Recent studies in the Cln3−/− and Cln3Δex7/8 mouse models indicate that juvenile Batten disease has a neurodevelopmental component resulting in early disease phenotypes (at ≤1 month of age) in these mice (Osório et al., 2009; Weimer et al., 2009). This evidence concerns the gene CLN3 and juvenile neuronal ceroid lipofuscinosis.