Spinocerebellar ataxia was for a long time a group of syndromes defined on the basis of the clinical picture. In recent years a marked diagnostic advance was achieved by identification of molecular disorders which are its cause. It is anticipated that molecular genetics will elucidate the group of spinocerebellar disorders and make it possible to identify further clinical units. In the presented article the authors summarize contemporary findings and views regarding spinocerebellar degeneration, discuss the optimal diagnostic procedure of examination and present briefly clinical units along with a commentary regarding the differential diagnosis in the sphere of metabolic disorders known for some time which may start by spinocerebellar ataxia.

        Key words: spinocerebellar ataxias, hereditary ataxias, hereditary metabolis disorders