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

Publication date: July 2018
Source:Clinical Neurophysiology, Volume 129, Issue 7
Author(s): Joachim Bas, Emilien Delmont, Farzad Fatehi, Emmanuelle Salort-Campana, Annie Verschueren, Jean Pouget, Marie-Noëlle Lefebvre, Aude-Marie Grapperon, Shahram Attarian
ObjectiveThe 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.MethodsMUNIX 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. Disability was evaluated using several functional scales, including CMT neuropathy score version 2 (CMTNSv2) and overall neuropathy limitation scale (ONLS).ResultsA 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).ConclusionMUNIX correlates with muscle strength and clinical measurements of disability in patients with CMT disease.SignificanceThe 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.



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