Inheritance is autosomal recessive. A progressive, inherited disease of the nervous system more commonly found in individuals of Eastern European Jewish and French-Canadian ancestry. Individuals who are affected are unable to break down a particular fat within the cells of the brain. As the fat builds up, damage to cells of the brain occur in the first year of life and the condition is usually fatal in early childhood. Rare, mild adult-onset forms also exist.
Inheritance is autosomal dominant.