Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS

被引:124
作者
Arpan, Ishu [1 ]
Willcocks, Rebecca J. [1 ]
Forbes, Sean C. [1 ]
Finkel, Richard S. [5 ]
Lott, Donovan J. [1 ]
Rooney, William D. [6 ]
Triplett, William T. [1 ]
Senesac, Claudia R. [1 ]
Daniels, Michael J. [7 ,8 ]
Byrne, Barry J. [3 ,4 ]
Finanger, Erika L. [6 ]
Russman, Barry S. [6 ,9 ]
Wang, Dah-Jyuu [5 ]
Tennekoon, Gihan I. [5 ]
Walter, Glenn A. [2 ]
Sweeney, H. L. [10 ]
Vandenborne, Krista [1 ]
机构
[1] Univ Florida, Dept Phys Therapy, Gainesville, FL 32611 USA
[2] Univ Florida, Dept Physiol & Funct Genom, Gainesville, FL USA
[3] Univ Florida, Dept Pediat, Gainesville, FL USA
[4] Univ Florida, Powell Gene Therapy Ctr, Dept Mol Genet & Microbiol, Gainesville, FL USA
[5] Childrens Hosp Philadelphia, Philadelphia, PA USA
[6] Oregon Hlth & Sci Univ, Portland, OR 97201 USA
[7] Univ Texas Austin, Dept Integrat Biol, Austin, TX 78712 USA
[8] Univ Texas Austin, Dept Stat & Data Sci, Austin, TX 78712 USA
[9] Shriners Hosp Children, Philadelphia, PA USA
[10] Univ Penn, Dept Physiol, Philadelphia, PA 19104 USA
关键词
DUCHENNE MUSCULAR-DYSTROPHY; RELAXATION-TIME; QUANTITATIVE ASSESSMENT; PREDNISONE; T2; TISSUE; QUANTIFICATION; TRIAL; FAT; AGE;
D O I
10.1212/WNL.0000000000000775
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objective: To evaluate the effects of corticosteroids on the lower extremity muscles in boys with Duchenne muscular dystrophy (DMD) using MRI and magnetic resonance spectroscopy (MRS). Methods: Transverse relaxation time (T2) and fat fraction were measured by MRI/MRS in lower extremity muscles of 15 boys with DMD (age 5.0-6.9 years) taking corticosteroids and 15 corticosteroid-naive boys. Subsequently, fat fraction was measured in a subset of these boys at 1 year. Finally, MRI/MRS data were collected from 16 corticosteroid-naive boys with DMD (age 5-8.9 years) at baseline, 3 months, and 6 months. Five boys were treated with corticosteroids after baseline and the remaining 11 served as corticosteroid-naive controls. Results: Cross-sectional comparisons demonstrated lower muscle T2 and less intramuscular (IM) fat deposition in boys with DMD on corticosteroids, suggesting reduced inflammation/damage and fat infiltration with treatment. Boys on corticosteroids demonstrated less increase in IM fat infiltration at 1 year. Finally, T2 by MRI/MRS detected effects of corticosteroids on leg muscles as early as 3 months after drug initiation. Conclusions: These results demonstrate the ability of MRI/MRS to detect therapeutic effects of corticosteroids in reducing inflammatory processes in skeletal muscles of boys with DMD. Our work highlights the potential of MRI/MRS as a biomarker in evaluating therapeutic interventions in DMD.
引用
收藏
页码:974 / 980
页数:7
相关论文
共 34 条
[1]   Relationships of thigh muscle contractile and non-contractile tissue with function, strength, and age in boys with Duchenne muscular dystrophy [J].
Akima, Hiroshi ;
Lott, Donovan ;
Senesac, Claudia ;
Deol, Jasjit ;
Germain, Sean ;
Arpan, Ishu ;
Bendixen, Roxanna ;
Sweeney, H. Lee ;
Walter, Glenn ;
Vandenborne, Krista .
NEUROMUSCULAR DISORDERS, 2012, 22 (01) :16-25
[2]   DEFLAZACORT IN DUCHENNE DYSTROPHY - STUDY OF LONG-TERM EFFECT [J].
ANGELINI, C ;
PEGORARO, E ;
TURELLA, E ;
INTINO, MT ;
PINI, A ;
COSTA, C .
MUSCLE & NERVE, 1994, 17 (04) :386-391
[3]   T2 mapping provides multiple approaches for the characterization of muscle involvement in neuromuscular diseases: a cross-sectional study of lower leg muscles in 5-15-year-old boys with Duchenne muscular dystrophy [J].
