Hereditary striatonigral and cerebello-olivary degeneration of the Kerry Blue Terrier

Abstract

The character and progression of central nervous system and non-nervous system lesions of a hereditary neurodegenerative disease in the Kerry Blue Terrier were studied in 10 affected dogs ranging in age from 5 to 12 months. Evaluation of the lesions in the naturally occurring disease and comparison of them with those of an experimental animal model indicated a possible pathogenesis for the neuronal degeneration in this disease. Also, this study indicated a possible role for this disease as an animal model for similar diseases that affect man. The present study confirmed the neuroanatomic distribution of neuronal degeneration in the cerebellar cortex (Purkinje cells), caudal olivary nucleus, substantia nigra, and caudate nucleus reported from previous studies and, in addition, revealed neuronal degeneration in the putamen during advanced stages of the disease. The cerebellar cortex and caudate nucleus (and possibly the putamen) were the primary areas of the brain affected while neurodegeneration in the caudal olivary nucleus and substantia nigra was concluded to have resulted from a transsynaptic mechanism. ...