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Abstract #2072

Longitudinal TBSS Reveals Progressing Demyelination in the Mouse Model of Progressive Neurodegenerative Disease EPM1

Otto H. H. Manninen1, Teemu Laitinen2, Outi Kopra1, Olli Grhn2, Anna-Elina Lehesjoki1

1Folkhlsan Institute of Genetics & Neuroscience Center, University of Helsinki, Helsinki, Finland; 2Department of Neurobiology, University of Eastern Finland, Kuopio, Finland

We studied white matter (WM)-changes in the neurodegenerative Unverricht-Lundborg-disease (EPM1) by performing longitudinal FA TBSS in the gene-targeted mouse model of the disease. After ex-vivo DTI, we did a TBSS comparison of FA between Cstb-/- mice and wildtypes at 2, 4 and 6 months of age. TBSS detected no significant changes at 2 months, with significant decrease in FA at 4 months at cerebellum and thalamus, that progressed into more drastic decrease at same areas at 6 months, suggesting demyelination as cause of WM-changes in EPM1.