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Abstract #0344

Quantitative MRI of skeletal muscle in a cross-sectional cohort of spinal muscular atrophy type 2 and type 3

Louise A.M. Otto1, Ludo W.L. van der Pol1, Lara Schlaffke 2, Camiel A. Wijngaarde1, Marloes Stam1, Renske I. Wadman1, Inge Cuppen3, Ruben P.A. van Eijk1,4, Fay-Lynn Asselman1, Bart Bartels5, Danny van der Woude5, Jeroen Hendrikse6, and Martijn Froeling6
1Neurology, UMC Utrecht Brain Center, University Medical Center, Utrecht, Utrecht, Netherlands, 2Neurology, BG-University Hospital Bergmannsheil, Ruhr-University Bochum, Bochum, Germany, 3Neurology and Child Neurology, UMC Utrecht Brain Center, University Medical Center, Utrecht, Utrecht, Netherlands, 4Biostatistics & Research Support, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, Netherlands, 5Child Development and Exercise Center, UMC Utrecht Brain Center, University Medical Center, Utrecht, Utrecht, Netherlands, 6Radiology, UMC Utrecht Brain Center, University Medical Center, Utrecht, Utrecht, Netherlands

qMRI of skeletal muscle has shown promising results in other neuromuscular diseases, but multi-parametric imaging has not been executed in Spinal Muscular Atrophy. We investigated a cohort of 31 patients and 20 controls with protocol consisting of DIXON, T2 mapping and DTI on a 3T MR scanner. All parameters differed significantly between patients and controls. DTI elucidates distinct properties of the muscle, suggesting atrophy by a lowered MD and increased FA. DTI shows correlation with muscle strength and motor function. This suggests the potential of diffusion tensor imaging of muscle in monitoring disease progression in SMA.

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