Neurovisceral ceroid-lipofuscinosis in blind Devon cattle.
Ten Devon cattle from a single property were affected with blindness from 14 months of age. Severe retinal degeneration progressing to atrophy was associated with widespread intracellular accumulation of pigment in the retinal ganglion cells, central nervous system and major organs. The pigment was consistent with ceroid-lipofuscin granules on histological, histochemical and ultrastructural examination. Although a familial relationship existed between affected individuals, a pattern of inheritance could not be established by examination of available breeding records. The disease is compared to similar disorders reported in man and other species.
Harper
P A
PA
Veterinary Laboratories, Department of Agriculture, New South Wales, Glenfield, Australia.
Walker
K H
KH
Healy
P J
PJ
Hartley
W J
WJ
Gibson
A J
AJ
Smith
J S
JS
D000818:Animals; D001284:Atrophy; D001766:Blindness / Q000209:etiology / Q000235:genetics / Q000473:pathology; D002417:Cattle; D002418:Cattle Diseases / Q000235:genetics / Q000473:pathology*; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D005260:Female; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000235:genetics / Q000473:pathology / Q000662:veterinary*; D012165:Retinal Ganglion Cells / Q000032:analysis / Q000648:ultrastructure