CLINICAL ASPECTS OF HEREDITARY ATAXIAS

The hereditary ataxias are a group of complex genetic disorders the un derstanding of which is undergoing a revolution because of advances in molecular genetics. Within the last few years, at least seven differe nt gene loci have been found to be responsible for these syndromes, an d the search is on for additional loci that undoubtedly exist. This re view summarizes the clinical features of the various hereditary ataxia s with known gene loci, as well as others that are now defined on a cl inical basis. It also deals with some of the imaging and neuropharmaco logic advances that have been made in this group of disorders.