MR quantification of muscle fatty replacement in McArdle's disease

McArdle's disease is an energy-dependant disorder of skeletal muscle caused by the inability to break down glycogen. The aim of this study was to quantify fatty replacement in patients with McArdle's disease. Calf and thigh axial spin echo T 1-weighted magnetic resonance (MR) images (repet...

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Bibliographic Details
Published in:Magnetic resonance imaging 1996, Vol.14 (10), p.1137-1141
Main Authors: de Kerviler, Eric, Leroy-Willig, Anne, Duboc, Denis, Eymard, Bruno, Syrota, André
Format: Article
Language:English
Subjects:
Adipose Tissue - pathology
Adult
Aged
Biological and medical sciences
Carbohydrates (enzymatic deficiencies). Glycogenosis
Errors of metabolism
Female
Glycogen Storage Disease Type V - diagnosis
Glycogen Storage Disease Type V - pathology
Humans
Leg
Magnetic Resonance Imaging
Male
McArdle's disease
Medical sciences
Metabolic diseases
Middle Aged
Muscle diseases
Muscle, Skeletal - pathology
Muscular glygogenoses
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Summary:McArdle's disease is an energy-dependant disorder of skeletal muscle caused by the inability to break down glycogen. The aim of this study was to quantify fatty replacement in patients with McArdle's disease. Calf and thigh axial spin echo T 1-weighted magnetic resonance (MR) images (repetition time 500 ms, echo time 25 ms) were obtained at 0.5 T in nine patients with McArdle's disease (age 51 ± 16 years, range 26–74) and nine sex- and age-matched healthy subjects (age 52 ± 16 years, range 29–78) to quantify intramuscular fat. Regions of interest were drawn manually, encompassing the largest cross section of muscle. A fatty replacement index (IF) was determined from histograms of signal in the regions of interest in calf and thigh muscles. In normal subjects, IF = 3.6 ± 2.8% in calf and 4.9 ± 2.3% in thigh. In patients, IF = 11 ± 9.3% in calf and 13.5 ± 10.4% in thigh, significantly different from IF values in normal subjects ( p = .03). IF correlated well with age in patients ( p = .03). In older patients, up to 25% of the muscle volume was replaced by fat. Patients with McArdle's disease, usually weakly disabled, exhibit significant muscle fatty replacement on MR images. These findings suggest a progressive muscle loss over time related to the disease process.
ISSN:0730-725X
1873-5894
DOI:10.1016/S0730-725X(96)00236-6