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

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.
AuthorsS E Nilsson, A Wrigstad
JournalEye (London, England) (Eye (Lond)) Vol. 11 ( Pt 5) Pg. 698-706 ( 1997) ISSN: 0950-222X [Print] England
PMID9474321 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
  • 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)

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