[Gyrate atrophy with macular dystrophy].
In a case of gyrate atrophy with hyperornithinemia a macular dystrophy was found. The ERG amplitude was markedly decreased whereas the b-wave implicit time was found to be normal. There was an obvious parallel between the extent of the peripheral and central alterations of the retina in both eyes. It may be assumed that in this case of gyrate atrophy the remaining retinal areas suffered little damage as far as qualitative parameters are concerned. Macular dystrophy may be one form of manifestation of the underlying disease.
D000328:Adult; D001284:Atrophy; D002829:Choroid / Q000473:pathology*; D004596:Electroretinography; D005451:Fluorescein Angiography; D006801:Humans; D008268:Macular Degeneration / Q000175:diagnosis* / Q000201:enzymology; D008297:Male; D009953:Ornithine-Oxo-Acid Transaminase / Q000172:deficiency*; D000637:Transaminases / Q000172:deficiency*; D014792:Visual Acuity; D014794:Visual Fields
D000637:Transaminases; D009953:Ornithine-Oxo-Acid Transaminase
Atrophia gyrata mit Makuladystrophie.