Hand weakness in Charcot-Marie-Tooth disease 1X

P J Arthur-Farraj, S M Murphy, M Laura, M P Lunn, H Manji, J Blake, G Ramdharry, Z Fox, M M Reilly, P J Arthur-Farraj, S M Murphy, M Laura, M P Lunn, H Manji, J Blake, G Ramdharry, Z Fox, M M Reilly

Abstract

There have been suggestions from previous studies that patients with Charcot-Marie-Tooth disease (CMT) have weaker dominant hand muscles. Since all studies to date have included a heterogeneous group of CMT patients we decided to analyse hand strength in 43 patients with CMT1X. We recorded handedness and the MRC scores for the first dorsal interosseous and abductor pollicis brevis muscles, median and ulnar nerve compound motor action potentials and conduction velocities in dominant and non-dominant hands. Twenty-two CMT1X patients (51%) had a weaker dominant hand; none had a stronger dominant hand. Mean MRC scores were significantly higher for first dorsal interosseous and abductor pollicis brevis in non-dominant hands compared to dominant hands. Median nerve compound motor action potentials were significantly reduced in dominant compared to non-dominant hands. We conclude that the dominant hand is weaker than the non-dominant hand in patients with CMT1X.

Copyright © 2012 Elsevier B.V. All rights reserved.

References

    1. Videler A.J., Beelen A., van Schaik I.N., de Visser M., Nollet F. Limited upper limb functioning has impact on restrictions in participation and autonomy of patients with hereditary motor and sensory neuropathy 1a. J Rehabil Med. 2009;41:746–750.
    1. Vinci P., Esposito C., Perelli S.L., Antenor J.A., Thomas F.P. Overwork weakness in Charcot–Marie–Tooth disease. Arch Phys Med Rehabil. 2003;84:825–827.
    1. van Pomeren M., Selles R.W., van Ginneken B.T., Schreuders T.A., Janssen W.G., Stam H.J. The hypothesis of overwork weakness in Charcot–Marie–Tooth: a critical evaluation. J Rehabil Med. 2009;41:32–34.
    1. Bennett R.L., Knowlton G.C. Overwork weakness in partially denervated skeletal muscle. Clin Orthop. 1958;12:22–29.
    1. Johnson E.W., Braddom R. Over-work weakness in facioscapulohumeral muscular dystrophy. Arch Phys Med Rehabil. 1971;52:333–336.
    1. Lindeman E., Leffers P., Spaans F. Strength training in patients with myotonic dystrophy and hereditary motor and sensory neuropathy: a randomized clinical trial. Arch Phys Med Rehabil. 1995;76:612–620.
    1. Chan K.M., Amirjani N., Sumrain M., Clarke A., Strohschein F.J. Randomized controlled trial of strength training in post-polio patients. Muscle Nerve. 2003;27:332–338.
    1. Garssen M.P., Bussmann J.B., Schmitz P.I. Physical training and fatigue, fitness, and quality of life in Guillain-Barre syndrome and CIDP. Neurology. 2004;63:2393–2395.
    1. Kleopa K.A., Scherer S.S. Molecular genetics of X-linked Charcot–Marie–Tooth disease. Neuromolecular Med. 2006;8:107–122.
    1. Shy M.E., Siskind C., Swan E.R. CMT1X phenotypes represent loss of GJB1 gene function. Neurology. 2007;68:849–855.
    1. Taylor R.A., Simon E.M., Marks H.G., Scherer S.S. The CNS phenotype of X-linked Charcot–Marie–Tooth disease: more than a peripheral problem. Neurology. 2003;61:1475–1478.
    1. Dubourg O., Tardieu S., Birouk N. The frequency of 17p11.2 duplication and Connexin 32 mutations in 282 Charcot–Marie–Tooth families in relation to the mode of inheritance and motor nerve conduction velocity. Neuromuscul Disord. 2001;11:458–463.
    1. Gutierrez A., England J.D., Sumner A.J. Unusual electrophysiological findings in X-linked dominant Charcot–Marie–Tooth disease. Muscle Nerve. 2000;23:182–188.
    1. Hahn A.F., Bolton C.F., White C.M. Genotype/phenotype correlations in X-linked dominant Charcot–Marie–Tooth disease. Ann N Y Acad Sci. 1999;883:366–382.
    1. Shy M.E., Blake J., Krajewski K. Reliability and validity of the CMT neuropathy score as a measure of disability. Neurology. 2005;64:1209–1214.
    1. Laird N.M., Ware J.H. Random-effects models for longitudinal data. Biometrics. 1982;38:963–974.
    1. Selles R.W., van Ginneken B.T., Schreuders T.A., Janssen W.G., Stam H.J. Dynamometry of intrinsic hand muscles in patients with Charcot–Marie–Tooth disease. Neurology. 2006;67:2022–2027.
    1. Krajewski K.M., Lewis R.A., Fuerst D.R. Neurological dysfunction and axonal degeneration in Charcot–Marie–Tooth disease type 1A. Brain. 2000;123:1516–1527.
    1. White CM, Pritchard J, Turner-Stokes L. Exercise for people with peripheral neuropathy. Cochrane Database Syst Rev 2004; CD003904.
    1. Ramdharry G.M. Rehabilitation in practice. management of lower motor neuron weakness. Clin Rehabil. 2010;24:387–397.

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