title: Quantitative MRI and strength measurements in the assessment of muscle quality in Duchenne muscular dystrophy.
abstract: The purpose of this study was to assess leg muscle quality and give a detailed description of leg muscle involvement in a series of Duchenne muscular dystrophy patients using quantitative MRI and strength measurements. Fatty infiltration, as well as total and contractile (not fatty infiltrated) cross sectional areas of various leg muscles were determined in 16 Duchenne patients and 11 controls (aged 8-15). To determine specific muscle strength, four leg muscle groups (quadriceps femoris, hamstrings, anterior tibialis and triceps surae) were measured and related to the amount of contractile tissue. In patients, the quadriceps femoris showed decreased total and contractile cross sectional area, attributable to muscle atrophy. The total, but not the contractile, cross sectional area of the triceps surae was increased in patients, corresponding to hypertrophy. Specific strength decreased in all four muscle groups of Duchenne patients, indicating reduced muscle quality. This suggests that muscle hypertrophy and fatty infiltration are two distinct pathological processes, differing between muscle groups. Additionally, the quality of remaining muscle fibers is severely reduced in the legs of Duchenne patients. The combination of quantitative MRI and quantitative muscle testing could be a valuable outcome parameter in longitudinal studies and in the follow-up of therapeutic effects.
authors.affiliation: Dept of Neurology, Leiden University Medical Center, The Netherlands. Electronic address: b.h.a.wokke@lumc.nl.
authors.forename: B H
authors.initials: BH
authors.lastname: Wokke
authors.affiliation: Dept of Neurology, Leiden University Medical Center, The Netherlands.
authors.forename: J C
authors.initials: JC
authors.lastname: van den Bergen
authors.affiliation: Dept of Radiology, C.J. Gorter Center, Leiden University Medical Center, The Netherlands.
authors.forename: M J
authors.initials: MJ
authors.lastname: Versluis
authors.affiliation: Dept of Neurology, Leiden University Medical Center, The Netherlands.
authors.forename: E H
authors.initials: EH
authors.lastname: Niks
authors.affiliation: Dept of Radiology, Division of Image Processing (LKEB), Leiden University Medical Center, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Milles
authors.affiliation: Dept of Radiology, C.J. Gorter Center, Leiden University Medical Center, The Netherlands.
authors.forename: A G
authors.initials: AG
authors.lastname: Webb
authors.affiliation: Dept of Medical Statistics, Leiden University Medical Center, The Netherlands.
authors.forename: E W
authors.initials: EW
authors.lastname: van Zwet
authors.affiliation: Dept of Human Genetics, Leiden University Medical Center, The Netherlands.
authors.forename: A
authors.initials: A
authors.lastname: Aartsma-Rus
authors.affiliation: Dept of Neurology, Leiden University Medical Center, The Netherlands.
authors.forename: J J
authors.initials: JJ
authors.lastname: Verschuuren
authors.affiliation: Dept of Radiology, C.J. Gorter Center, Leiden University Medical Center, The Netherlands.
authors.forename: H E
authors.initials: HE
authors.lastname: Kan
mesh_terms: D000273:Adipose Tissue / Q000473:pathology; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002648:Child; D006801:Humans; D006984:Hypertrophy / Q000473:pathology / Q000503:physiopathology; D007091:Image Processing, Computer-Assisted; D007866:Leg; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D053580:Muscle Strength; D018482:Muscle, Skeletal / Q000473:pathology* / Q000503:physiopathology*; D020388:Muscular Dystrophy, Duchenne / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology*; D009929:Organ Size; D052097:Quadriceps Muscle / Q000473:pathology / Q000503:physiopathology
chemical_list: D000305:Adrenal Cortex Hormones
keywords: Duchenne muscular dystrophy; Muscle quality; Quantitative MRI