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January 1969

Tay-Sachs' Disease

Author Affiliations

Durham, NC
From the Divisions of Neurosurgery and Neurology, Duke University Medical Center, and the Durham Veterans Administration Hospital, Durham, NC.

Arch Neurol. 1969;20(1):103-104. doi:10.1001/archneur.1969.00480070113013

OUR KNOWLEDGE of the amaurotic family idiocies, and especially of Tay-Sachs' disease, has increased during the years in stepwise fashion.1,2

In 1881, Waren Tay, an English ophthalmologist, first described the macular changes that were subsequently associated with this condition.3 Tay later described similar changes in two siblings of the infant first reported.4 Then in 1887, Bernard Sachs, a neurologist in New York, published the original clinical and pathological description of the disease.5 In studying additional cases, he noted the familial nature of the condition, which he called amaurotic family idiocy.6-8 Other physicians published case reports, and Tay-Sachs' disease was established as an entity. Subsequently, this fatal disorder was found to be an autosomal recessive trait, occurring primarily, but not exclusively, in Jewish families from eastern Europe.2,9

Early in the twentieth century, other types of cerebroretinal degeneration were described in older patients by