Heterogeneity of muscle fat infiltration in children with spina bifida

Abstract

Children with spina bifida have well recognized functional deficits of muscle, but little is known about the associated changes in muscle anatomy and composition. This study used water-fat magnetic resonance imaging (MRI) to measure fat infiltration in the lower extremity muscles of 11 children with myelomeningocele, the most severe form of spina bifida. MRI measurements of muscle fat fraction (FF) were compared against manual muscle test (MMT) scores for muscle strength. The FF measurements were objective and reliable with mean inter-rater differences of <2% and intraclass correlation coefficients>0.98. There was a significant inverse relationship between muscle FF and MMT scores (P ≤ 0.001). Surprisingly, however, muscles with negligible strength (MMT 0-1) exhibited a bimodal distribution of FF with one group having FF>70% and another group having FF<20%. The MRI also revealed striking heterogeneity amongst individual muscles in the same muscle group (e.g., 4% fat in one participant's lateral gastrocnemius vs. 88% in her medial gastrocnemius), as well as significant asymmetry in FF in one participant with asymmetric strength and sensation. These results suggest that quantitative water-fat MRI may serve as a biomarker for muscle degeneration which may reveal subclinical changes useful for predicting functional potential and prognosis.

Keywords: Muscle fat fraction; Myelomeningocele; Spina bifida; Water-fat MRI.