epidemiology
Last reviewed 10/2023
Friedreich’s ataxia is the most common inherited ataxia in the Caucasian population.
- it is very rare in those from Southeast Asia and Africa
- estimated incidence in Caucasians is about 1 in 29 000, with a carrier frequency of 1 in 85
- accounts for about half of all genetic ataxia and three quarters of inherited ataxia in people under 25 years (1)
The prevalence of FRDA is 2:100,000-4:100,000. The carrier frequency is 1:60-1:100 (2).
In the UK, there are at least 10,000 adults and 500 children with progressive ataxia according to the latest estimates (3).
It is the commonest inherited ataxia in Europe.
- the estimated disease incidence based on carrier frequency of 1 in 85 is 1:29,000
- in Europe, prevalence is quoted between 1 in 20,000 to 1 in 50,000, with some geographical variability
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