Abstract #2567

MRI Characterization of Cerebellar Atrophy in a Non-Human Primate Model of Neuronal Ceroid Lipofuscinosis

Ian Tagge¹, Lois Colgin², Rebecca Ducore², Betsy Ferguson³, Steven Kohama⁴, Jodi McBride⁴, Martha Neuringer⁴, Sam Peterson⁴, Scott Wong⁵, Robert Zweig⁶, and Anne Lewis²

¹Advanced Imaging Research Center, Oregon Health & Science University, Portland, OR, United States, ²Pathology, Oregon National Primate Research Center, ³Primate Genetics, Oregon National Primate Research Center, ⁴Oregon National Primate Research Center, ⁵VGTI, Oregon National Primate Research Center, ⁶Clinical Medicine, Oregon National Primate Research Center

Neuronal ceroid lipofuscinosis (NCL; also known as Batten Disease) is a fatal neurodegenerative disorder that typically presents in childhood. Currently, no treatments are known that can halt or reverse the effects of NCL. A naturally occurring form of NCL analogous to late infantile-onset NCL in humans has been identified in a population of Japanese macaques (JMs). MRI examinations revealed marked cerebellar degeneration in NCL animals >4y/o (~14y/o equivalent human age) compared with controls, which is strikingly similar to human disease. This novel JM model presents a new opportunity for characterizing disease progression, identifying biomarkers, and pre-clinical therapeutic testing.

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