Publisher Summary This chapter summarizes the present knowledge about the genetic
basis of hereditary ataxia. The degenerative ataxias comprise a heterogeneous group of
diseases, which are clinically characterized by progressive ataxia. The neuropathological
hallmark of degenerative ataxia is degeneration of the cerebellar cortex and its afferent
spinal pathways. Classifications of degenerative ataxias distinguish between hereditary and
non-hereditary ataxia. Recently, a number of genes that cause hereditary ataxia have been …