POLG and Ataxia: Therefore, ARSACS differs from other common recessive ataxias with neuropathy like polymerase gamma (POLG)-related ataxia [34, 35], the cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) [36], Friedreich’s ataxia (FRDA) [37], Ataxia telangiectasia (AT) [38], and ataxia with oculomotor apraxia (AOA) Type 1 [39] and 2 [40], which are all presenting a mainly axonal type of neuropathy.