A study of cerebellar and cerebral cortical degeneration in miniature poodle pups with emphasis on the ultrastructure of Purkinje cell changes

Abstract

In a litter of three Miniature Poodles, a normal female pup contrasted with two males which displayed severe equilibrium and postural disturbances soon after birth. In the male pups diffuse cerebral cortical degeneration accompanied cerebellar cortical atrophy, which was characterized by extensive loss and degeneration of Purkinje neurons. As in some prior histological descriptions of canine cerebellar atrophies, degenerating Purkinje cells appeared either pale, swollen and vacuolated or shrunken and hyperchromatic. Swollen vacuolated cell bodies and condensed necrotic neurons were scattered through the cerebral cortex. Ultrastructural studies revealed that the vacuolar degeneration in Purkinje and cerebral neurons was referable to marked dilation of endoplasmic reticulum and loss of ribosomes. In shrunken Purkinje neurons, increased cytoplasmic eosinophilia was associated with a decline in Nissl bodies and accumulations of mitochondria and especially lamellar bodies. The latter, stacked derivatives of endoplasmic reticulum, were not encountered in shrunken cerebral perikarya. Lamellar bodies reached giant proportions in the dendritic stems of degenerating Purkinje neurons. In Purkinje axons, however, these bodies often were overshadowed by aggregates of axoplasmic tubules. The cytological changes in these Poodle pups were notably different from those reported in earlier ultrastructural studies of canine inherited cerebellar degenerations.