. Twenty-nine patients with MND and 22 age-matched and gender-matched healthy controls were assessed with clinical measures, electrophysiological motor unit number index (MUNIX) and T2-weighted whole-body muscle MRI, at first clinical presentation and 4 months later...
March 2018: Journal of Neurology, Neurosurgery, and Psychiatry
Farzad Fatehi, Emilien Delmont, Aude-Marie Grapperon, Emmanuelle Salort-Campana, Amandine Sévy, Annie Verschueren, José Boucraut, Shahram Attarian
OBJECTIVE: To investigate the relationship between Motor Unit Number Index (MUNIX) and functional scales in patients with anti-Myelin Associated Glycoprotein (MAG) neuropathy and to know if MUNIX is modify after rituximab (RTX) therapy. METHODS: 17 patients were enrolled, of whom 6 were prospectively evaluated during one year after RTX treatment. MUNIX technique was assessed in abductor digiti mini (ADM), abductor pollicis brevi (APB) and tibialis anterior (TA) muscles...
July 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Yun Peng, Yingchun Zhang
OBJECTIVE: To evaluate the repeatability of (Motor Unit Number Index) MUNIX under repeatability conditions, specify the origin of variations and provide strategies for quality control. METHODS: MUNIX calculations were performed on the bicep brachii muscles of eight healthy subjects. Negative effect of suboptimal electrode positions on MUNIX accuracy was eliminated by employing the high-density surface electromyography technique. MUNIX procedures that utilized a variety of surface interferential pattern (SIP) epoch recruitment strategies (including the original MUNIX procedure, two proposed improvement strategies and their combinations) were described...
July 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
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