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A Journal on Ophthalmology
Minerva Oftalmologica 2007 December;49(4):193-6
Congenital stationary night blindness: clinical features and differential diagnosis
Gesualdo C., Solimeo A., Maggio L., Maggio E., Testa F.
Dipartimento di Oftalmologia Seconda Università degli Studi di Napoli, Napoli
Congenital stationary night blindness (CSNB) is a group of non progressive retinal disorders characterised by impaired night vision that occurs in autosomal dominant, autosomal recessive or X-linked forms. The purpose of this study was to describe the clinical features in a patient, excluding other forms of congenital stationary night blindness such as fundus albipuntatus and Oguchi’s disease, and other similar retinal disease, such as retinitis pigmentosa and retinoschisis X-linked. The patient affected by congenital stationary night blindness was followed clinically and genetically at the Retinal Degenerations Center of the Second University of Naples. The patient underwent a complete ophthalmologic evaluation including ocular anamnesis, Ishihara color test, best-corrected visual acuity by Snellen charts, slit-lamp biomicroscopy of anterior segment, dilated fundus examination, Goldmann visual field evaluation, fundus photography, optical coherence tomography (OCT) and electroretinography (ERG). The only significant symptoms were night blindness associated with myopia. The ophthalmic examination showed myopic dystrophy in both eyes. The visual field area was light restricted. ERG measurements showed a reduction for the scotopic component, while the maximal component was negative with a/b inversion. The photopic component was normal. In conclusion, this study highlights the importance to know the main features of congenital stationary night blindness in order to diagnose it correctly and to distinguish it from other similar degenerative retinal diseases.