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Abstract
Objective To investigate the spatial patterns and the probable sequences of gray matter atrophy in spinocerebellar ataxia type 3 (SCA3).
Methods A total of 47 patients with SCA3 and 49 age- and sex-matched healthy controls participated in the study. High-resolution T1-weighted MRI were examined in all participants. We used the causal network of structural covariance (CasCN) to identify the sequence of gray matter atrophy patterns. This was achieved by applying Granger causality analysis to a gray matter atrophy staging scheme performed by voxel-based morphometry from the network level.
Results Participants in the premanifest stage of the disease showed the presence of focal gray matter atrophy in the vermis. As the disease duration increased, there was progressive gray matter atrophy in the cerebellar, neostriatum, frontal lobe, and parietal lobe. The patients with SCA3 also showed proximal and distal cortical atrophy sequences exerting from the vermis to the regions mainly located in the cerebellum-neostriatum-cortical network.
Conclusion Our results, although preliminary in nature, indicate that the gray matter atrophy in SCA3 lies and extends to involve more regions according to distinct anatomical patterns, mainly in the cerebellum-neostriatum-cortical network. These findings advance our understanding on the natural history of structural damage in SCA3, while confirming known clinical features. This could provide unique insight into the ordered sequential process of regional brain atrophy that targets a particular network.
Glossary
- CaSCN=
- causal structural covariance network;
- FDR=
- false discovery rate;
- GC=
- Granger causality;
- GCA=
- Granger causality analysis;
- GM=
- gray matter;
- MNI=
- Montreal Neurologic Institute;
- ROI=
- region of interest;
- SARA=
- Scale of Assessment and Rating of Ataxia;
- SCA3=
- spinocerebellar ataxia type 3;
- VBM=
- voxel-based morphometry
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
- Received November 28, 2019.
- Accepted in final form July 22, 2020.
- © 2020 American Academy of Neurology
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