Retinal function in Bothnia dystrophy. An electrophysiological study.
2003 (English)In: Vision Research, ISSN 0042-6989, E-ISSN 1878-5646, Vol. 43, no 24, 2559-2571 p.Article in journal (Refereed) Published
Using prolonged dark adaptometry, standard dark adaptation (DA) and prolonged DA full-field electroretinograms (ERGs), we analysed the retinal function in patients with Bothnia dystrophy (BD), a variant of recessive retinitis punctata albescens (RPA). A compromised rod and cone function, a likely dysfunction of the Müller cells, and indications of disturbed neuronal function of the inner retina, were found. With prolonged DA, a gradual increase in retinal sensitivity to light and an improvement of the ERG components occurred. The findings indicate a prolonged synthesis of photopigments, retardation of the visual process in the retinal pigment epithelium (RPE), and a loss of retinal cells, probably starting at a relatively early age in BD.
Place, publisher, year, edition, pages
2003. Vol. 43, no 24, 2559-2571 p.
etinal pigment epithelium; Retinitis pigmentosa, Neural retina, Electroretinogram, Cellular retinyl aldehyde-binding protein (CRALBP)
Research subject Ophtalmology
IdentifiersURN: urn:nbn:se:umu:diva-34334DOI: 10.1016/S0042-6989(03)00440-1PubMedID: 13129542Local ID: 744OAI: oai:DiVA.org:umu-34334DiVA: diva2:320674