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

doi:10.1016/j.nmd.2012.02.008

P.J Arthur-Farraj, S.M Murphy + Show 7 more

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https://doi.org/10.1016/j.nmd.2012.02.008

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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.

Highlights

The degree of hand weakness in neurological diseases such as Charcot-Marie-Tooth disease (CMT) is of particular importance since it has a large impact on day to day functioning and quality of life [1]
We showed that the abductor pollicis brevis (APB) muscle was weaker than first dorsal interosseous (FDIO), confirming previous suggestions that there is a greater impact on median nerve function compared to the ulnar nerve [9,14]
One would expect that overwork would exert its effects over time so that differences should increase with age; our patient sample size was not large enough to assess the effect of patient age, or disease severity, measured by the CMT neuropathy score (CMTNS), on hand strength

Summary

Introduction

The degree of hand weakness in neurological diseases such as Charcot-Marie-Tooth disease (CMT) is of particular importance since it has a large impact on day to day functioning and quality of life [1]. A study of 124 patients suggested that patients with Charcot-Marie-Tooth disease (CMT) might be susceptible to overwork weakness when comparing the strength of dominant to non-dominant hand muscles [2]. It was used to explain apparent activity-dependent weakness that developed in patients with post-polio syndrome [4]. It has been described in patients with facioscapulohumeral muscular dystrophy [5]. Trials of exercise in patients with CMT, myotonic dystrophy, Guillain–Barresyndrome, chronic inflammatory polyradiculoneuropathy (CIDP) and even post-polio syndrome have not shown deterioration in muscle strength [6,7,8]

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