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Friedreich's ataxia
 
   

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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