Myotonic dystrophy type 1, the most common adult-onset form of muscular dystrophy, is a multisystem disease with progressively worsening symptoms. Fatigue constitutes the most common non-muscular symptom in patients with this disease,1 and can exact a heavy toll on their quality of life.
This multicentre, single-blind, randomised trial aimed to determine whether cognitive behavioural therapy optionally combined with graded exercise compared with standard care alone improved the health status of patients with myotonic dystrophy type 1.
Participants were randomly assigned (1:1) to either cognitive behavioural therapy plus standard care and optional graded exercise or standard care alone. Cognitive behavioural therapy focused on addressing reduced patient initiative, increasing physical activity, optimising social interaction, regulating sleep-wake patterns, coping with pain, and addressing beliefs about fatigue and myotonic dystrophy type 1. Cognitive behavioural therapy was delivered over a 10-month period in 10-14 sessions. The primary outcome was the 10-month change from baseline in scores on the DM1-Activ-c scale, a measure of capacity for activity and social participation (score range 0-100).
The results demonstrated that cognitive behavioural therapy increased the capacity for activity and social participation in patients with myotonic dystrophy type 1 at 10 months. With no curative treatment and few symptomatic treatments, cognitive behavioural therapy could be considered for use in severely fatigued patients with myotonic dystrophy type 1.
