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The author has given in German a careful and detailed study from the clinical and the hereditary point of view of that form of juvenile idiocy which Vogt in 1906 differentiated from the better known infantile form of Tay-Sachs' disease. The typical clinical picture was that of one or more previously healthy children in one family, who became affected, generally during the school years, from the ages of 6 to 14. The first symptom is usually failing vision, which leads to complete blindness in the course of months. Several children of the same family may become affected in the same age year. The disease starts gradually. Ophthalmoscopic examination shows atrophic disks. Mental development is arrested and then retrogresses. The disease progresses little by little to deepest psychic dementia, marasmus and death in the course of from five to ten years. The motor functions also diminish steadily, leading finally to
Fridenberg P. Juvenile Amaurotic Idiocy. Research Concerning the Clinical and Hereditary Aspects.. Arch Ophthalmol. 1932;7(5):819–821. doi:10.1001/archopht.1932.00820120171019