Abstract
BackgroundDiffusion tensor imaging (DTI) can identify amyotrophic lateral sclerosis (ALS)-associated patterns of brain alterations at the group level according to a neuropathological staging system. ObjectiveThe study was designed to investigate the in vivo staging in ALS patients with the C9orf72 expansion and potential differences to ALS patients with the SOD1 mutation. MethodsDTI-based white matter mapping was performed both by an unbiased voxel-wise statistical comparison and by a hypothesis-guided tract-wise analysis of fractional anisotropy (FA) maps according to the ALS-staging pattern for 27 ALS patients with C9orf72 expansion vs 15 ALS patients with SOD1 mutation vs 32 matched healthy controls. Clinical and neuropsychological data were acquired and correlated to DTI data. ResultsThe analysis of white matter integrity demonstrated regional FA reductions along the CST and also in frontal and prefrontal brain areas according to the proposed propagation pattern for the ALS patients with C9orf72 expansion and sporadic patients. This pattern could not be identified for the SOD1 mutation at the group level. In contrast, in the tract-specific analysis according to the neuropathological ALS-staging pattern, C9orf72 expansion ALS patients showed significant alterations of ALS-related tract systems similar to sporadic patients. ConclusionsThe DTI study including the tract-of-interest-based analysis showed a microstructural corticoefferent involvement pattern according to the staging scheme in C9orf72-associated ALS patients but not in the SOD1 mutation.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.