Cerebromacular degeneration, or the juvenile form of amaurotic familial idiocy, was first described by Batten1 in 1903. The clinical features of this disease were progressive loss of the intellectual faculties, loss of vision and loss of motor power. This condition differed from TaySachs disease (infantile amaurotic familial idiocy) by the fact that cases did not occur predominantly in the Jewish race, that blindness was not associated with the same typical picture of the fundus, that its first clinical manifestations appeared at a later age and that the progress was slower. In 1905 Vogt2 described the morbid anatomy, and Spielmeyer,3 in the same year, reported cases of a condition resembling retinitis pigmentosa in which the pigmentary changes were thought at first to be a complication of the disease.
In 1908 Stock4 first published the histologic changes in the eye in 3 of Spielmeyer's original cases. He noted
GIVNER I, ROIZIN L. JUVENILE AMAUROTIC FAMILIAL IDIOCY: ITS OCULAR PATHOLOGY. Arch Ophthalmol. 1944;32(1):39–47. doi:10.1001/archopht.1944.00890070057006
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