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Abstract #2672

Probing Cerebello-Cerebral Functional Connectivity in Spinocerebellar Ataxias

Sheeba Anteraper1, Ying Zhang2, Xavier Guell3, Michal Povazan4, Guita Banan5, Romain Valabregue6, Philip Ehses7, Jennifer Faber7, James M Joers2, Chiadi U Onyike4, Peter B Barker4, Jeremy D Schmahmann3, Eva-Maria Ratai3, S H Subramony5, Thomas H Mareci5, Khalaf O Bushara2, Alexandra Durr6, Thomas Klockgether7, Tetsuo Ashizawa8, Christophe Lenglet2, and Gülin Öz2
1Carle Foundation Hospital, Champaign, IL, United States, 2University of Minnesota, Minneapolis, MN, United States, 3Massachusetts General Hospital, Boston, MA, United States, 4Johns Hopkins University, Baltimore, MD, United States, 5University of Florida, Gainesville, FL, United States, 6Sorbonne University, Paris, France, 7German Center for Neurodegenerative Diseases, Bonn, Germany, 8The Houston Methodist Research Institute, Houston, TX, United States


Spinocerebellar Ataxias (SCAs) are a group of rare, autosomal dominant diseases that result in progressive degeneration of the cerebellum. Of these, SCA1 is the fastest progressing. SCA3, the most prevalent SCA worldwide, is relentlessly progressive, disabling, and eventually fatal with no efficacious treatments other than supportive therapy. There is a strong need to improve our mechanistic understanding of the changes in the cerebello-cerebral circuitry prior to disease manifestation so that novel therapeutic strategies can be developed. Analyzing high quality magnetic resonance imaging data from the NIH-funded project, “Clinical Trial Readiness for SCA1 and SCA3 (READISCA),” will guide such efforts.

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