Neuronal ceroid lipofuscinosis in Rambouillet sheep: characterization of the clinical disease.

A chronic progressive, neurodegenerative disease has been identified in 4 closely related flocks of purebred Rambouillet sheep. Blindness, circling, proprioceptive deficits, reduced cognition, and poor body condition are the main clinical signs. Prominent lesions include markedly decreased cerebral size and weight, enlarged cerebral ventricles, and intraneuronal accumulations of autofluorescent pigment. Affected sheep usually die between 1 and 2 years of age. The clinical signs and postmortem findings are consistent with neuronal ceroid-lipofuscinosis.