The group of diseases known as Batten disease or the neuronal ceroid lipofuscinoses (NCL) are progressive degenerative, genetic metabolic diseases that occur in children and adults.
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.
Juvenile – onset between 5 and 9 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 mid thirties.