Is Exercise-Induced Fatigue a Problem in Children with Duchenne Muscular Dystrophy?

Neuropediatrics. 2020 Oct;51(5):342-348. doi: 10.1055/s-0040-1708859. Epub 2020 May 5.

Abstract

Objective: Duchenne muscular dystrophy (DMD) is a devastating X-linked muscular disorder. The number of studies investigating new therapeutic approaches is substantially increasing. This study aims to investigate the impact and diagnostic value of exercise-induced fatigue in DMD, which has been proposed as a suitable outcome parameter in other conditions like spinal muscular atrophy.

Patients and methods: A cohort of 55 DMD patients (49 of them treated with steroids and 9 with ataluren) underwent a total of 241 6MWT (mean 4.4 tests/patient) which were retrospectively analyzed. Exercise-induced fatigue was assessed by the ratio between the distance achieved in the sixth minute and the distance in the second minute of the 6MWT. In previous studies a quotient above 1 was defined as a sign of fatigue.

Results: The average fatigue quotient in the whole cohort of patients was 1.0. In a further analysis no impact of age, steroid therapy, ataluren therapy, overall disability, and distance in the 6-minute walk test (6MWT) on fatigue in DMD patients could be shown.

Conclusion: Our data show that fatigue does not play a relevant role in DMD. Analysis of fatigue is not a useful outcome parameter in DMD studies. For this reason we suggest the 2MWT, which is better accepted by the patients, as an alternative to the commonly 6MWT.

MeSH terms

  • Adolescent
  • Child
  • Exercise / physiology*
  • Exercise Test / standards*
  • Fatigue / etiology
  • Fatigue / physiopathology*
  • Humans
  • Male
  • Muscular Dystrophy, Duchenne / complications
  • Muscular Dystrophy, Duchenne / diagnosis*
  • Muscular Dystrophy, Duchenne / physiopathology*
  • Predictive Value of Tests
  • Retrospective Studies