Ziegler et al. (1972) described a large kindred in which many persons were affected with a variable neurologic disorder: late-onset cerebellar ataxia, muscular rigidity, bradykinesia, dysarthria, fasciculations, muscle atrophy, and spasticity, appearing in various combinations. There were many ... Ziegler et al. (1972) described a large kindred in which many persons were affected with a variable neurologic disorder: late-onset cerebellar ataxia, muscular rigidity, bradykinesia, dysarthria, fasciculations, muscle atrophy, and spasticity, appearing in various combinations. There were many instances of male-to-male transmission. Pathologic studies in 1 case showed degeneration in spinocerebellar tracts, Purkinje cells, and dentate nuclei of cerebellum, dorsal root ganglion cells, and cauda equina nerve roots. Peripheral neuropathy was present in several cases as evidenced by demyelination found on sural nerve biopsy and by findings of nerve conduction studies. Several cases showed relief of rigidity when levodopa was administered. The 2 sisters reported by Sigwald et al. (1964) seem to have had a similar disorder, but the lack of evidence of dominant inheritance in that family leaves doubt as to the identity.