Table 1.
Demographic characteristics of XDP and Control cohorts.
| XDP patients | Controls | *statistic(p) | ||
|---|---|---|---|---|
| 17 XDP vs. 17 controls | Age | 40.5 ± 8.2 y | 37.2 ± 7.9 y | F = 1.420(0.242) |
| Sex (male/female) | 16/1 | 15/2 | χ2 = 0.366(0.500) | |
| Handedness (R/L/Ambidex) | 15/1/1 | 16/0/1 | χ2 = 1.032(0.597) | |
| Scanner (Achieva/Ingenia) | 13/4 | 16/1 | χ2 = 0.211(0.168) | |
| Age at onset (range) | 36.6 ± 7.2 y (29–49) | |||
| BFM-M total (range) | 51.3 ± 23.1 (3.5–88) | |||
| BFM-M speech (range) | 7.3 ± 5.5 (0–16) | |||
| UPDRS-III total (range) | 37.6** ± 16.4 (5–62) | |||
| HADS-D (range, n) | 5.9 ± 2.2 (3–9, 8) | |||
| HADS-A (range, n) | (7.7 ± 3.5 (1–12, 7) | |||
| 10 XDP vs. 10 controls | Age | 39.5 ± 6.5 y | 38.8 ± 7.7 y | F = 0.048(0.829) |
| Sex (male/female) | 10/0 | 10/0 | ||
| Handedness (R/L/Ambidex) | 8/1/1 | 9/0/1 | χ2 = 1.059(0.589) | |
| Scanner (Achieva/Ingenia) |
10/0 | 10/0 | ||
| Age at onset (range) | 36.3 ± 6.6 y (29–48) | |||
| BFM-M total (range) | 45.4 ± 25.3 (3.5–81) | |||
| BFM-M speech (range) | 7.8 ± 6.2 (0–16) | |||
| UPDRS-III total (range) | 33.9 ± 18.1 (5–62) | |||
| HADS-D (range, n) | 5.8 ± 2.3 (3–9, 6) | |||
| HADS-A (range, n) | 8.0 ± 4.2 (1–12, 5) | |||
XDP: X-linked dystonia-parkinsonism.
*For categorical variables we used Chi-Square tests and for continuous variables we used ANOVAs. No statistics were performed for variables that matched 100% between groups.
**Please see Methods Section 2.2 regarding the effects of dystonia symptoms on UPDRS scores. The patients in this study scored high primarily due to severe dystonia.