Fig. 1

Functional evaluations during DHA therapy. The figure summarizes trends of SPRS, SARA, 10MWT and 6MWT scores in P1 and P2 before starting DHA (T0) and after 20 months of treatment. The SPRS (a) and SARA (b) scores, time taken to perform the 10MWT (seconds) (c), and meters covered on the 6MWT (d) are reported for both patients (P1, blue lines; P2, red lines). The box-plots in (a) display the SPRS scores of ARSACS patients in the age groups 30–39 and 40–49 (derived from [15]). The dashed lines in (b), (c) and (d) represent the motor performance trends reported in the literature in aged-matched ARSACS patients with spastic-ataxic diseases measured by SARA, 10MWT and 6MWT (reported in [14])