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Friedreich's ataxia is the most common inherited ataxia with a prevalence of 1 in 50000. The mode of inheritance is autosomal recessive. There is progressive gait and limb ataxia, loss of proprioception, pyramidal weakness and dysarthria. Extra-neurological involvement includes: - hypertrophic cardiomyopathy in most patients
- diabetes mellitus in 10%
Onset is usually during adolescence.
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