Tay-Sachs disease is rare in the general population. The genetic mutations that cause this disease are more common in people of Ashkenazi (eastern and central European) Jewish heritage than in those of other backgrounds. Approximately 1 in every 27 Jews in the United States is a carrier of the
Tay-Sachs disease gene. In recent years, however, screening for mutations and genetic counseling (including prenatal diagnosis) has made the condition much less frequent in this population.
The genetic mutations that cause Tay-Sachs disease are also more common in certain French-Canadian communities of Quebec, some Amish groups, and the Cajun community of Louisiana.
For the general population, about 1 in 250 people are carriers of the Tay-Sachs disease gene.