Abstract Background: Hereditary neurodegenerative ataxias are a heterogeneous group of diseases affecting the cerebellum, brainstem and spinocerebellar pathways. These diseases are rare, with an estimated prevalence in southeast Norway of 6,5/100 000. Both autosomal dominant and autosomal recessive cases are described. The main symptoms are gait-, truncal-, and limb ataxia, tremor, dysarthria, oculomotor disturbances, dysphagia and impaired coordination. These diseases are difficult to treat because of their heterogeneity and poorly understood pathogenesis. The purpose of this study will be to investigate currently effective treatment options, and treatments that may be effective in the future. Results: Treatment with medications like acetazolamide, 4- aminopyridine, bethametasone and 3,4- diaminopyridine improve SARA-score and prevent attacks of ataxia. These medications reduce some of the symptoms, but only in the period of time when the medication is taken. Physical rehabilitation and coordinative training improve gait-, and ADL-function, and reduce symptoms of ataxia. Continuous training is of great importance for stabilizing the improvement. Gene therapy is currently investigated in vitro, with cells from patients with polyglutamindiseases. The idea is to reduce the amount of mRNA expressed by the mutant allele. Conclusion: Medical and physical therapy will only reduce the patients symptoms, and not interfere with disease progression. The possibilities within the field of gene therapy must be further investigated, as there are no in vivo results from this field at this time. In the future, the scientists meet several challenges, for example the low number of patients scattered across the world, compared with diagnostic difficulties.