Retinal function in Bothnia dystrophy. An electrophysiological study

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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. © 2003 Elsevier Ltd. All rights reserved.




Burstedt, M. S. I., Sandgren, O., Golovleva, I., & Wachtmeister, L. (2003). Retinal function in Bothnia dystrophy. An electrophysiological study. Vision Research, 43(24), 2559–2571.

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