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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 958-963 |
| Number of pages | 6 |
| Journal | Muscle and Nerve |
| Volume | 57 |
| Issue number | 6 |
| DOIs | |
| State | Published - Jun 1 2018 |
Fingerprint
Keywords
- biomarkers
- facioscapulohumeral muscular dystrophy
- magnetic resonance spectroscopy
- metabolite quantification
- skeletal muscle
- trimethylamine
ASJC Scopus subject areas
- Physiology
- Clinical Neurology
- Cellular and Molecular Neuroscience
- Physiology (medical)
Cite this
Multivoxel proton magnetic resonance spectroscopy in facioscapulohumeral muscular dystrophy. / Leung, Doris G.; Wang, Xin; Barker, Peter B; Carrino, John A.; Wagner, Kathryn Rae.
In: Muscle and Nerve, Vol. 57, No. 6, 01.06.2018, p. 958-963.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Multivoxel proton magnetic resonance spectroscopy in facioscapulohumeral muscular dystrophy
AU - Leung, Doris G.
AU - Wang, Xin
AU - Barker, Peter B
AU - Carrino, John A.
AU - Wagner, Kathryn Rae
PY - 2018/6/1
Y1 - 2018/6/1
N2 - 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.
AB - 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.
KW - biomarkers
KW - facioscapulohumeral muscular dystrophy
KW - magnetic resonance spectroscopy
KW - metabolite quantification
KW - skeletal muscle
KW - trimethylamine
UR - http://www.scopus.com/inward/record.url?scp=85047499071&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85047499071&partnerID=8YFLogxK
U2 - 10.1002/mus.26048
DO - 10.1002/mus.26048
M3 - Article
C2 - 29266323
AN - SCOPUS:85047499071
VL - 57
SP - 958
EP - 963
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 6
ER -