T2 relaxation times are increased in Skeletal muscle of DMD but not BMD patients

Beatrijs H Wokke; Janneke C Van Den Bergen; Melissa T Hooijmans; Jan J Verschuuren; Erik H Niks; Hermien E Kan

doi:10.1002/mus.24679

T2 relaxation times are increased in Skeletal muscle of DMD but not BMD patients

Beatrijs H Wokke
, Janneke C Van Den Bergen
, Melissa T Hooijmans
, Jan J Verschuuren
, Erik H Niks
, Hermien E Kan

Center Proteomics Metabolomics, Leiden University Medical Center, Leiden, Netherlands.
Department of Radiology, C.J. Gorter Center for High Field MRI, Leiden, the Netherlands

Research output: Contribution to journal › Article › Academic › peer-review

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Abstract

INTRODUCTION: Exon-skipping drugs in Duchenne muscular dystrophy (DMD) aim to restore truncated dystrophin expression, which is present in the milder Becker muscular dystrophy (BMD). MRI skeletal muscle T2 relaxation times as a representation of edema/inflammation could be quantitative outcome parameters for such trials.

METHODS: We studied T2 relaxation times, adjusted for muscle fat fraction using Dixon MRI, in lower leg muscles of DMD and BMD patients and healthy controls.

RESULTS: T2 relaxation times correlated significantly with fat fractions in patients only (P < 0.001). After adjusting for muscle fat, T2 relaxation times were significantly increased in 6 muscles of DMD patients (P < 0.01), except for the extensor digitorum longus. In BMD, T2 relaxation times were unchanged.

CONCLUSIONS: T2 relaxation times could be a useful outcome parameter in exon-skipping trials in DMD but are influenced by fat despite fat suppression. This should be accounted for when using quantitative T2 mapping to investigate edema/inflammation.

Original language	English
Pages (from-to)	38-43
Number of pages	6
Journal	Muscle & nerve
Volume	53
Issue number	1
DOIs	https://doi.org/10.1002/mus.24679
Publication status	Published - Jan 2016
Externally published	Yes

Keywords

Adolescent
Adult
Child
Female
Humans
Leg/pathology
Magnetic Resonance Imaging
Male
Middle Aged
Muscle, Skeletal/pathology
Muscular Dystrophy, Duchenne/pathology
Relaxation
Young Adult

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title = "T2 relaxation times are increased in Skeletal muscle of DMD but not BMD patients",

abstract = "INTRODUCTION: Exon-skipping drugs in Duchenne muscular dystrophy (DMD) aim to restore truncated dystrophin expression, which is present in the milder Becker muscular dystrophy (BMD). MRI skeletal muscle T2 relaxation times as a representation of edema/inflammation could be quantitative outcome parameters for such trials.METHODS: We studied T2 relaxation times, adjusted for muscle fat fraction using Dixon MRI, in lower leg muscles of DMD and BMD patients and healthy controls.RESULTS: T2 relaxation times correlated significantly with fat fractions in patients only (P < 0.001). After adjusting for muscle fat, T2 relaxation times were significantly increased in 6 muscles of DMD patients (P < 0.01), except for the extensor digitorum longus. In BMD, T2 relaxation times were unchanged.CONCLUSIONS: T2 relaxation times could be a useful outcome parameter in exon-skipping trials in DMD but are influenced by fat despite fat suppression. This should be accounted for when using quantitative T2 mapping to investigate edema/inflammation.",

keywords = "Adolescent, Adult, Child, Female, Humans, Leg/pathology, Magnetic Resonance Imaging, Male, Middle Aged, Muscle, Skeletal/pathology, Muscular Dystrophy, Duchenne/pathology, Relaxation, Young Adult",

author = "Wokke, \{Beatrijs H\} and \{Van Den Bergen\}, \{Janneke C\} and Hooijmans, \{Melissa T\} and Verschuuren, \{Jan J\} and Niks, \{Erik H\} and Kan, \{Hermien E\}",

note = "{\textcopyright} 2015 Wiley Periodicals, Inc.",

year = "2016",

month = jan,

doi = "10.1002/mus.24679",

language = "English",

volume = "53",

pages = "38--43",

journal = "Muscle \& nerve",

issn = "0148-639X",

publisher = "John Wiley and Sons Inc.",

number = "1",

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TY - JOUR

T1 - T2 relaxation times are increased in Skeletal muscle of DMD but not BMD patients

AU - Wokke, Beatrijs H

AU - Van Den Bergen, Janneke C

AU - Hooijmans, Melissa T

AU - Verschuuren, Jan J

AU - Niks, Erik H

AU - Kan, Hermien E

PY - 2016/1

Y1 - 2016/1

N2 - INTRODUCTION: Exon-skipping drugs in Duchenne muscular dystrophy (DMD) aim to restore truncated dystrophin expression, which is present in the milder Becker muscular dystrophy (BMD). MRI skeletal muscle T2 relaxation times as a representation of edema/inflammation could be quantitative outcome parameters for such trials.METHODS: We studied T2 relaxation times, adjusted for muscle fat fraction using Dixon MRI, in lower leg muscles of DMD and BMD patients and healthy controls.RESULTS: T2 relaxation times correlated significantly with fat fractions in patients only (P < 0.001). After adjusting for muscle fat, T2 relaxation times were significantly increased in 6 muscles of DMD patients (P < 0.01), except for the extensor digitorum longus. In BMD, T2 relaxation times were unchanged.CONCLUSIONS: T2 relaxation times could be a useful outcome parameter in exon-skipping trials in DMD but are influenced by fat despite fat suppression. This should be accounted for when using quantitative T2 mapping to investigate edema/inflammation.

AB - INTRODUCTION: Exon-skipping drugs in Duchenne muscular dystrophy (DMD) aim to restore truncated dystrophin expression, which is present in the milder Becker muscular dystrophy (BMD). MRI skeletal muscle T2 relaxation times as a representation of edema/inflammation could be quantitative outcome parameters for such trials.METHODS: We studied T2 relaxation times, adjusted for muscle fat fraction using Dixon MRI, in lower leg muscles of DMD and BMD patients and healthy controls.RESULTS: T2 relaxation times correlated significantly with fat fractions in patients only (P < 0.001). After adjusting for muscle fat, T2 relaxation times were significantly increased in 6 muscles of DMD patients (P < 0.01), except for the extensor digitorum longus. In BMD, T2 relaxation times were unchanged.CONCLUSIONS: T2 relaxation times could be a useful outcome parameter in exon-skipping trials in DMD but are influenced by fat despite fat suppression. This should be accounted for when using quantitative T2 mapping to investigate edema/inflammation.

KW - Adolescent

KW - Adult

KW - Child

KW - Female

KW - Humans

KW - Leg/pathology

KW - Magnetic Resonance Imaging

KW - Male

KW - Middle Aged

KW - Muscle, Skeletal/pathology

KW - Muscular Dystrophy, Duchenne/pathology

KW - Relaxation

KW - Young Adult

U2 - 10.1002/mus.24679

DO - 10.1002/mus.24679

M3 - Article

SN - 0148-639X

VL - 53

SP - 38

EP - 43

JO - Muscle & nerve

JF - Muscle & nerve

IS - 1

ER -

T2 relaxation times are increased in Skeletal muscle of DMD but not BMD patients

Abstract

Keywords

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