Batten Disease Definition & Overview

Definition

Batten disease is the most common form of a group of rare disorders known as neuronal ceroid lipofuscinoses (NCLs). Batten disease is an inherited genetic disorder that causes a build-up of lipopigments in the body’s tissue. Batten disease refers to the juvenile form of NCL, but the other forms of NCL can also be referred to as Batten disease. About 2-4 of every 100,000 births are affected. The forms of NCL include:

Infantile NCL
Late infantile NCL
Juvenile NCL
Adult NCL

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