Retinal function in Bothnia dystrophy. An electrophysiological study.

• 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.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Oftalmologi (hsv//swe)

MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Ophthalmology (hsv//eng)

Keyword

etinal pigment epithelium; Retinitis pigmentosa

Neural retina

Electroretinogram

Cellular retinyl aldehyde-binding protein (CRALBP)

Ophtalmiatrics

Oftalmiatrik

Ophtalmology

oftalmiatrik

Publication and Content Type

ref (subject category)

art (subject category)