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C PalittoS Cavarretta EMVingolo, A Chiaravalloti, C Teodori, S Cavarretta, EM Vingolo; Electroretinography findings in Goldmann-Favre Syndrome . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1182.
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Purpose: Evaluate clinical and functional heterogeneity in four pedigrees of Goldmann-Favre syndrome. Method: On 9 patients, 3 females and 6 males, Goldmann-Favre syndrome was assessed by following opthalmological examination including: visual acuity measurement, biomicroscopy of the anterior segment, ophthalmoscopy, fluorescein angiography, visual field testing and electroretinogram standard. Results: The clinical features are usually bilateral and simmetrical. The vitreous changes are degenerative :fibrillar strands,liquefactio and often posterior vitreous detachment. The fundus features include peripheral pigmentary retinopathy similar to retinitis pigmentosa,central or peripheral retinoschisis, cystoid macular edema,and in the later stage ,vascular abnormalities ( non perfusion,attenuated vessel) with optic disc pallor. Also posterior subcapsular cataract is common. In all patients ERG showed a negative shape. In all patients scotopic electroretinogram almost non recordable, photopic electroretinogram showed a marked reduction of the b-wave, but the a-wave almost normal amplitude. Conclusions: A diagnosis of Goldmann-Favre sindrome should be considered in patients presenting with an early history of poor night vision , bilateral atypical pigmentary changes of the retina, and degenerative changes in the vitreous humour. Electroretinography findings play a mayor rule in Goldmann-Favre syndrome diagnosis, above all in differential diagnosis,with retinitis pigmentosa, X-linked juvenile retinoschisis.
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