THE PRINCIPAL lesions of hereditary ataxia of rabbits occur first within the vestibular and cochlear nerves and nuclei and later extend to the vestibulocerebellar and pontocerebellar system.1-3 Lesions in the motor fifth and seventh-nerve nuclei, reticular formation, lateral lemniscus, decussation of the medial lemniscus, and trapezoid body are common but less severe. Degenerative changes may also occur in the tegmentum at the level of the inferior colliculus and in the lateral and ventral nuclei of cervical cord.3
Glycogen deposits in the hindbrain areas of the ataxic rabbits have been identified by light microscopy,4 and the distribution and localization of the glycogen, mainly within neuropil and glia, have been demonstrated by electron microscopy.5 Quantitative biochemical estimations of glycogen confirmed the presence of increased amount in the medulla, pons, and midbrain.6 In addition, the activity of glucose 6-phosphate dehydrogenase was increased in the pons and cerebellum,