CLN3 disease displays a “top-down” propagation of disease related atrophy—starting with abolishment of ERG in the last half of the first decade, followed by cerebral and cerebellar atrophy in early adolescence and loss of postural tone and affection of the peripheral nerves in late adolescents/early twenties, with the extrapyramidal system affected during this time course. The gene discussed is CLN3; the disease is Atrophy.