Electrophysiology in some animal and human hereditary diseases involving the retinal pigment epithelium.

Abstract

The present paper surveys slow electrophysiological responses recorded by a d.c. technique in some hereditary eye diseases involving the retinal pigment epithelium (RPE) in animals (English setter dogs and Polish Owczarec Nizinny (PON) dogs with ceroid lipofuscinosis and Briard dogs with a slowly progressive rod-cone dystrophy associated with RPE inclusions) and in humans (Best's disease). The electroretinogram c-wave was typically either decreased in amplitude, lacking or replaced by a negative wave. These c-wave changes could be seen at fairly early stages of disease, when the a- and b-waves of the electroretinogram were still within normal limits.

• Authors: S E Nilsson, A Wrigstad
• Journal: Eye (London, England) (Eye (Lond)) Vol. 11 ( Pt 5) Pg. 698-706 ( 1997) ISSN: 0950-222X [Print] England
• PMID: 9474321 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adolescent
• Adult
• Animals
• Child
• Dog Diseases (genetics, pathology, physiopathology)
• Dogs
• Electrophysiology
• Electroretinography
• Humans
• Macular Degeneration (genetics, physiopathology)
• Middle Aged
• Neuronal Ceroid-Lipofuscinoses (genetics, physiopathology, veterinary)
• Pigment Epithelium of Eye (physiopathology, ultrastructure)
• Retinal Diseases (genetics, physiopathology, veterinary)
• Retinitis Pigmentosa (genetics, physiopathology, veterinary)