JUVENILE AMAUROTIC FAMILY IDIOCY

Abstract

Few degenerative diseases of the nervous system have received wider attention than amaurotic family idiocy (Tay-Sachs disease). The condition was believed at first to be limited to early infancy, but cases occurring in late infancy, in adolescence and even in adult life have been reported in increasing numbers. A characteristic type of this disease occurs in children between the ages of 5 and 15 years and is termed, accordingly, juvenile amaurotic family idiocy, or the Spielmeyer-Vogt type, since isolated cases were described by Spielmeyer,1in 1908, and by Vogt,2in 1906, although Batten3first recognized the disease in 1903. In 1931 an extensive report on the clinical and genetic aspects of juvenile amaurotic family idiocy was published by Sjogren,4whose material included 59 affected families observed in Sweden; the pathologic features of these cases were later reported on by Sjoval and Ericsson.5 Although numerous reports