Table 1. Clinical Characteristics of Cohort Studies and Case Series (N≥2) 2008–2018.
| Author | Year | Cohort Description (Male: Female) | Age at Symptom Onset (Range in Years) | CAG Repeat Length (Range) | Clinical Characteristics (No. of Cases with Each Reported Symptom) | |||||
|---|---|---|---|---|---|---|---|---|---|---|
| Ataxia | Choreoathetosis | Extrapyramidal Signs | Cognitive Impairment | Seizures | Psychiatric Symptoms | |||||
| Sugiyama et al.14 | 2018 | 10 (4:6) | 61–71 | 52–58 | 10 | 1 | – | 5 | 1 | 2 |
| Sone et al.53 | 2015 | 4 (1:3) | 9–23 | 60–67 | 4 | – | 1 | 3 | 4 | – |
| Maruyama et al.11 | 2012 | 9 (6:3) | 0.5–12 | 62–93 | 5 | 5 | 5 | – | 9 | 3 |
| Yoon et al.54 | 2012 | 3 (1:2) | 37–42 | 59–62 | 3 | – | – | 1 | 1 | – |
| Aridon et al.70* | 2012 | 10 (8:2) | 13–48 | 58–65 | 9 | 2 | – | – | 6 | 2 |
| Sunami et al.46* | 2011 | 2 (2:0) | 19–56 | 65–70 | 2 | 1 | – | 2 | 1 | 2 |
| Hasegawa et al.9 | 2010 | 183 (82:101) | 0–72 | 56–82 | 156 | 78 | – | 124 | 98 | 45 |
| Egawa et al.56 | 2008 | 17 (9:8) | 2–18 | 64–76 | – | – | – | ≥11 | 17 | – |
| Wardle et al.7 | 2008 | 17 (11:6) | 29–46 | 51–66 | 9 | 6 | 1 | 11 | 9 | 3 |
*
Single family study.
–, No Reported Cases.