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

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@article{e994bbc0b51946d9bf14bbb07e4d298f,
title = "Relationship between muscle inflammation and fat replacement assessed by MRI in facioscapulohumeral muscular dystrophy",
abstract = "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+.",
keywords = "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",
author = "Dahlqvist, {Julia R} and Grete Andersen and Tahmina Khawajazada and Christoffer Vissing and Carsten Thomsen and John Vissing",
year = "2019",
month = "5",
doi = "10.1007/s00415-019-09242-y",
language = "English",
volume = "266",
pages = "1127--1135",
journal = "Journal of Neurology",
issn = "0340-5354",
publisher = "Dr. Dietrich/Steinkopff Verlag",
number = "5",

}

RIS

TY - JOUR

T1 - Relationship between muscle inflammation and fat replacement assessed by MRI in facioscapulohumeral muscular dystrophy

AU - Dahlqvist, Julia R

AU - Andersen, Grete

AU - Khawajazada, Tahmina

AU - Vissing, Christoffer

AU - Thomsen, Carsten

AU - Vissing, John

PY - 2019/5

Y1 - 2019/5

N2 - 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+.

AB - 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+.

KW - Disease Progression

KW - Fats/metabolism

KW - Female

KW - Humans

KW - Image Processing, Computer-Assisted

KW - Longitudinal Studies

KW - Magnetic Resonance Imaging

KW - Male

KW - Middle Aged

KW - Muscle, Skeletal/metabolism

KW - Muscular Dystrophy, Facioscapulohumeral/complications

KW - Myositis/complications

KW - Retrospective Studies

U2 - 10.1007/s00415-019-09242-y

DO - 10.1007/s00415-019-09242-y

M3 - Journal article

VL - 266

SP - 1127

EP - 1135

JO - Journal of Neurology

JF - Journal of Neurology

SN - 0340-5354

IS - 5

ER -

ID: 58110280