Abstract

Retinal degeneration is a major finding in the human and ovine ceroid lipofuscinosis. Sequential electroretinographic (ERG) studies in a young, asymptomatic, affected lamb are presented here, which demonstrate a progressive loss of the scotopic b-wave and unrecordable c-waves under halothane anesthesia. Even at this initial stage of disease, lesions were evident in the form of dystrophic retinal pigment epithelial (RPE) villi, and loss of photoreceptor cells and rod outer segments. Cone inner segments were enlarged and scanning electron microscopy emphasized these abnormalities. All cells in tapetal and nontapetal areas contained fluorescent inclusions with similar emission spectra (maximum = 539 nm). By transmission electron microscopy, storage bodies consisted of 'finger-print' profiles and were most prominent in bipolar cells. The pathological features of the retina correlate well with the observed ERG changes, reaffirming the sheep as a useful model to delineate early events in ceroid lipofuscinosis.

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call

Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.