Ultrastructure of the Retina in Canine Neuronal Ceroid Lipofuscinosis

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Abstract
Electron microscopic studies on the retinae of 3 English setters, homozygous for the trait of canine neuronal ceroid lipofuscinosis (NCL), revealed preservation of photoreceptors early and late in the course of the disease. This is in sharp contrast to the severe neuroepithelial degeneration in human NCL, for which canine NCL is considered a spontaneous animal model. Ubiquitous accretion of NCL-specific lipopigments was present in all cell types of the retina, suggesting that NCL-specific lipopigment accumulation in photoreceptors per se is not lethal to these cells. The ultrastructure of lipopigments comprised curvilinear, fingerprint, lamellar, and complex patterns. Retinae of heterozygotes exhibited occasional regular lipofuscin granules.