Definition

Batten disease is a disorder of the nervous system that begins in childhood. Symptoms of the disorder include mental impairment, seizures, and loss of sight and motor skills.

Description

Batten disease is characterized by an abnormal buildup of lipopigments—substances made up of fats and proteins—in bubble-like compartments within cells. The compartments, called lysosomes, normally take in and break down waste products and complex molecules for the cell. In Batten disease, this process is disrupted, and the lipopigments accumulate. This breakdown is genetic. It is marked by vision failure and the loss of intellect and neurological functions, which begin in early childhood.

Batten disease is a form of a family of progressive neurological disorders known as neuronal...