CLN6 and juvenile neuronal ceroid lipofuscinosis: 2009), whereas glial activation and neurodegeneration generally begins in the upper cortex in large animal models of Batten disease (best described in CLN6 sheep (Oswald et al. 2005, 2008)) and human Batten disease patients, with the cerebellum remaining largely spared. In contrast, the neuropathological changes we report here suggest similar regional targeting in CLN5 mice (von Schantz et al. 2009) and sheep.