title: Hand weakness in Charcot-Marie-Tooth disease 1X.
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.
authors.affiliation: MRC Centre for Neuromuscular Diseases, Department of Molecular Neuroscience, UCL Institute of Neurology and the National Hospital for Neurology and Neurosurgery, Queen Square, London, UK. p.arthurfarraj@gmail.com
authors.forename: P J
authors.initials: PJ
authors.lastname: Arthur-Farraj
authors.forename: S M
authors.initials: SM
authors.lastname: Murphy
authors.forename: M
authors.initials: M
authors.lastname: Laura
authors.forename: M P
authors.initials: MP
authors.lastname: Lunn
authors.forename: H
authors.initials: H
authors.lastname: Manji
authors.forename: J
authors.initials: J
authors.lastname: Blake
authors.forename: G
authors.initials: G
authors.lastname: Ramdharry
authors.forename: Z
authors.initials: Z
authors.lastname: Fox
authors.forename: M M
authors.initials: MM
authors.lastname: Reilly
mesh_terms: D000328:Adult; D002607:Charcot-Marie-Tooth Disease / Q000150:complications* / Q000235:genetics / Q000473:pathology; D017630:Connexins / Q000235:genetics; D004185:Disability Evaluation; D004576:Electromyography; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006225:Hand / Q000294:innervation / Q000503:physiopathology*; D018737:Hand Strength / Q000502:physiology*; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology*; D009154:Mutation / Q000235:genetics; D009431:Neural Conduction / Q000235:genetics / Q000502:physiology; D012189:Retrospective Studies; D012737:Sex Factors; D014459:Ulnar Nerve / Q000503:physiopathology; D000097002:Gap Junction beta-1 Protein
chemical_list: D017630:Connexins; D000097002:Gap Junction beta-1 Protein