Gyrate atrophy of the choroid and retina: ERG of the neural retina and the pigment epithelium.
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چکیده
منابع مشابه
Gyrate atrophy of the choroid and retina: ERG of the neural retina and the pigment epithelium.
The function of the neural retina and the pigment epithelium in 10 patients with gyrate atrophy was examined by the conventional alternating current electroretinogram and by direct current electroretinography to study the c-wave. The a- and b-wave responses were subnormal in all patients and the ERG was undetectable in patients with an advanced stage of the disease. The c-wave was recordable by...
متن کاملGyrate Atrophy of the Choroid and Retina: A Case Report
Introduction Gyrate atrophy of the choroid and retina is a metabolic disorder, which is inherited in an autosomal recessive pattern. Although gyrate atrophy is rare, it is concerning as it results in blindness. It is characterized by hyperornithinemia, retinal atrophy, leads to progressive myopia and tunnel vision,...
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The inheritance of the autosomal recessive type of gyrate atrophy of the choroid and retina seems well documented (Botermans, I 972), but the aetiology of the disease is still unknown. Animal experiments and some disturbances of lipid, carbohydrate, and protein metabolism connected with chorio-retinal degenerations in man support the possibility of an enzymatic disorder as an aetiological facto...
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An adult male, domestic short-haired cat with generalized retinal atrophy was found to have a 60-fold increase in plasma ornithine and ornithinuria. Ornithine-8-aminotransferase activity was undetectable in its tissues and in its cultured skin fibroblasts. This feline condition is thus analogous to gyrate atrophy of the choroid and retina in humans.
متن کاملGyrate atrophy of the choroid and retina with hyperornithinemia.
A case of gyrate atrophy of the choroid and retina and hyperornithinemia in a 28-year-old man was subjected to extensive clinical and biochemical investigation. The familial occurrence of the ocular disease and of abnormality of amino acids was unique to this patient, being absent in parents and siblings. He presented with progressive visual loss, and had cataracts and large areas of peripheral...
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ژورنال
عنوان ژورنال: British Journal of Ophthalmology
سال: 1990
ISSN: 0007-1161
DOI: 10.1136/bjo.74.6.363