TY - JOUR
T1 - Relationship between physical performance and quality of life in Charcot-Marie-Tooth disease
T2 - a pilot study
AU - Roberts-Clarke, Daniel
AU - Fornusek, Che
AU - Saigal, Nidhi
AU - Halaki, Mark
AU - Burns, Joshua
AU - Nicholson, Garth
AU - Fiatarone Singh, Maria
AU - Hackett, Daniel
PY - 2016/12/1
Y1 - 2016/12/1
N2 - 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.
AB - 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.
KW - exercise
KW - functionality
KW - hereditary motor and sensory neuropathy
KW - muscular power
KW - resistance training
UR - http://www.scopus.com/inward/record.url?scp=85007587042&partnerID=8YFLogxK
U2 - 10.1111/jns.12191
DO - 10.1111/jns.12191
M3 - Article
C2 - 27699915
AN - SCOPUS:85007587042
SN - 1085-9489
VL - 21
SP - 357
EP - 364
JO - Journal of the Peripheral Nervous System
JF - Journal of the Peripheral Nervous System
IS - 4
ER -