Progressive ataxia and seizures in a Cocker Spaniel: a new type of neurodegenerative disease with novel intra-neuronal inclusions

Abstract

AIMS: To describe the histopathological lesions of a new canine disease characterised by progressive ataxia, head tremor and seizures, and to deduce the cause of the lesions.

METHODS: Formalin-fixed tissues were processed into paraffin wax and epoxy resin for light and transmission electron microscopy of variously stained tissue sections.

RESULTS: Significant lesions relevant to the disease were found only in the brain. They consisted of hypoplasia of the cerebellum and the presence of large pale inclusions in the perikaryon of neurons in the neocortex and in macrophages. The inclusion material was not compartmentalised and did not stain for carbohydrate, mucopolysaccharide or lipid. This material displaced nuclei to the periphery of the cells where they were seen as basophilic distorted crescent-shaped structures.

CONCLUSIONS: The inclusions were probably made of polymerised protein similar, though not identical, to those of Pick, Lewy and Collins bodies that characterise a variety of chronic neurodegenerative diseases of humans. A genetic basis to this disease was considered probable.

KEY WORDS:

• Dog
• ataxia
• neurodegeneration
• intra-neuronal inclusions
• protein