Table 1.
Characteristics of the French Canadian and French cohorts.
| French Canadian cohort (n = 22) |
French cohort (n = 11) |
|
|---|---|---|
| Male sex, no. (%) | 13 (59%) | 4 (36%) |
| Repeat count of GAA expansion, median (IQR) | 333 (287–392) | 370 (313–492) |
| Age at onset of episodic symptoms (mean ± SD) | 54 ± 9 | 64 ± 9 |
| Age at onset of permanent ataxia (mean ± SD) | 59 ± 10 | 66 ± 10 |
| Inheritance | ||
| Familial, no. (%) | 17 (77%) | 7 (64%) |
| Sporadic, no. (%) | 5 (23%) | 4 (36%) |
| Episodic onset, no. (%) | 16 (73%) | 8 (73%) |
| Alcohol intolerance, no. (%) | 9/17 (53%) | 5/8 (63%) |
| Nystagmus (gaze-evoked horizontal and/or downbeat), no. (%) | 20 (91%) | 8 (73%) |
| Episodic diplopia or visual blurring, no. (%) | 15/21 (71%) | 5/9 (56%) |
| Gait ataxia, no. (%) | 21 (95%) | 11 (100%) |
| Appendicular ataxia, no. (%) | 19/21 (90%) | 10 (91%) |
| Cerebellar dysarthria, no. (%) | 8/21 (38%) | 7 (64%) |
| Vertigo or dizziness, no. (%) | 8/20 (40%) | 5/10 (50%) |
| Cerebellar atrophy on MRI, no. (%) | 12/20 (60%) | 4/9 (44%) |
No.: number; SD: standard deviation; IQR: interquartile range; MRI: magnetic resonance imaging.