Friedreich’s ataxia


Disease modifying treatment available:
Time critical diagnosis and management:
Lateralising:


Definition

None




Aetiology

Most people with Friedreich ataxia carry a biallelic GAA repeat expansion in the first intron of the frataxin (FXN) gene. This leads to transcriptional repression and loss of function of frataxin protein.




Clinical features


None

Friedreich’s ataxia may cause the following:





Investigations


None

None


Laboratory Investigation Result

Treatment


Management is generally supportive.

Omaveloxolone, an NRF2 activator, has been approved for treatment in the USA and Europe following the results of the MOXIe study.





Research


Open questions

None