PPT1 and neuronal ceroid lipofuscinosis: The EEG background abnormalities and pronounced spontaneous seizure phenotypes in Cln2R207X mice revealed by our longitudinal EEG recording are progressive in nature and comparable to those in mice modeling infantile NCL or CLN1 disease (Cln1–/– mice) and other genetic epilepsy models, such as tuberous sclerosis complex 1 mice (13, 44, 45).