Predictor | Measure | N | Category | Mean | ± SD | 95% CI | p-value* | |
---|---|---|---|---|---|---|---|---|
Sociodemographic aspects | ||||||||
 Sex | VAS | 65 | Male | 64.2 | 19.9 | 59.2–69.1 | 0.790 | |
55 | Female | 63.6 | 22.1 | 57.6–69.5 | ||||
Overall score | 65 | Male | 62.2 | 20.2 | 57.5–67.2 | 0.277 | ||
55 | Female | 65.5 | 19.0 | 60.3–70.6 | ||||
 Age | VAS | 55 | 18–28 y | 67.1 | 20.3 | 61.6–72.6 | 0.136 | |
63 | 29–61 y | 60.9 | 21.0 | 55.6–66.2 | ||||
Overall score | 55 | 18–28 y | 64.4 | 20.6 | 58.9–70.0 | 0.666 | ||
53 | 29–61 y | 62.7 | 19.0 | 57.9–67.5 | ||||
 Employment | VAS | 49 | Yes | 70.6 | 19.7 | 64.9–76.2 | 0.001†| |
34 | No | 54.7 | 20.8 | 46.4–60.9 | ||||
Overall score | 49 | Yes | 71.7 | 19.9 | 66.9–76.4 | < 0.001†| ||
34 | No | 51.5 | 16.5 | 44.5–58.4 | ||||
 Grade of disability | VAS | 38 | 0–80 | 65.0 | 17.4 | 59.2–70.7 | 0.008†| |
56 | 90–100 | 54.3 | 18.1 | 49.4–59.1 | ||||
Overall score | 38 | 0–80 | 66.1 | 16.5 | 60.7–71.5 | < 0.001†| ||
56 | 90–100 | 54.2 | 16.5 | 49.7–58.6 | ||||
Clinical aspects | ||||||||
 Mutation type | VAS | 25 | TSC1 | 70.3 | 18.0 | 62.9–77.8 | 0.099 | |
31 | TSC2 | 61.0 | 23.5 | 52.3–69.6 | ||||
Overall score | 25 | TSC1 | 68.8 | 17.3 | 61.6–75.9 | 0.424 | ||
31 | TSC2 | 63.0 | 21.4 | 55.2–70.9 | ||||
 Active epilepsy | VAS | 49 | Yes | 56.7 | 19.4 | 51.2–62.3 | 0.003†| |
71 | No | 68.8 | 20.5 | 64.0–73.7 | ||||
Overall score | 49 | Yes | 52.1 | 17.7 | 47.0–57.2 | < 0.001†| ||
71 | No | 71.7 | 16.7 | 67.8–75.7 | ||||
 Structural brain | VAS | 85 | Yes | 62.3 | 20.9 | 57.8–66.8 | 0.232 | |
35 | No | 67.7 | 20.5 | 60.6–74.7 | ||||
Overall score | 85 | Yes | 60.5 | 20.1 | 56.2–64.8 | 0.007†| ||
35 | No | 71.5 | 16.3 | 65.9–77.1 | ||||
 SEGA | VAS | 50 | Yes | 62.0 | 22.1 | 55.7–68.3 | 0.458 | |
70 | No | 65.2 | 20.0 | 60.5–70.0 | ||||
Overall score | 50 | Yes | 59.4 | 20.1 | 53.7–65.2 | 0.041 | ||
70 | No | 66.7 | 18.9 | 62.2–71.2 | ||||
 Neuropsychiatric | VAS | 59 | Yes | 55.1 | 20.4 | 49.8–60.5 | < 0.001†| |
61 | No | 72.3 | 17.6 | 67.8–76.9 | ||||
Overall score | 59 | Yes | 53.2 | 18.0 | 48.5–57.9 | < 0.001†| ||
61 | No | 73.8 | 15.4 | 69.9–77.8 | ||||
 AML | VAS | 70 | Yes | 61.5 | 22.3 | 56.2–66.9 | 0.146 | |
50 | No | 67.2 | 18.4 | 61.9–72.4 | ||||
Overall score | 70 | Yes | 63.6 | 20.8 | 58.6–68.5 | 0.819 | ||
50 | No | 63.9 | 18.2 | 58.7–69.0 | ||||
 Lymphangioleiomyomatosis | VAS | 12 | Yes | 63.3 | 14.4 | 54.2–72.5 | 0.829 | |
108 | No | 63.9 | 21.5 | 59.8–68.1 | ||||
Overall score | 12 | Yes | 70.2 | 14.9 | 60.8–79.7 | 0.256 | ||
108 | No | 63.0 | 20.0 | 59.2–66.8 | ||||
 Skin | VAS | 115 | Yes | 63.8 | 20.9 | 59.9–67.7 | 0.822 | |
5 | No | 66.0 | 20.7 | 40.3–91.8 | ||||
Overall score | 115 | Yes | 63.7 | 19.6 | 60.1–67.3 | 0.855 | ||
5 | No | 63.3 | 22.9 | 34.8–91.8 | ||||
 Number of affected organs | VAS | 31 | 1–3 | 76.7 | 16.7 | 70.6–82.8 | Ref | < 0.001†|
72 | 4–6 | 62.2 | 19.2 | 57.7–66.7 | 0.001 | |||
17 | 7–8 | 47.7 | 21.6 | 36.6–58.8 | < 0.001 | |||
Overall score | 31 | 1–3 | 71.7 | 18.4 | 65.0–78.5 | Ref | 0.007†| |
72 | 4–6 | 62.8 | 18.9 | 58.4–67.2 | 0.026 | |||
17 | 7–8 | 52.9 | 20.0 | 42.6–63.2 | 0.002 | |||
Therapeutic aspects | ||||||||
 Anti-seizure medication polytherapya | VAS | 52 | Yes | 58.1 | 19.0 | 52.8–64.4 | 0.178 | |
31 | No | 64.8 | 20.1 | 57.4–72.2 | ||||
Overall score | 52 | Yes | 54.9 | 17.8 | 49.9–59.8 | 0.032 | ||
31 | No | 63.5 | 18.6 | 56.6–70.3 | ||||
 Everolimus | VAS | 51 | Yes | 59.3 | 22.0 | 53.1–65.5 | 0.063 | |
69 | No | 67.3 | 19.4 | 62.6–71.9 | ||||
Overall score | 51 | Yes | 60.5 | 20.0 | 54.9–66.1 | 0.212 | ||
69 | No | 66.1 | 19.1 | 61.5–70.7 | ||||
 LAEP | VAS | 36 | < 35 | 76.7 | 18.0 | 70.6–82.8 | < 0.001†| |
83 | ≥35 | 58.0 | 19.3 | 53.8–62.2 | ||||
Overall score | 36 | < 35 | 80.5 | 12.7 | 76.2–84.7 | < 0.001†| ||
83 | ≥35 | 56.0 | 17.2 | 52.3–59.8 |