Work

Multivoxel proton magnetic resonance spectroscopy in facioscapulohumeral muscular dystrophy

Year: 2017

Type: article

Abstract: ABSTRACT Introduction: Facioscapulohumeral muscular dystrophy (FSHD) is a hereditary disorder that causes progressive muscle wasting. This study evaluates the use of proton magnetic resonance spectroscopy (1H MRS) as a biomarker of muscle strength and function in FSHD. Methods: Thirty‐six individuals with FSHD and 15 healthy controls underwent multivoxel 1H MRS of a cross‐section of the mid‐thigh. Concentrations of creatine, intramyocellular and extramyocellular lipids, and trimethylamine (TMA)‐containing compounds in skeletal muscle were calculated. Metabolite concentrations for individuals with FSHD were compared with those of controls. The relationship between metabolite concentrations and muscle strength was also examined. Results: The TMA/creatine (Cr) ratio in individuals with FSHD was reduced compared with controls. The TMA/Cr ratio in the hamstrings also showed a moderate linear correlation with muscle strength. Discussion: 1H MRS offers a potential method of detecting early muscle pathology in FSHD prior to the development of fat infiltration. Muscle Nerve 57: 958–963, 2018

Source: Muscle & Nerve

DOI: 10.1002/mus.26048

Authors: Doris G. Leung, Xin Wang, Peter B. Barker, John A. Carrino, Kathryn R. Wagner

Institutions: Kennedy Krieger Institute, Johns Hopkins University, Johns Hopkins Medicine, University of Nebraska Medical Center, Hospital for Special Surgery

Language: English

Topic: Muscle Physiology and Disorders

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: green