Batten Disease

Batten disease, also called Spielmeyer-Vogt-Sjogren-Batten disease or juvenile Batten disease, rare and fatal neurodegenerative disease that begins in childhood. The disease is named for British physician Frederick Batten, who in 1903 described the cerebral degeneration and macular changes characteristic of the condition. Batten disease is among the most commonly occurring of the neuronal ceroid lipofuscinoses (NCLs), a group of inherited lysosomal storage disorders, the primary feature of which is progressive neurodegeneration with cognitive decline, loss of motor ability, worsening seizures, and early death.