Information related to the 76 study participants
| Single assessment | Assessed twice 12 months apart | Total | |||||||
|---|---|---|---|---|---|---|---|---|---|
| SD | Range | SD | Range | SD | p Value | ||||
| FARS, Friedreich Ataxia Rating Scale; FIM, Functional Independence Measure; FRDA, Friedreich’s ataxia; GAA1, the size of the smaller of the two GAA expansions; GAA2, the size of the larger of the two GAA expansions; ICARS, International Cooperative Ataxia Rating Scale; MBI, Modified Barthel Index. | |||||||||
| The p value is the significant difference between those seen once and those seen twice, 12 months apart. | |||||||||
| Number of participants | 33 | 43 | 76 | ||||||
| Gender: male % | 45.5 | 53.5 | 50 | 0.64 | |||||
| Diabetes: % taking drugs | 5.9 | 4.9 | 5.3 | 1.0 | |||||
| Late-onset FRDA: % with first symptom >25 years | 9.1 | 16.3 | 13.2 | 0.50 | |||||
| Antioxidant use: % taking regular treatment | 54.5 | 41.9 | 47.4 | 0.36 | |||||
| GAA2 mean: repeat number | 911 | 152.7 | 658–1245 | 952.1 | 155.7 | 548–1345 | 934.9 | 154.7 | 0.026 |
| GAA1 mean: repeat number | 708.9 | 194.3 | 126–1005 | 646.8 | 212.4 | 284–1077 | 672.8 | 206 | 0.20 |
| Age at initial examination: mean (years) | 29.6 | 13.5 | 9–62 | 32.2 | 12.5 | 10–57 | 31.1 | 12.9 | 0.42 |
| Age of onset: mean (years) | 13.6 | 7.5 | 4–39 | 15.5 | 6.8 | 4–33 | 14.7 | 7.1 | 0.27 |
| Disease duration: mean (years) | 16.2 | 11.1 | 1.1–39.4 | 16.7 | 10.6 | 2–44.8 | 16.5 | 10.8 | 0.83 |
| FARS: mean score | 86.6 | 31.7 | 29.0–126.5 | 75.1 | 27.6 | 20.8–124.5 | 80.1 | 29.8 | 0.10 |
| ICARS: mean score | 53.5 | 21.7 | 14–82 | 47.5 | 19.2 | 15–80 | 50.1 | 20.4 | 0.20 |
| FIM: mean score | 100.5 | 20.5 | 63–125 | 112.4 | 13 | 75–126 | 107.6 | 17.7 | <0.01 |
| MBI: mean score | 72.7 | 23.4 | 34–100 | 83.6 | 16.4 | 33–100 | 79.2 | 20.1 | 0.02 |