An autopsy case of a 51-year-old housewife with dominantly inherited ataxia is reported. Pathologic examination revealed an unusual combination of systemic degenerations: spinocerebellar tracts, pontine basis and middle cerebellar peduncles, dentato-rubral and nigro-subthalamo-pallidal systems, upper and lower motor neurons, ascending sensory system, and optic tracts. The inferior olive and cerebellar cortex showed only a slight change. The combination of the dentato-rubral with nigro-subthalamo-pallidal degenerations in a case with cerebellipetal systemic degeneration was particularly striking. Furthermore, combined degeneration of the ocular motor nuclei, vestibular nuclear group, perihypoglossal nuclei, fastigial nucleus, medial longitudinal fasciculus, and vestibulospinal tracts occurred. According to recent experimental results, these lesions were restricted precisely to the anatomophysiologically interrelated system for the control of eye movement. The present case is considered an example of systemic degeneration as a pathologic basis for ophthalmoplegia.