Amacrine and horizontal cell dysfunction in adult ceroid-lipofuscinosis (Kufs disease) and anatomical correlates in the ovine model.
Psychophysical examination of the visual performance of a patient with Kufs disease suggests abnormal functioning of the amacrine and horizontal cell systems of lateral inhibition. The sheep model offers insight into early patho-physiology of these cell types.
D000818:Animals; D004195:Disease Models, Animal; D005260:Female; D006801:Humans; D008875:Middle Aged; D009472:Neuronal Ceroid-Lipofuscinoses / Q000473:pathology / Q000503:physiopathology*; D010775:Photic Stimulation; D011601:Psychophysics / Q000379:methods; D012016:Reference Values; D012756:Sheep; D014787:Vision Tests / Q000295:instrumentation / Q000379:methods