Szemészet, 2003 (140. évfolyam, 1-4. szám)
2003-12-01 / 4. szám
56 Szemészet követően is, ezért a pontos nyomon követés és a panaszok fokozódása esetén az immunválasz befolyásolása válhat szükségessé. Delayed visual loss due to paraneoplastic retinopathy: case report Hargitai J.,1 Farkas Á.,1 Fiedler O.,1 Thirkill C.E.,2 Salacz Gy.1 'Semmelweis University, 2nd Department of Ophthalmology, Budapest 2University of California, Department of Ophthalmology, Davis, USA Introduction: We describe an unusual case of cancer-associated retinopathy. A 52 year old patient with the history of left upper lobectomy due to lung cancer was referred to our Department after he noticed a gradual decrease of vision over a period of two years. Methods: General ophthalmic testing (visual acuity, biomicroscopy, retinoscopy, visual field test and colour-vision test), and electrophysiology measurements (full-field and multifocal ERGs) were performed. The patient’s sera was evaluated for abnormal antibody activity by indirect immunohistochemistry on sectioned rhesus monkey eye and and by Western blot analysis on an extract of Rhesus monkey retina. Results: Marked decrease of vision was present in both eyes (acuity 0.16). The anterior segment was unremarkable, but there were two small chorioretinal scars on both fundi outside the fovea. Visual-field testing revealed a generalised severe depression in both eyes. Colour vision was normal. Full-field and multifocal ERGs showed highly abnormal responses. Antibody activity on sectioned rhesus monkey eye revealed a selective staining of the ciliary body and the retina’s outer plexiform layer. Western blot on an extract of rhesus monkey retina identified abnormal antibody activity focused on a 40 kD molecular weight protein. Conclusions: The observed abnormal immunologic activity is consistent with that of the 40 kD CAR syndrome. Paraneoplastic retinopathy may persist years after the definitive treatment of the tumour. Therefore careful follow-up is required, and in some cases modulation of the immune response may be necessary. Usher-szindróma ritka esete Németh Gábor, Nagy Valéria, Sípos István, Berta András Debreceni Egyetem OEC, Szemklinika Az Usher-szindróma igen ritka genetikai betegség, mely kétoldali idegi eredetű halláscsökkenés és retinitis pigmentosa együttes előfordulása jellemez. Célunk egy súlyos eset ismertetésén, genetikai vizsgálatán keresztül bemutatni e ritka szindróma típusait, szemészeti vonatkozásait és differenciáldiagnózisát. A rare case of Usher syndrome Németh G., Nagy V., Sipos L, Berta A. University of Debrecen, Department of Ophthalmology Usher syndrome is a rare genetic disease characterised by bilateral neural hearing loss and retinitis pigmentosa. Our aim is to present this rare syndrome’s types, ophthalmological signs, and methods for differential diagnosis, as exemplified in a severe case with its associated genetic findings. 2003 augusztus 29. - Retina 4. szekció
