Kufs disease is a late-onset variant of the neuronal ceroid lipofuscinoses (NCLs). There are four types of NCL, all of which have begun to be referred to as Batten disease. NCLs are inherited neurodegenerative storage disorders involving the accumulation of excessive abnormal lipo-pigments with fingerprint or granular ultrastructural patterns in the neurons and other cells throughout the central nervous system. This disease results in motor disturbances and neurological deficits that range in severity.
An adult-onset, neuronal ceroid lipofuscinosis. The onset of symptoms is usually between 21 and 26 years, and always before 40. The disease is characterized by the development of dementia, myoclonic jerks, blindness, and retinitis pigmentosa.