MRI correlates of motoneuron loss in SMA

• DOI: 10.1007/s00415-022-11326-1
• Publication Date: 2022-10-01T03:50:01Z
• Authors: Alina Sprenger-Sv...; Johannes Haensch; Kilian Weiss; Nils Große Hokamp; David Maintz; Marc Schlamann; Gereon R. Fink; Natalie Schloss; Kai Laukamp; Gilbert Wunderlich; Helmar C. Lehmann; Thorsten Lichtens...
• Keywords: Neuroradiology; Statistical parametric mapping; Supplementary motor area; Intramuscular fat

Magnetic resonance imaging (MRI) is currently explored as supplemental tool to monitor disease progression and treatment response in various neuromuscular disorders. We here assessed the utility of a multi-parametric magnetic protocol including quantitative water T2 mapping, Dixon-based proton density fat fraction (PDFF) estimation diffusion tensor (DTI) detect loss spinal motor neurons subsequent muscle damage adult SMA patients.Sixteen patients 13 age-matched controls were enrolled this prospective, longitudinal study. All participants underwent MRI measurements PDFF DTI sciatic nerve. furthermore (T2w) biceps femoris (BFM) quadriceps (QFM). Ten returned for second scan six months later. parameter correlated with clinical data. on nusinersen treatment.There significantly higher intramuscular fractions BFM QFM compared healthy at baseline after 6 months. Furthermore, values positively fractions. The Hammersmith functional scale QFM's scans nerve not different between two groups.This study demonstrates that, mapping may distinguish controls, due massive accumulation SMA. More extensive long-term studies are warranted further evaluate these modalities surrogate markers during treatment.