Teenage exercise is associated with earlier symptom onset in dysferlinopathy: a retrospective cohort study

Ursula R Moore, Marni Jacobs, Roberto Fernandez-Torron, Jiji Jang, Meredith K James, Anna Mayhew, Laura Rufibach, Plavi Mittal, Michelle Eagle, Avital Cnaan, Pierre G Carlier, Andrew Blamire, Heather Hilsden, Hanns Lochmüller, Ulrike Grieben, Simone Spuler, Carolina Tesi Rocha, John W Day, Kristi J Jones, Diana X Bharucha-Goebel, Emmanuelle Salort-Campana, Matthew Harms, Alan Pestronk, Sabine Krause, Olivia Schreiber-Katz, Maggie C Walter, Carmen Paradas, Jean-Yves Hogrel, Tanya Stojkovic, Shin'ichi Takeda, Madoka Mori-Yoshimura, Elena Bravver, Susan Sparks, Jordi Diaz-Manera, Luca Bello, Claudio Semplicini, Elena Pegoraro, Jerry R Mendell, Kate Bushby, Volker Straub, Ursula R Moore, Marni Jacobs, Roberto Fernandez-Torron, Jiji Jang, Meredith K James, Anna Mayhew, Laura Rufibach, Plavi Mittal, Michelle Eagle, Avital Cnaan, Pierre G Carlier, Andrew Blamire, Heather Hilsden, Hanns Lochmüller, Ulrike Grieben, Simone Spuler, Carolina Tesi Rocha, John W Day, Kristi J Jones, Diana X Bharucha-Goebel, Emmanuelle Salort-Campana, Matthew Harms, Alan Pestronk, Sabine Krause, Olivia Schreiber-Katz, Maggie C Walter, Carmen Paradas, Jean-Yves Hogrel, Tanya Stojkovic, Shin'ichi Takeda, Madoka Mori-Yoshimura, Elena Bravver, Susan Sparks, Jordi Diaz-Manera, Luca Bello, Claudio Semplicini, Elena Pegoraro, Jerry R Mendell, Kate Bushby, Volker Straub

No abstract available

Keywords: muscular dystrophy; neuromuscular.

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Risk of symptomatic disease and wheelchair use over time in dysferlinopathy. Graphs show the probability of remaining event free during the time under study using Cox proportional hazards regression. Events are the onset of symptoms (A), the first time a wheelchair is used (B) and the need to use a wheelchair full time (C). Survival probabilities significantly different from exercise group 0 are marked with an asterisk (*). For first-time wheelchair use (B), this graph shows the results excluding those patients over 50 years of age at the screening visit as including these patients led to a violation of the proportional hazards assumption.

References

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    1. Klinge L, Aboumousa A, Eagle M, et al. . New aspects on patients affected by dysferlin deficient muscular dystrophy. J Neurol Neurosurg Psychiatry 2010;81:946–53. 10.1136/jnnp.2009.178038
    1. U.S. Department of Health and Human Services PHS, Centers for Disease Control and Prevention, National Center for Chronic Disease Prevention and Health Promotion, Division of Nutrition and Physical Activity. Promoting physical activity: a guide for community action—general physical activities defined by level of intensity: Human Kinetics, 1999.
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    1. Begam M, Galen SS, Roche JA. Exhaustion of regenerative potential due to a low threshold for exercise-induced myogenesis might explain progressive weakness in dysferlin-linked muscle diseases. FASEB J 2016;30(Suppl 1):lb716.

Source: PubMed

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