Familial ataxia of the rabbit Sawin-Anders type

Summary

This hereditary animal ataxia is selective in its sites of involvement within the nervous system, which include principally the central cerebellar, vestibular and cochlear nuclei. Ultrastructural detail has been described for central cerebellar and vestibular nuclei. Herein the cochlear complex of 18 rabbits with this ataxic condition (ax/ax from the strain AX of the Jackson Laboratory) have been examined. The gene is a lethal one, but the animals were used before they became moribund and between 7 and 57 days after the onset of symptoms.

By light microscopy nine cell types (Osen, 1969a, b; 1970) have been identified in the cochlear nuclei of the cat. That distribution can also be identified in electron micrographs of rabbit cochlear nuclei, providing there is a singular opportunity to compare cellular vulnerabilities within the ataxic condition, and establish the principal features of associated neuropil alterations. The cochlear nuclei, cerebellar cortex and central nuclei, and the vestibular nuclei, arise from the ependyma of the rhombic lip of the fourth ventricle, making them close allies in their genetic origins.

Pathological alterations were evident in scattered neurons from all nuclear sources by 7–15 days following symptom onset. At 15 days the number of altered neurons evident in electronmicrographs had increased markedly, cells becoming involved at a more rapid pace than those already affected could be removed. Much glycogen is evident from 7 days onwards in both neuropil and neurons. It occurs in considerable amounts in astrocytic processes and less abundantly in endbulbs and somata. By 20 to 25 days spongioform changes in neuropil are prominent, and thereafter the extracellular spaces coalesce to produce a lacunar appearance showing little glycogen. It would appear, therefore, that all neuron types, the endbulbs, and the astrocytic processes are markedly involved simultaneously in the spongioform transformation which features this type of ataxia. Involvement of cochlear nuclei only differs in pathological detail from that found at the other involved sites, and the differences seen relate principally to the architectonics of the nuclei, including size and density of the packing of contained elements.