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
seizures 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.
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