Research directions
Treatment and mitigation of neurodegenerative disorders is of particular interest to researchers, and several potential options for SCA1 are under investigation. As the pathology of SCA1 is complex, there are several possible approaches to treatment, which include clearance of expanded ataxin 1 proteins, reducing the toxicity of expanded ataxin 1 proteins, suppressing production of ataxin 1, multiple gene therapies, and replacing lost brain cells. Because many SCAs, including SCA1, are polyglutamine diseases and operate by similar mechanisms to Huntingtons disease many promising treatments for Huntingtons disease are being investigated for SCAs as well. Gene downregulation and silencing
Because spinocerebellar ataxias are often linked to a mutation on a single gene, modifying how the gene is expressed can modify the phenotype. There are several approaches to modifying the expression of mutant proteins, including techniques that completely stop expression, known as gene silencing. In SCA1, pathogenesis requires constant expression of the mutant ATXN1 gene, and silencing has been shown to halt further progression of the disease, clear nuclear inclusions and aggregates and lead to partial recovery of motor functions in rodent models with conditional expression of the gene. The conditional expression of ATXN1 in mice models differs from how the gene would be silenced therapeutically but the results indicate that therapeutic methods of gene silencing may be viable for treatment and management of SCA1.
In cases where SCAs are not present in family history or where family history is unavailable, testing for the 4 most common SCAs will return positive results for 50% of suspected SCA cases. : 11  Individuals who are at risk of inheriting SCA1 but are currently presymptomatic can also be screened with molecular genetic testing. Genetic testing
Genetic testing is the only definitive way to differentiate between spinocerebellar ataxia types because of the similarity between clinical characteristics of these diseases and the large  variance between cases. Genetic testing is available for many SCA types, including the relatively common types SCA1, 2, 3, 6, and 7; and the less common SCA8, 10, 12, 14, and 17. However, genetic testing is high in cost and has a low diagnostic yield, with positive diagnoses being found in only 24% of tests ordered by a subspecialist and 10% overall.Genetic testing can be administered at various stages of disease progression. When genetic testing is administered after the onset of symptoms, the test is said to be diagnostic; in adults prior to onset of symptoms it is presymptomatic, and the test can be performed for prenatal or preimplantation diagnoses. The European Molecular Quality Genetics Network (EMQN) recommends criteria for each type, which must be met before testing can begin. The EQMN recommends that labs receive a written clinical evaluation of symptoms by a neurologist and a disclosure of family history, or lack of history, before initiating diagnostic genetic testing.