Research
Print page Print page
Switch language
Rigshospitalet - a part of Copenhagen University Hospital
Published

Relationship between muscle inflammation and fat replacement assessed by MRI in facioscapulohumeral muscular dystrophy

Research output: Contribution to journalJournal articleResearchpeer-review

  1. Characteristic muscle signatures assessed by quantitative MRI in patients with Bethlem myopathy

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. Physical activity in myotonic dystrophy type 1

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. European muscle MRI study in limb girdle muscular dystrophy type R1/2A (LGMDR1/LGMD2A)

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. High-intensity training in patients with spinal and bulbar muscular atrophy

    Research output: Contribution to journalJournal articleResearchpeer-review

  1. Creation and implementation of a European registry for patients with McArdle disease and other muscle glycogenoses (EUROMAC registry)

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. Phenotypic Spectrum of α-Dystroglycanopathies Associated With the c.919T>a Variant in the FKRP Gene in Humans and Mice

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. Bimagrumab vs Optimized Standard of Care for Treatment of Sarcopenia in Community-Dwelling Older Adults: A Randomized Clinical Trial

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. Vacuoles, Often Containing Glycogen, Are a Consistent Finding in Hypokalemic Periodic Paralysis

    Research output: Contribution to journalJournal articleResearchpeer-review

  5. Editorial: Remaining diagnostic issues and start of a treatment era for muscle diseases

    Research output: Contribution to journalJournal articleResearchpeer-review

View graph of relations

OBJECTIVE: Unlike most muscular dystrophies that progress symmetrically at a constant rate, facioscapulohumeral muscular dystrophy (FSHD) is characterized by stepwise, asymmetric progression of muscle wasting, and weakness. Muscle tissue is progressively replaced by fat; however, its relation to preceding inflammation is unclear. In this longitudinal study of FSHD, we assessed muscle inflammation and fat replacement and their relation quantitatively. We also investigated whether fat replacement in muscle varies along its length.

METHODS: Forty-five patients with FSHD were evaluated twice, 14 months apart. Using MRI sequences with short TI inversion recovery (STIR), we quantified the degree of STIR hyperintensity in muscles (≥ 2 SD above control intensity). STIR hyperintensities (STIR+) suggest edema or inflammation. We used Dixon MRI to quantify fat content.

RESULTS: Of 370 thigh muscles, 83 were STIR+ at baseline and 103 at follow-up. The highest frequency of STIR+ was seen in muscles with inter-mediate fat content (40-60% fat). The progression of fat replacement was higher in STIR+ muscles (5.0 ± 4.0%) vs. STIR- muscles [2.3 ± 3.3% (P < 0.0001)]. In addition, muscles with severe STIR+ at baseline had a higher fat replacement progression than muscles with milder STIR+ (R = 0.39, P = 0.001). The fat content was higher in the distal part vs. proximal part of most muscles (P < 0.05). However, the progression of the fat replacement was uniform along the length of all the muscles.

CONCLUSION: Muscles with STIR+, indicating inflammation, have a faster progression of fat replacement than STIR- muscles, and the fat replacement progression correlated with the severity of STIR+.

Original languageEnglish
JournalJournal of Neurology
Volume266
Issue number5
Pages (from-to)1127-1135
Number of pages9
ISSN0340-5354
DOIs
Publication statusPublished - May 2019

    Research areas

  • Disease Progression, Fats/metabolism, Female, Humans, Image Processing, Computer-Assisted, Longitudinal Studies, Magnetic Resonance Imaging, Male, Middle Aged, Muscle, Skeletal/metabolism, Muscular Dystrophy, Facioscapulohumeral/complications, Myositis/complications, Retrospective Studies

ID: 58110280