Rare hereditary metabolic disease with peripheral neuropathy Disorder of ornithine metabolism Gyrate atrophy of choroid and retina UMLS:C0599035 HOGA MESH:C537132 orphanet Ornithine aminotransferase deficiency ICD10:E72.4 Hyperornithinemia - gyrate atrophy of choroid and retina OMIM:258870 Hyperornithinemia Hyperornithinemia is an inborn disorder of ornithine metabolism due to mitochondrial ornithine aminotransferase deficiency.The main clinical manifestation of the disease is gyrate atrophy of the choroid and retina that begins during childhood with myopia and night blindness, followed by concentric shrinking of the visual field (tunnel vision) and a peculiar aspect of retinopathy on the funduscopy. The electroretinogram soon goes flat. Patients often develop subcapsular posterior cataract between the ages of 10 and 20 and become virtually blind between the ages of 40 and 50. Most patients have normal intelligence. Unclassified familial retinal dystrophy Metabolic disease with cataract Metabolic disease with pigmentary retinitis