Ovine ceroid-lipofuscinosis: a model of Batten's disease

An inherited neurological disease of sheep was characterized by the intracellular accumulation of autofluorescent lipopigments in neurones and a wide variety of other cells within the body. The staining, fluorescent, ultrastructural and physical characteristics of the storage material were similar to those found in a heterogeneous group of storage diseases of children known as Batten's disease or the ceroid-lipofuscinoses. The ovine disease did not exactly fit any of the main human entities, but had features in common with both the late infantile and juvenile forms. It was concluded that this was a useful model for studying the pathogenesis of this type of storage disease and for therapeutic trials. A flock of sheep is maintained for this purpose.