(HRQoL) is an important outcome in CMT patients clinical trials

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Neuromuscul Disord. 2008 Jul 23.

Factors that influence health-related quality of life in Australian

adults with Charcot-Marie-Tooth disease.

Redmond AC, Burns J, Ouvrier RA.

School of Medicine, The University of Leeds, Leeds, United Kingdom.

Health-related, quality of life (HRQoL) is an important outcome in

clinical trials of patients with Charcot-Marie-Tooth disease (CMT).

In a cross-sectional survey of 295 Australian adults with CMT, HRQoL

was measured using the Short Form-36 (SF-36) and predictors of

reduced HRQoL were identified with a CMT-specific health status

questionnaire.

People with CMT demonstrated lower HRQoL scores than the general

Australian population in all SF-36 dimensions. The disparity between

people with CMT and normative data was greater for physical

dimensions than for mental health dimensions. SF-36 scores were

generally lower in older vs younger people, but not between men and

women, or between CMT types.

HRQoL in CMT was predicted strongly by lower limb weakness and to a

lessor extent by leg cramps, suggesting clinical trials targeting

weakness and cramps may improve HRQoL in patients with CMT.