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These often are referred to as juvenile, chronic and adult-onset forms of hex A deficiency. Affected individuals have low levels of the hex A enzyme that is missing entirely in the classical, infantile form. This may help explain why symptoms begin later in life and, generally, are milder than in the classical, infantile Tay-Sachs disease...

This may help explain why symptoms begin later in life and, generally, are milder than in the classical, infantile Tay-Sachs disease. Children with juvenile hex A deficiency develop symptoms between the ages of 2 and 5 that resemble those of the classical, infantile form. Although the course of the disease is slower, death generally occurs by age 15...

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