The mean duration from age at onset to age at wheelchair-bound is 15,5 years 97 . On average, patients with FRDA die at an age ranging from 36,6 17 and 37,5 9 . The main study of mortality in FRDA was carried out by Tsou et al. The survey concluded that cardiac dysfunctions remain the major cause of death, despite improvements in diagnostic accuracy and medical management. The main cardiac dysfunctions are congestive cardiac failures and arrhythmia; others include stroke, ischemic heart disease and pneumonia (Fig. 13).Particularly, patients died from cardiac causes at younger ages, with disease durations that were on average 10 ages shorter than patients dying from noncardiac causes. Furthermore, patients with disease duration ≥ 20 years had significantly lower odds of dying from cardiac dysfunction. The authors also underlined that (GAA)n repeats inversely correlated with age of death 17 . This was recently confirmed by Pousset et al., who underlined (GAA)n repeats on both alleles of the FNX gene as the best predictor of mortality in FRDA; the two other predictors of mortality were seen to be LVEF reflecting systolic functions and LVMI reflecting hypertrophy.Fig. 13 Main causes of death in a large group of FRDA patients. 17 Diagnosis and management