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      Electro-oculography in autosomal dominant vitreoretinochoroidopathy.

      Archives of ophthalmology (Chicago, Ill. : 1960)
      Adult, Choroid Diseases, genetics, physiopathology, Dark Adaptation, Electrooculography, Electroretinography, Eye Diseases, Female, Fluorescein Angiography, Fundus Oculi, Humans, Light, Male, Middle Aged, Pedigree, Retinal Diseases, Retinitis Pigmentosa, Vitreous Body

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          Abstract

          Thirteen members of a family presumed to be harboring the gene for autosomal dominant vitreoretinochoroidopathy were examined. In four affected members, electro-oculography demonstrated marked reduction of the Arden ratio (range, 1.1 to 1.5; normal, > or = 1.8), despite electroretinographic evidence of mildly affected rod function and normal cone function. These findings suggested that a diffuse disturbance of the photoreceptor-pigment epithelium complex may have been present prior to wide-spread loss of photoreceptor function in the affected members of this family. As in previously described families, the pattern of inheritance appeared consistent with autosomal dominance.

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