. 2018 Nov;89(11):1224-1226.

doi: 10.1136/jnnp-2017-317329. Epub 2018 Jan 29.

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

Ursula R Moore¹, Marni Jacobs^{2

3}, Roberto Fernandez-Torron^{1

4}, Jiji Jang^{2

3}, Meredith K James¹, Anna Mayhew¹, Laura Rufibach⁵, Plavi Mittal⁵, Michelle Eagle¹, Avital Cnaan^{3

2}, Pierre G Carlier⁶, Andrew Blamire⁷, Heather Hilsden¹, Hanns Lochmüller¹, Ulrike Grieben⁸, Simone Spuler⁸, Carolina Tesi Rocha⁹, John W Day⁹, Kristi J Jones¹⁰, Diana X Bharucha-Goebel^{11

12}, Emmanuelle Salort-Campana¹³, Matthew Harms¹⁴, Alan Pestronk¹⁴, Sabine Krause¹⁵, Olivia Schreiber-Katz¹⁵, Maggie C Walter¹⁵, Carmen Paradas¹⁶, Jean-Yves Hogrel¹⁷, Tanya Stojkovic¹⁷, Shin'ichi Takeda¹⁸, Madoka Mori-Yoshimura¹⁸, Elena Bravver¹⁹, Susan Sparks¹⁹, Jordi Diaz-Manera^{20

21}, Luca Bello²², Claudio Semplicini²², Elena Pegoraro²², Jerry R Mendell²³, Kate Bushby¹, Volker Straub¹

Affiliations

¹ The John Walton Muscular Dystrophy Research Centre, MRC Centre for Neuromuscular Diseases, Institute of Genetic Medicine, Newcastle upon Tyne, UK.
² Children's Research Institute, Division of Biostatistics and Study Methodology, Children's National Health System, Washington, District of Columbia, USA.
³ Department of Pediatrics, Epidemiology and Biostatistics, George Washington University, Washington, District of Columbia, USA.
⁴ Neuromuscular Area, Biodonostia Health Research Institute, Neurology Service, Donostia University Hospital, Donostia-San Sebastian, Spain.
⁵ The Jain Foundation, Seattle, Washington, USA.
⁶ AIM and CEA NMR Laboratory, Institute of Myology, Pitié-Salpêtrière University Hospital, Paris, France.
⁷ Magnetic Resonance Centre, Institute for Cellular Medicine, Newcastle University, Newcastle Upon Tyne, UK.
⁸ Charite Muscle Research Unit, Experimental and Clinical Research Center, The Max Delbrück Center for Molecular Medicine, Berlin, Germany.
⁹ Department of Neurology and Neurological Sciences, School of Medicine, Stanford University, Stanford, California, USA.
¹⁰ Institute for Neuroscience and Muscle Research, Children's Hospital at Westmead, University of Sydney, Sydney, New South Wales, Australia.
¹¹ Department of Neurology, Children's National Health System, Washington, District of Columbia, USA.
¹² National Institutes of Health (NINDS), Bethesda, Maryland, USA.
¹³ Department of Neuromuscular and ALS Center, La Timone Hospital, Aix-Marseille Université, Marseille, France.
¹⁴ Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
¹⁵ Department of Neurology, Friedrich-Baur-Institute, Ludwig-Maximilians-University of Munich, Munich, Germany.
¹⁶ Neuromuscular Unit, Hospital Universitario Virgen del Rocío/Instituto de Biomedicina de Sevilla, Sevilla, Spain.
¹⁷ Institut de Myologie, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Boulevard de l'Hôpital, Paris, France.
¹⁸ Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.
¹⁹ Carolinas Healthcare System Neurosciences Institute, Charlotte, North Carolina, USA.
²⁰ Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER), Barcelona, Spain.
²¹ Neuromuscular Disorders Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
²² Department of Neuroscience, University of Padova, Padova, Italy.
²³ Nationwide Children's Hospital, Columbus, Ohio, USA.

PMID: 29378789
PMCID: PMC6227796
DOI: 10.1136/jnnp-2017-317329

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

Ursula R Moore et al. J Neurol Neurosurg Psychiatry. 2018 Nov.

. 2018 Nov;89(11):1224-1226.

doi: 10.1136/jnnp-2017-317329. Epub 2018 Jan 29.

Authors

Affiliations

¹ The John Walton Muscular Dystrophy Research Centre, MRC Centre for Neuromuscular Diseases, Institute of Genetic Medicine, Newcastle upon Tyne, UK.
² Children's Research Institute, Division of Biostatistics and Study Methodology, Children's National Health System, Washington, District of Columbia, USA.
³ Department of Pediatrics, Epidemiology and Biostatistics, George Washington University, Washington, District of Columbia, USA.
⁴ Neuromuscular Area, Biodonostia Health Research Institute, Neurology Service, Donostia University Hospital, Donostia-San Sebastian, Spain.
⁵ The Jain Foundation, Seattle, Washington, USA.
⁶ AIM and CEA NMR Laboratory, Institute of Myology, Pitié-Salpêtrière University Hospital, Paris, France.
⁷ Magnetic Resonance Centre, Institute for Cellular Medicine, Newcastle University, Newcastle Upon Tyne, UK.
⁸ Charite Muscle Research Unit, Experimental and Clinical Research Center, The Max Delbrück Center for Molecular Medicine, Berlin, Germany.
⁹ Department of Neurology and Neurological Sciences, School of Medicine, Stanford University, Stanford, California, USA.
¹⁰ Institute for Neuroscience and Muscle Research, Children's Hospital at Westmead, University of Sydney, Sydney, New South Wales, Australia.
¹¹ Department of Neurology, Children's National Health System, Washington, District of Columbia, USA.
¹² National Institutes of Health (NINDS), Bethesda, Maryland, USA.
¹³ Department of Neuromuscular and ALS Center, La Timone Hospital, Aix-Marseille Université, Marseille, France.
¹⁴ Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
¹⁵ Department of Neurology, Friedrich-Baur-Institute, Ludwig-Maximilians-University of Munich, Munich, Germany.
¹⁶ Neuromuscular Unit, Hospital Universitario Virgen del Rocío/Instituto de Biomedicina de Sevilla, Sevilla, Spain.
¹⁷ Institut de Myologie, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Boulevard de l'Hôpital, Paris, France.
¹⁸ Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.
¹⁹ Carolinas Healthcare System Neurosciences Institute, Charlotte, North Carolina, USA.
²⁰ Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER), Barcelona, Spain.
²¹ Neuromuscular Disorders Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
²² Department of Neuroscience, University of Padova, Padova, Italy.
²³ Nationwide Children's Hospital, Columbus, Ohio, USA.

PMID: 29378789
PMCID: PMC6227796
DOI: 10.1136/jnnp-2017-317329

No abstract available

Keywords: muscular dystrophy; neuromuscular.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

**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.

See this image and copyright information in PMC

References

1. Harris E, Bladen CL, Mayhew A, et al. . The clinical outcome study for dysferlinopathy: an international multicenter study. Neurol Genet 2016;2:e89 10.1212/NXG.0000000000000089 - DOI - PMC - PubMed
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 - DOI - PMC - PubMed
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.
1. Angelini C, Peterle E, Gaiani A, et al. . Dysferlinopathy course and sportive activity: clues for possible treatment. Acta Myol 2011;30:127–32. - PMC - PubMed
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.

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

Affiliations

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

Authors

Affiliations

Conflict of interest statement

Figures

References

Publication types

MeSH terms

Supplementary concepts

Associated data

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical