Hereditary striatonigral and cerebello-olivary degeneration of the Kerry Blue Terrier. II. Ultrastructural lesions in the caudate nucleus and cerebellar cortex.
The character and progression of ultrastructural lesions in the caudate nucleus and cerebellar cortex were studied in four Kerry Blue Terriers afflicted with a hereditary neurodegenerative disease. In the caudate nucleus, the initial lesion was mitochondrial hypertrophy in dendrites of intrinsic neurons. Degeneration of these neurons became widespread while axons of passage and terminal boutons were spared. During the final stages, there was severe disruption of the neuropil with loss of both neurons and glia. A narrow zone bordering the lateral ventricles, however, remained unaffected. In the cerebellar cortex, the lesions involved principally Purkinje cells and progressed through a pattern of degeneration comparable to that involving intrinsic neurons of the caudate nucleus. In the later stages, there was astroglial scarring of the molecular layer. In contrast to the caudate nucleus, there was no disruption of the neuropil with loss of structure in the cerebellum. The fact that progression of lesions during the early stages of the disease in both the caudate nucleus and cerebellar cortex was similar suggested a common mechanism for the neurodegeneration .
D000818:Animals; D001927:Brain Diseases / Q000662:veterinary*; D002421:Caudate Nucleus / Q000648:ultrastructure*; D002493:Central Nervous System Diseases / Q000235:genetics / Q000473:pathology; D002525:Cerebellar Cortex / Q000648:ultrastructure; D002531:Cerebellum; D003342:Corpus Striatum; D004283:Dog Diseases / Q000235:genetics / Q000473:pathology*; D004285:Dogs / Q000235:genetics*; D005260:Female; D008297:Male; D009410:Nerve Degeneration; D009847:Olivary Nucleus; D013378:Substantia Nigra