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.