van den Bosch the Hague (Will be published in extenso in Archives of Ophthalmology.) In spite of recent publications by Waardenburg, Alström and Olson, Franceschetti, and Henkes and Schappert, this pathological picture, which was first described by Leber, is seldom recognized, it is, however, by no means infrequent. The investigation into the causes of blindness in the Netherlands concerned 227 cases of congenital amaurosis, viz. 114 adult blind patients and 113 children of whom 109 were blind and 4 were partially sighted. Since the Netherlands have approximately 6000 blind persons, 600 of whom are children, these figures mean that the affection is the cause of blindness in 3,8% of all blind persons and in 18% of the blind children. This affection was erroneously diagnosed by the consulting ophthalmologists as: tapjetoretinal degeneration, optic nerve atrophy, (congenital syphilitic) retinochoroiditis and congenital amblyopia (possibly of central origin). The following symptoms are obligatory: Congenital blindness or very poor central vision. In the majority of cases: only very slight fundus changes in comparison with the bad visual function. Absent or nearly absent electroretinogram. Recessive hereditary occurrence. Facultative characteristics are: Nystagmus-relatively often. Photophobia-much rarer. Cataract. Keratoglobus or keratoconus. Schappert-Kimmijser‚Henkes and van den Bosch 421The two latter symptoms are absent at birth. The affection is described in more detail with the aid of case histories, a characteristic fundus picture and electroretinograms and a first pedigree of 13 cases originating from the Isle of Urk. Discussion. Zeeman congratulated Mrs. Schappert on the collecting of such instructive experience regarding this affection, which, on account of apparent infrequency and poverty of symptoms offers great difficulties to the ophthalmologist, but which had been shown to be one of the main causes of blindness in children. The affection deserves our keen attention even after the valuable monograph by Alström. This latter publication seems unsurpassable as a genetic study. As

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