TABLE 1.
Demographic and characterizing cohort data
| n | Age | Male/female | Age of onseta | Disease duration in yearsa | CAG repeats, longer allele | SARA sum score | |
|---|---|---|---|---|---|---|---|
| Healthy controls | 63 | 42.81 (13.65) | 35/28 | na | na | na | 0.22 (0.46) |
| Preataxic | 48 | 37.75 (9.47) | 18/30 | 39.73 (7.87) | −1.98 (9.83) | 68.29 (3.55) | 1.31 (0.94) |
| Ataxic | 210 | 46.84 (11.24) | 118/92 | 34.12 (9.45) | 12.67 (9.45) | 71.10 (4.23) | 12.41 (5.50) |
Data are expressed as mean and standard deviation for age, age at onset, disease duration, CAG repeat length of the longer allele, and SARA sum score and as number for the group size and the male/female distribution.
a
Estimated age at onset on the basis of CAG repeat length following the model provided by Tezenas et al (Tezenas du Montcel et al, 2014) and disease duration in years, defined as the actual age minus the estimated age at onset, resulting in negative values for the expected time to onset in preataxic SCA3 mutation carriers and positive values for ataxic SCA3 mutation carriers.