NEURONAL CEROID-LIPOFUSCINOSIS IN RAMBOUILLET SHEEP - CHARACTERIZATION OF THE CLINICAL-DISEASE

A chronic progressive, neurodegenerative disease has been identified i n 4 closely releted flocks of purebred Rambouillet sheep. Blindness, c ircling, proprioceptive deficits, reduced cognition, and poor body con dition are the main clinical signs. Prominent lesions include markedly decreased cerebral size and weight, enlarged cerebral ventricles, and intraneuronal accumulations of autofluorescent pigment. Affected shee p usually die between 1 and 2 years of age. The clinical signs and pos tmortem findings are consistent with neuronal ceroid-lipofuscinosis.