Quantifying disease activity in fatty-infiltrated skeletal muscle by IDEAL-CPMG in Duchenne muscular dystrophy

Highlights • IDEAL-CPMG enables quantification of disease activity in fatty-infiltrated muscles. • IDEAL-CPMG muscle AFF and T2,w are useful biomarkers in clinical trials of DMD. • IDEAL-CPMG AFF at baseline may help stratify subjects with DMD in clinical trials. • IDEAL-CPMG T2,w is an objective bi...

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Bibliographic Details
Published inNeuromuscular disorders : NMD Vol. 26; no. 10; pp. 650 - 658
Main Authors Mankodi, Ami, Bishop, Courtney A, Auh, Sungyoung, Newbould, Rexford D, Fischbeck, Kenneth H, Janiczek, Robert L
Format Journal Article
LanguageEnglish
Published England Elsevier B.V 01.10.2016
Subjects
Adipose Tissue - diagnostic imaging
Adolescent
Apparent fat fraction
Child
Child, Preschool
Double-Blind Method
Duchenne muscular dystrophy
Fatty degeneration
Follow-Up Studies
Humans
IDEAL-CPMG
Image Interpretation, Computer-Assisted - methods
Least-Squares Analysis
Linear Models
Magnetic resonance imaging
Magnetic Resonance Imaging - methods
Male
Muscle, Skeletal - diagnostic imaging
Muscle, Skeletal - physiopathology
Muscular Dystrophy, Duchenne - diagnostic imaging
Muscular Dystrophy, Duchenne - genetics
Muscular Dystrophy, Duchenne - physiopathology
Muscular Dystrophy, Duchenne - therapy
Neurology
Oligonucleotides - therapeutic use
Skeletal muscle
T2 relaxation times
Thigh - diagnostic imaging
Treatment Outcome
Walk Test
Walking
Water T2
AFF
T 2 relaxation times
MRI
Fatty degeneration
magnetic resonance spectroscopy
six-minute walk distance
Water T 2
ROI
Skeletal muscle
MRS
IDEAL-CPMG
Apparent fat fraction
Magnetic resonance imaging
Duchenne muscular dystrophy
iterative decomposition of water and fat with echo asymmetry and least-squares estimation Carr–Purcell–Meiboom–Gill
region of interest
DMD
6MWD
T 2,w
T2 relaxation times
T2,w
Water T2
T relaxation times
Water T
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Summary:Highlights • IDEAL-CPMG enables quantification of disease activity in fatty-infiltrated muscles. • IDEAL-CPMG muscle AFF and T2,w are useful biomarkers in clinical trials of DMD. • IDEAL-CPMG AFF at baseline may help stratify subjects with DMD in clinical trials. • IDEAL-CPMG T2,w is an objective biomarker of disease activity in subjects with DMD.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-1
ObjectType-News-3
content type line 23
ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2016.07.013