Batten disease is relatively rare occurring in about one in 30,000 births. The illness leads to a progressive deterioration of the brain and nervous system. Although our understanding of Batten disease is improving all the time, there is at present no cure or treatment that has any significant impact on the inexorable decline in bodily functions and inevitable early death. A number of different forms of Batten Disease have been identified. They are classified by age but are all genetically different.
Death normally occurs in mid childhood.
Death normally occurs between the ages of 8 and 16. Juvenile - onset between 5 and 9 years, characterised by visual loss initially, and later seizures, loss of motor abilities and dementia. Death may occur any time from the late teens to the mid thirties. Age of death is variable but life expectancy is shortened. In addition, there are several other less common variant forms.