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Motor unit number index correlates with disability in Charcot-Marie-Tooth disease

Joachim Bas Emilien Delmont Farzad Fatehi Emmanuelle Salort-Campana 1, 2 Annie Verschueren Jean Pouget 1, 2 Marie-Noelle Lefebvre Aude-Marie Grapperon Shahram Attarian 1
1 MMG - Marseille medical genetics - Centre de génétique médicale de Marseille
2 FILNEMUS - Filière Neuromusculaire
Abstract : Objective: The aim of this study was to assess the usefulness of motor unit number index (MUNIX) tech-nique 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 min-imi (ADM) and the tibialis anterior (TA) muscles in the nondominant side. A MUNIX sum score was cal-culated by adding the MUNIX of these 3 muscles. Muscle strength was measured using the MRC (medical research council) scale. Disability was evaluated using several functional scales, including CMT neuropa-thy score version 2 (CMTNSv2) and overall neuropathy limitation scale (ONLS). Results: A total of 56 CMT patients were enrolled. The MUNIX scores of the ADM, APB and TA muscles correlated with the MRC score of the corresponding muscle (p < 0.01). The MUNIX sum score correlated with the clinical scales CMTNSv2 (r = -0.65, p < 0.01) and ONLS (r = -0.57, p < 0.01). Conclusion: MUNIX correlates with muscle strength and clinical measurements of disability in patients with CMT disease. Significance: The MUNIX technique evaluates motor axonal loss and correlates with disability. The MUNIX sum score may be a useful outcome measure of disease progression in CMT. (C) 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.
Keywords : Charcot-Marie-Tooth CMT CMT1A Neuropathy MUNIX Outcome
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Submitted on : Wednesday, January 23, 2019 - 6:00:39 PM
Last modification on : Thursday, February 7, 2019 - 3:41:11 PM

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Joachim Bas, Emilien Delmont, Farzad Fatehi, Emmanuelle Salort-Campana, Annie Verschueren, et al.. Motor unit number index correlates with disability in Charcot-Marie-Tooth disease. CLINICAL NEUROPHYSIOLOGY, 2018, 129 (7), pp.1390-1396. ⟨hal-01991355⟩

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