T2 mapping in Duchenne muscular dystrophy: distribution of disease activity and correlation with clinical assessments

Abstract

Purpose: To analyze T2 maps of pelvic and thigh muscles in Duchenne muscular dystrophy (DMD), to identify the most severely affected muscle, and to correlate the T2 of muscle with the grade of fatty infiltration at nonquantitative magnetic resonance (MR) imaging and results of clinical assessment.

Materials and methods: This prospective study was HIPAA compliant and was approved by the institutional review board; written consent was obtained from all participants' parents or guardians. Thirty-four boys with DMD (mean age, 8.4 years) were evaluated clinically (age, clinical function score, timed Gower score, time to run 30 feet, and serum creatine kinase [CK] level) and with nonquantitative MR imaging and axial T2 mapping from the iliac crest to the mid thigh. The T2 maps and mean T2 of 18 muscles in the pelvis and thighs were analyzed to identify the most severely involved muscle. The amount of fatty infiltration was assigned a grade of zero to four for all pelvic and thigh muscles by using T1-weighted nonquantitative MR images. The Spearman correlation coefficients model was used to correlate the mean T2, nonquantitative MR imaging score and clinical assessments.

Results: The gluteus maximus muscle had the highest T2. The mean T2 for this muscle showed a significant correlation with the nonquantitative MR imaging score for fatty infiltration (P < .001) and with all clinical assessments except CK level.

Conclusion: Gluteus maximus muscles are most severely affected in patients with DMD. The T2 of the gluteus maximus muscle can be used as a quantitative and objective measure of disease severity.