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Neuropediatrics 2020; 51(05): 342-348
DOI: 10.1055/s-0040-1708859
Original Article

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

Astrid Blaschek‡   *
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Martin Rodrigues‡   *
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Lena Ille
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Mohammed Idriess
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Therese Well
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Birgit Warken
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Christine Müller
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Iris Hannibal
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Moritz Tacke
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Wolfgang Müller-Felber
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
,
Katharina Vill
1   Department of Pediatric Neurology and Developmental Medicine, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany
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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.

Keywords

Duchenne muscular dystrophy - outcome measures - clinical trials - 6-minute walk test - fatigue

* The authors contributed equally to the manuscript.




Publication History

Received: 09 November 2019

Accepted: 12 February 2020

Article published online:
05 May 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Stuttgart · New York

 

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