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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.
GLOSSARY
- 6MWT=
- 6-minute walk test;
- CHOP=
- Children's Hospital of Philadelphia;
- DMD=
- Duchenne muscular dystrophy;
- Gra=
- gracilis;
- MG=
- medial gastrocnemius;
- MRS=
- magnetic resonance spectroscopy;
- OHSU=
- Oregon Health & Science University;
- Per=
- peroneals;
- Sol=
- soleus;
- STEAM=
- stimulated echo acquisition mode;
- TA=
- tibialis anterior;
- TP=
- tibialis posterior;
- TE=
- echo time;
- TR=
- repetition time;
- UF=
- University of Florida;
- VL=
- vastus lateralis
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Editorial, page 956
Supplemental data at Neurology.org
- Received February 19, 2014.
- Accepted in final form June 3, 2014.
- © 2014 American Academy of Neurology
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