POLG and Autosomal recessive spastic ataxia of Charlevoix-Saguenay: A role for speech impairment as a potential objective marker to track disease progression has been previously explored in several genetic ataxias and polyglutamine diseases presenting with dysarthria, such as Friedreich ataxia (FRDA) [7, 8], SCA2 [9], autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) [10], POLG-associated ataxia [11], and HD [12–14].