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 Eijk^1,4, 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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