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February 1959

Amaurosis Congenita (Leber)

Author Affiliations

The Hague; Rotterdam; Leyden, Netherlands
Eye Clinic, Rotterdam (Dr. Henkes); Human Geneticist of the Netherlands Institute for Preventive Medicine, Leyden (Dr. van den Bosch).

AMA Arch Ophthalmol. 1959;61(2):211-218. doi:10.1001/archopht.1959.00940090213003

Introduction  In 1869 Leber,1 at the time consulting ophthalmologist of the Ilvesheim Institute for the Blind, described a type of blindness occurring at birth or soon after. In some of the affected children residual vision was found. In most of the cases a marked nystagmus was present and photophobia was recorded in spite of the extremely poor visual function. Even in the few cases in which the visual function was less defective, it was obvious that the pupil reactions were slow and incomplete. In some of the cases of complete blindness, however, a rudimentary pupil reaction was observed.In those cases having some residual vision, the visual fields were in most instances not markedly contracted. In some of the lessaffected children a marked degree of night blindness was recorded. In the first months of life it was not possible to observe essential alterations in the fundus oculi. In a

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