Juvenile Batten disease is caused by mutations in the CLN3 gene, commonly a 1-kb deletion encompassing exons 7 and 8, that result in the loss of full-length CLN3 protein (The International Batten Disease Consortium, 1995; Munroe et al., 1997; Kitzmüller et al., 2008). This evidence concerns the gene CLN3 and juvenile neuronal ceroid lipofuscinosis.