Batten disease is also called Spielmeyer-Vogt-Sjogren-Batten Disease. This congenital disorder
(it is an autosomal recessive inherited
disorder) strikes between the ages of 5 and 10, causing the afflicted child to suddenly go blind
. The child begins to suffer from seizure
s and mental deterioration
; afflicted individuals typically die before the age of 20.
This disorder is the most common type of a class of diseases called neuronal ceroid lipofuscinoses. The disease is caused by the accumulation of toxic proteins in nerve cells; the root of the disorder is a mutation of a gene on chromosome 16.
The disorder was first described 1826 by Dr. Christian Stengel in a Norwegian medical journal; however, it did not become widely known to the medical community until it was written about by a physician named F. E. Batten in 1903.
For more information, visit http://www.bdsra.org/