Table 1.
Population characteristics at baseline
| Characteristic | Frequency/No. (%) or Mean ± SD | |||
|---|---|---|---|---|
| SCA1, n = 80 | SCA2, n = 125 | SCA3, n = 105 | SCA6, n = 74 | |
| Men | 51 (64) | 59 (47) | 55 (52) | 40 (54) |
| Age, y | 46 ± 12 | 46 ± 14 | 49 ± 12 | 64 ± 11 |
| Age at onset, y | 38 ± 11 | 35 ± 13 | 38 ± 11 | 54 ± 10 |
| Disease duration, y | 8 ± 5 | 11 ± 6 | 11 ± 6 | 10 ± 6 |
| CAGa | 47 ± 5 | 39 ± 3 | 67 ± 4 | 22 ± 1 |
| Diabetes | 3 (4) | 5 (4) | 2 (2) | 5 (7) |
| Cancer | 0 (0) | 0 (0) | 2 (4) | 2 (6) |
| Gastrointestinal diseases | 7 (9) | 7 (6) | 8 (8) | 10 (10) |
| BMI, kg/m2 | 24.9 ± 4 | 25.1 ± 4 | 23.2 ± 4 | 25.4 ± 4 |
| Underweight | 13 (17) | 13 (11) | 13 (14) | 13 (14) |
| Normal weight | 34 (46) | 62 (51) | 57 (62) | 34 (44) |
| Overweight | 18 (24) | 34 (28) | 16 (17) | 18 (32) |
| Obese | 10 (13) | 12 (10) | 6 (7) | 10 (11) |
| SARA score | 12.9 (7) | 14.9 (7) | 13.5 (7) | 14.6 (6) |
| Disease stage | ||||
| 1 | 56 (70.9) | 79 (69.3) | 49 (48.0) | 34 (47.9) |
| 2 | 19 (24.1) | 24 (21.1) | 37 (36.3) | 33 (46.5) |
| 3 | 4 (5.1) | 11 (9.6) | 16 (15.7) | 4 (5.6) |
| Anxiety or depression, yes | 37 (46.3) | 59 (47.2) | 49 (46.7) | 35 (47.3) |
| Any psychotropic use, yes | 15 (18.8) | 18 (14.4) | 20 (19.1) | 7 (9.5) |
| INAS score, no. of signs | 4.4 ± 2 | 4.1 ± 2 | 4.8 ± 2 | 2.0 ± 1 |
| Hyperreflexia, yes | 56 (70.0) | 18 (14.5) | 39 (37.9) | 15 (20.3) |
| Areflexia, yes | 13 (16.2) | 78 (62.9) | 59 (56.2) | 16 (21.6) |
| Extensor plantor, yes | 34 (46.6) | 38 (33.3) | 41 (39.4) | 2 (2.8) |
| Spasticity, yes | 49 (61.3) | 13 (10.7) | 43 (41.3) | 9 (12.5) |
| Paresie, yes | 15 (18.8) | 18 (14.5) | 23 (22.1) | 5 (6.8) |
| Muscle atrophy, yes | 17 (21.2) | 25 (20.3) | 38 (36.9) | 9 (12.3) |
| Fasciculations, yes | 28 (34.5) | 51 (41.1) | 33 (31.4) | 1 (1.4) |
| Myoclonus, yes | 1 (1.2) | 18 (14.5) | 4 (3.8) | 0 (0.0) |
| Rigidity, yes | 1 (1.2) | 7 (5.6) | 11 (10.7) | 6 (8.1) |
| Chorea/dyskinesia, yes | 3 (3.8) | 11 (8.9) | 7 (6.7) | 2 (2.7) |
| Dystonia, yes | 5 (6.2) | 18 (14.4) | 20 (19.0) | 3 (4.1) |
| Resting tremor, yes | 6 (7.5) | 17 (13.8) | 4 (3.8) | 1 (1.4) |
| Sensory symptoms, yes | 45 (60.0) | 88 (72.1) | 65 (66.3) | 36 (50.0) |
| Urinary dysfunction, yes | 25 (31.2) | 50 (40.3) | 49 (47.1) | 26 (35.1) |
| Cognitive impairment, yes | 12 (15.0) | 32 (25.8) | 19 (18.8) | 7 (9.5) |
| Brainstem oculomotor signs, yes | 29 (36.2) | 44 (35.5) | 51 (49.5) | 11 (14.9) |
| Dysphagia, yes | 42 (53.2) | 62 (54.4) | 59 (57.8) | 39 (54.9) |
| Double vision, yes | 13 (16.2) | 26 (20.8) | 58 (55.2) | 36 (48.6) |
SD, standard deviation; SCA, spinocerebellar ataxia; BMI, body mass index; SARA, Scale for the Assessment and Rating Ataxia; INAS, Inventory of Non‐ataxia Signs.
a
Values indicate the length of the expanded allele (repeat units).