Arpan, Ishu ;
Forbes, Sean C. ;
Lott, Donovan J. ;
Senesac, Claudia R. ;
Daniels, Michael J. ;
Triplett, William T. ;
Deol, Jasjit K. ;
Sweeney, H. Lee ;
Walter, Glenn A. ;
Vandenborne, Krista .
NMR IN BIOMEDICINE, 2013, 26 (03) :320-328
[4]   Role of proton MR for the study of muscle lipid metabolism [J].
Boesch, Chris ;
Machann, Juergen ;
Vermathen, Peter ;
Schick, Fritz .
NMR IN BIOMEDICINE, 2006, 19 (07) :968-988
[5]   Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management [J].
Bushby, Katharine ;
Finkel, Richard ;
Birnkrant, David J. ;
Case, Laura E. ;
Clemens, Paula R. ;
Cripe, Linda ;
Kaul, Ajay ;
Kinnett, Kathi ;
McDonald, Craig ;
Pandya, Shree ;
Poysky, James ;
Shapiro, Frederic ;
Tomezsko, Jean ;
Constantin, Carolyn .
LANCET NEUROLOGY, 2010, 9 (01) :77-93
[6]   LONG-TERM BENEFIT FROM PREDNISONE THERAPY IN DUCHENNE MUSCULAR-DYSTROPHY [J].
FENICHEL, GM ;
FLORENCE, JM ;
PESTRONK, A ;
MENDELL, JR ;
MOXLEY, RT ;
GRIGGS, RC ;
BROOKE, MH ;
MILLER, JP ;
ROBISON, J ;
KING, W ;
SIGNORE, L ;
PANDYA, S ;
SCHIERBECKER, J ;
WILSON, B .
NEUROLOGY, 1991, 41 (12) :1874-1877
[7]   Quantitative MRI can detect subclinical disease progression in muscular dystrophy [J].
Fischmann, Arne ;
Hafner, Patricia ;
Fasler, Susanne ;
Gloor, Monika ;
Bieri, Oliver ;
Studler, Ueli ;
Fischer, Dirk .
JOURNAL OF NEUROLOGY, 2012, 259 (08) :1648-1654
[8]   Skeletal Muscles of Ambulant Children with Duchenne Muscular Dystrophy: Validation of Multicenter Study of Evaluation with MR Imaging and MR Spectroscopy [J].
Forbes, Sean C. ;
Walter, Glenn A. ;
Rooney, William D. ;
Wang, Dah-Jyuu ;
DeVos, Soren ;
Pollaro, Jim ;
Triplett, William ;
Lott, Donovan J. ;
Willcocks, Rebecca J. ;
Senesac, Claudia ;
Daniels, Michael J. ;
Byrne, Barry J. ;
Russman, Barry ;
Finkel, Richard S. ;
Meyer, James S. ;
Sweeney, H. Lee ;
Vandenborne, Krista .
RADIOLOGY, 2013, 269 (01) :198-207
[9]   PREDNISONE IN DUCHENNE DYSTROPHY - A RANDOMIZED, CONTROLLED TRIAL DEFINING THE TIME COURSE AND DOSE-RESPONSE [J].
GRIGGS, RC ;
MOXLEY, RT ;
MENDELL, JR ;
FENICHEL, GM ;
BROOKE, MH ;
PESTRONK, A ;
MILLER, JP .
ARCHIVES OF NEUROLOGY, 1991, 48 (04) :383-388
[10]   Functional and Molecular Effects of Arginine Butyrate and Prednisone on Muscle and Heart in the mdx Mouse Model of Duchenne Muscular Dystrophy [J].
Guerron, Alfredo D. ;
Rawat, Rashmi ;
Sali, Arpana ;
Spurney, Christopher F. ;
Pistilli, Emidio ;
Cha, Hee-Jae ;
Pandey, Gouri S. ;
Gernapudi, Ramkishore ;
Francia, Dwight ;
Farajian, Viken ;
Escolar, Diana M. ;
Bossi, Laura ;
Becker, Magali ;
Zerr, Patricia ;
de la Porte, Sabine ;
Gordish-Dressman, Heather ;
Partridge, Terence ;
Hoffman, Eric P. ;
Nagaraju, Kanneboyina .
PLOS ONE, 2010, 5 (06)