Table 1.
Subject demographics and clinical characteristics
| Parameter/metric | SCA1 | SCA2 | SCA3 | SCA6 | CTRL |
|---|---|---|---|---|---|
|
| |||||
| Sex (F/M)1 | 10/8 | 10/9 | 11/10 | 15/5 | 9/13 |
| Age at study enrollment (years)1 | 49 ± 11a | 44 ± 11a | 51 ± 11a | 62 ± 10 | 53 ± 16 |
| Age at onset (years)2 | 39 ± 12a | 34 ± 10 a, b | 44 ± 8a | 53 ± 11 | - |
| Age of diagnosis (years) | 42 ± 12a | 38 ± 11a | 47 ± 13a | 58 ± 11 | - |
| Disease duration (years)3 | 11 ± 5 | 9 ± 8 | 8 ± 5 | 11 ± 7 | - |
| CAG repeat length4 | 45 ± 3 | 39 ± 3 | 71 ± 4 | 22 ± 1 | - |
| SARA score5 | 8.3 ± 4.0c | 9.5 ± 6.8c | 7.1 ± 2.9c | 9.8 ± 6.5c | 0.1 ± 0.2 |
| ADL score5 | 5.9 ± 4.0c | 6.2 ± 5.5c | 5.2 ± 3.3c | 7.9 ± 6.3c | 0.0 ± 0.0 |
| BMI (kg/m2)6 | 26.5 ± 3.5 | 25.9 ± 4.0 | 25.0 ± 2.4 | 27.0 ± 4.3 | 25.8 ± 5.1 |
Reported values are mean ± SD
No significant differences in sex and age were observed for SCA groups vs. the healthy control group and in sex between the SCA groups.
Mean age at onset for subjects with manifest ataxia (SARA > 2.5). Value is a self-reported estimate based on memory of perceived ataxia onset.
Disease duration is the difference between age at enrollment and the self-reported age of onset for subjects with manifest disease. Disease duration was not significantly different among SCA groups.
Number of triplet repeats in the expanded allele
Scale for the Assessment and Rating of Ataxia (SARA) and activities of daily living (ADL) scores were not significantly different among SCA groups.
No significant body mass index (BMI) difference was observed among all groups.
p<0.05 for each of SCA1, SCA2 and SCA3 vs. SCA6.
p<0.05 for SCA2 vs. SCA3.
p<0.002 for SCA vs. heathy controls.