Hereditary Striatonigral and Cerebello-Olivary Degeneration of the Kerry Blue Terrier. I. Gross and Light Microscopic Central Nervous System Lesions

Abstract

The character and progression of gross and light microscopic central nervous system lesions associated with a hereditary neurodegenerative disease with juvenile onset are described in ten affected Kerry blue terriers. The central nervous system lesions were progressive and, although there was some variability, followed a relatively well-defined temporal course. Degeneration of Purkinje cells in the cerebellar cortex was evident at the onset of clinical signs (approximately 4.5 months of age). After two weeks to one month of clinical illness, retrograde transsynaptic neuronal degeneration occurred in the olivary nucleus. Degeneration of both large and small neurons in the caudate nucleus began approximately two to three months after the onset of clinical signs, and by seven to eight months of clinical illness, the caudate nucleus was reduced to numerous microcystic cavities and almost was devoid of neurons except for a narrow subependymal zone and the tail of the nucleus. Neuronal depletion in the pars reticularis of the substantia nigra, which was evident after five to seven months of clinical illness, was attributed to an anterograde transsynaptic mechanism of neuronal degeneration. The disease in the Kerry blue terrier is compared with similar neurodegenerative diseases in man. The pathogenesis may involve altered neurotransmitter systems in the cerebellar cortex and caudate nucleus.