The childhood forms of the disease are autosomal regressive and represent one of the largest groups of progressive neurodegenerative diseases in children. It has been suggested that they are the most common group of progressive neurodegenerative disorders of childhood in Western countries. The characteristic features of these diseases are retinal and brain atrophy and the accumulation, in neurons and other cells, of fluorescent storage bodies. These fluorescent storage bodies or lipopigments build up in cells within the brain, eye and other tissues, causing curvilinear-crescent shaped, fingerprint shaped and granual osmophilic shaped deposits that are recognisable under a light microscope. A number of different forms of NCL have been identified.
Death normally occurs in mid childhood.
Death normally occurs between the ages of 8 and 12. Juvenile - onset between 5 and 12 years, characterised by developmental regression, leading, amongst other things, to vision loss, seizures, loss of motor abilities and dementia. Death may occur any time from the late teens to the early thirties. Age of death is variable but life expectancy is shortened. This form of the disease may be inherited through the dominant mode rather than the recessive mode of the other forms.