Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function.

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  • Additional Information
    • Author-Supplied Keywords:
      Abdominal muscles
      Anatomy
      Bioassays and physiological analysis
      Biochemistry
      Biology and life sciences
      Brain mapping
      Diagnostic medicine
      Diagnostic radiology
      Fats
      Functional magnetic resonance imaging
      Imaging techniques
      Lipids
      Magnetic resonance imaging
      Medicine and health sciences
      Muscle analysis
      Muscle functions
      Muscle physiology
      Muscles
      Musculoskeletal system
      Neuroimaging
      Neuroscience
      Physiological processes
      Physiology
      Radiology and imaging
      Research and analysis methods
      Research Article
      Respiration
      Skeletal muscles
    • NAICS/Industry Codes:
      621512 Diagnostic Imaging Centers
    • Abstract:
      Objectives: Enzyme replacement therapy has shown to be effective for childhood/adult onset Pompe disease (AOPD). The discovery of biomarkers useful for monitoring disease progression is one of the priority research topics in Pompe disease. Muscle MRI could be one possible test but the correlation between muscle MRI and muscle strength and function has been only partially addressed so far. Methods: We studied 34 AOPD patients using functional scales (Manual Research Council scale, hand held myometry, 6 minutes walking test, timed to up and go test, time to climb up and down 4 steps, time to walk 10 meters and Motor Function Measure 20 Scale), respiratory tests (Forced Vital Capacity seated and lying, Maximun Inspiratory Pressure and Maximum Expiratory Pressure), daily live activities scales (Activlim) and quality of life scales (Short Form-36 and Individualized Neuromuscular Quality of Life questionnaire). We performed a whole body muscle MRI using T1w and 3-point Dixon imaging centered on thighs and lower trunk region. Results: T1w whole body muscle MRI showed a homogeneous pattern of muscle involvement that could also be found in pre-symptomatic individuals. We found a strong correlation between muscle strength, muscle functional scales and the degree of muscle fatty replacement in muscle MRI analyzed using T1w and 3-point Dixon imaging studies. Moreover, muscle MRI detected mild degree of fatty replacement in paraspinal muscles in pre-symptomatic patients. Conclusion: Based on our findings, we consider that muscle MRI correlates with muscle function in patients with AOPD and could be useful for diagnosis and follow-up in pre-symptomatic and symptomatic patients under treatment. Take home message: Muscle MRI correlates with muscle function in patients with AOPD and could be useful to follow-up patients in daily clinic. [ABSTRACT FROM AUTHOR]
    • Abstract:
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    • Author Affiliations:
      1Neuromuscular Disorders Unit. Neurology Department. Hospital de la Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Spain
      2Centro de Investigación en Red en Enfermedades Raras (CIBERER)
      3Radiology department. Hospital de la Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Spain
      4Rehabilitation and physiotherapy department. Hospital de la Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Spain
      5Respiratory diseases department. Hospital de la Santa Creu i Sant Pau. Barcelona. Universitat Autònoma de Barcelona, Spain
    • ISSN:
      1932-6203
    • Accession Number:
      10.1371/journal.pone.0163493
    • Accession Number:
      118573428
  • Citations
    • ABNT:
      FIGUEROA-BONAPARTE, S. et al. Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function. PLoS ONE, [s. l.], v. 11, n. 10, p. 1–19, 2016. Disponível em: . Acesso em: 19 nov. 2019.
    • AMA:
      Figueroa-Bonaparte S, Segovia S, Llauger J, et al. Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function. PLoS ONE. 2016;11(10):1-19. doi:10.1371/journal.pone.0163493.
    • APA:
      Figueroa-Bonaparte, S., Segovia, S., Llauger, J., Belmonte, I., Pedrosa, I., Alejaldre, A., … null, null. (2016). Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function. PLoS ONE, 11(10), 1–19. https://doi.org/10.1371/journal.pone.0163493
    • Chicago/Turabian: Author-Date:
      Figueroa-Bonaparte, Sebastián, Sonia Segovia, Jaume Llauger, Izaskun Belmonte, Irene Pedrosa, Aída Alejaldre, Mercè Mayos, et al. 2016. “Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function.” PLoS ONE 11 (10): 1–19. doi:10.1371/journal.pone.0163493.
    • Harvard:
      Figueroa-Bonaparte, S. et al. (2016) ‘Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function’, PLoS ONE, 11(10), pp. 1–19. doi: 10.1371/journal.pone.0163493.
    • Harvard: Australian:
      Figueroa-Bonaparte, S, Segovia, S, Llauger, J, Belmonte, I, Pedrosa, I, Alejaldre, A, Mayos, M, Suárez-Cuartín, G, Gallardo, E, Illa, I, Díaz-Manera, J & null, null 2016, ‘Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function’, PLoS ONE, vol. 11, no. 10, pp. 1–19, viewed 19 November 2019, .
    • MLA:
      Figueroa-Bonaparte, Sebastián, et al. “Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function.” PLoS ONE, vol. 11, no. 10, Oct. 2016, pp. 1–19. EBSCOhost, doi:10.1371/journal.pone.0163493.
    • Chicago/Turabian: Humanities:
      Figueroa-Bonaparte, Sebastián, Sonia Segovia, Jaume Llauger, Izaskun Belmonte, Irene Pedrosa, Aída Alejaldre, Mercè Mayos, et al. “Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function.” PLoS ONE 11, no. 10 (October 6, 2016): 1–19. doi:10.1371/journal.pone.0163493.
    • Vancouver/ICMJE:
      Figueroa-Bonaparte S, Segovia S, Llauger J, Belmonte I, Pedrosa I, Alejaldre A, et al. Muscle MRI Findings in Childhood/Adult Onset Pompe Disease Correlate with Muscle Function. PLoS ONE [Internet]. 2016 Oct 6 [cited 2019 Nov 19];11(10):1–19. Available from: http://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=aph&AN=118573428