Hand weakness in Charcot-Marie-Tooth disease 1X

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• 作者：P.J. Arthur-Farraj ; ^{p.arthurfarraj@gmail.com} ; S.M. Murphy ^{sinead.murphy@amnch.ie} ; M. Laura ^{mlaura@ion.ucl.ac.uk} ; M.P. Lunn ^{michael.lunn@uclh.nhs.uk} ; H. Manji ^{hadi.manji@btinternet.com} ; J. Blake ^{julian.blake@nnuh.nhs.uk} ; G. Ramdharry ^{g.ramdharry@ion.ucl.ac.uk} ; Z. Fox ^{z.fox@ucl.ac.uk} ; M.M. Reilly ^{m.reilly@ion.ucl.ac.uk}
• 关键词：Charcot&ndash ; Marie&ndash ; Tooth disease ; Connexin 32 ; Hand strength ; Overwork weakness ; Neuropathy ; GJB1
• 刊名：Neuromuscular Disorders
• 出版年：2012
• 期刊代码：203_09608966
• 类别：med
• 出版时间：July, 2012
• 卷：22
• 期：7
• 页码：622-626
• 文件大小：150 K

摘要

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.