Relationship between physical performance and quality of life in Charcot-Marie-Tooth disease

a pilot study

Daniel Roberts-Clarke, Che Fornusek, Nidhi Saigal, Mark Halaki, Joshua Burns, Garth Nicholson, Maria Fiatarone Singh, Daniel Hackett*

*Corresponding author for this work

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Charcot-Marie-Tooth (CMT) is a rare inherited peripheral neuropathy in which quality of life (QoL) is reduced compared with the general population. This paper investigates the relationship between QoL and physical performance in people with CMT with the aim of identifying avenues for future research into rehabilitation strategies. Cross-sectional data was obtained from 10 participants (5 men, 5 women, age 46 ± 13 years, height 1.7 ± 0.1 m, body mass 77 ± 17 kg) with CMT (CMT1A n = 5; CMT-X n = 3; unknown genetic origin n = 2). Participants were evaluated for QoL, falls efficacy (FES), balance, mobility, muscle strength, and power. Physical component score (PCS) of the Short Form-36 (SF-36) was significantly and directly related to higher leg press power (r = 0.75, p = 0.02). Better FES scores were significantly related to faster habitual gait speed (r = −0.70, p = 0.02), left hip abduction, and seated row strength (r = −0.68, p = 0.03; r = −0.73, p = 0.03, respectively). Future research should aim to substantiate these preliminary findings in a larger cohort and investigate whether interventions targeting muscle strength and power can improve QoL and mobility outcomes in people with CMT.

Original languageEnglish
Pages (from-to)357-364
Number of pages8
JournalJournal of the Peripheral Nervous System
Volume21
Issue number4
DOIs
Publication statusPublished - 1 Dec 2016
Externally publishedYes

Keywords

  • exercise
  • functionality
  • hereditary motor and sensory neuropathy
  • muscular power
  • resistance training

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