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

Ishu Arpan; Rebecca J. Willcocks; Sean C. Forbes; Richard S. Finkel; Donovan J. Lott; William D. Rooney; William T. Triplett; Claudia R. Senesac; Michael J. Daniels; Barry J. Byrne; Erika L. Finanger; Barry S. Russman; Dah Jyuu Wang; Gihan I. Tennekoon; Glenn A. Walter; H. L. Sweeney; Krista Vandenborne

doi:10.1212/WNL.0000000000000775

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

Ishu Arpan, Rebecca J. Willcocks, Sean C. Forbes, Richard S. Finkel, Donovan J. Lott, William D. Rooney, William T. Triplett, Claudia R. Senesac, Michael J. Daniels, Barry J. Byrne, Erika L. Finanger, Barry S. Russman, Dah Jyuu Wang, Gihan I. Tennekoon, Glenn A. Walter, H. L. Sweeney, Krista Vandenborne

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124 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	974-980
Number of pages	7
Journal	Neurology
Volume	83
Issue number	11
DOIs	https://doi.org/10.1212/WNL.0000000000000775
State	Published - Sep 1 2014

ASJC Scopus subject areas

Clinical Neurology

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10.1212/WNL.0000000000000775

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Arpan, I., Willcocks, R. J., Forbes, S. C., Finkel, R. S., Lott, D. J., Rooney, W. D., Triplett, W. T., Senesac, C. R., Daniels, M. J., Byrne, B. J., Finanger, E. L., Russman, B. S., Wang, D. J., Tennekoon, G. I., Walter, G. A., Sweeney, H. L., & Vandenborne, K. (2014). Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS. Neurology, 83(11), 974-980. https://doi.org/10.1212/WNL.0000000000000775

Arpan, I, Willcocks, RJ, Forbes, SC, Finkel, RS, Lott, DJ, Rooney, WD, Triplett, WT, Senesac, CR, Daniels, MJ, Byrne, BJ, Finanger, EL, Russman, BS, Wang, DJ, Tennekoon, GI, Walter, GA, Sweeney, HL & Vandenborne, K 2014, 'Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS', Neurology, vol. 83, no. 11, pp. 974-980. https://doi.org/10.1212/WNL.0000000000000775

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title = "Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS",

abstract = "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.",

author = "Ishu Arpan and Willcocks, {Rebecca J.} and Forbes, {Sean C.} and Finkel, {Richard S.} and Lott, {Donovan J.} and Rooney, {William D.} and Triplett, {William T.} and Senesac, {Claudia R.} and Daniels, {Michael J.} and Byrne, {Barry J.} and Finanger, {Erika L.} and Russman, {Barry S.} and Wang, {Dah Jyuu} and Tennekoon, {Gihan I.} and Walter, {Glenn A.} and Sweeney, {H. L.} and Krista Vandenborne",

year = "2014",

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doi = "10.1212/WNL.0000000000000775",

language = "English (US)",

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T1 - Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS

AU - Arpan, Ishu

AU - Willcocks, Rebecca J.

AU - Forbes, Sean C.

AU - Finkel, Richard S.

AU - Lott, Donovan J.

AU - Rooney, William D.

AU - Triplett, William T.

AU - Senesac, Claudia R.

AU - Daniels, Michael J.

AU - Byrne, Barry J.

AU - Finanger, Erika L.

AU - Russman, Barry S.

AU - Wang, Dah Jyuu

AU - Tennekoon, Gihan I.

AU - Walter, Glenn A.

AU - Sweeney, H. L.

AU - Vandenborne, Krista

PY - 2014/9/1

Y1 - 2014/9/1

N2 - 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.

AB - 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.

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

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