About Batten's Disease

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 condition is named after the British paediatrician who first described it in 1903. Batten disease is relatively rare occurring in about one in 30000 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.

Infantile - onset between 6 months and 2 years, rapidly progressing with seizures, dementia, blindness and a severe loss of neurones. Death normally occurs in mid childhood.

For more information:

http://www.bdfa-uk.org.uk/downloads/bdfainfantileleaflet_1.pdf