TABLE 2.
Cognitive Function Neuro-QoL Domain T-Scores for Pediatric Patients With SWS by Extent of Total SWS (the Sum of a Subject’s Brain, Skin, and Eye Involvement Scores), Brain, Skin, and Eye Involvement
| Sturge-Weber Extent | Extent of Involvement | n | % | Cog. Fxn. Neuro-QoL (Mean T-score ± S.D.) | R | P-Value |
|---|---|---|---|---|---|---|
| Total SWS involvement | 0 | 0 | 0.00 | N/A | −0.63 | 0.002 |
| 1 | 4 | 19.05 | 47.35 ± 2.29 | |||
| 2 | 5 | 23.81 | 45.38 ± 7.07 | |||
| 3 | 4 | 19.05 | 38.18 ± 6.66 | |||
| 4 | 5 | 23.81 | 35.86 ± 6.95 | |||
| 5 | 3 | 14.29 | 36.10 ± 10.45 | |||
| 6 | 0 | 0.00 | N/A | |||
| Brain involvement | None | 0 | 0.00 | N/A | N/A | 0.1 |
| Unilateral | 18 | 81.82 | 41.76 ± 7.62 | |||
| Bilateral | 4 | 18.18 | 35.48 ± 7.05 | |||
| Skin involvement | None | 4 | 19.05 | 47.35 ± 2.29 | −0.46 | 0.04 |
| Unilateral | 11 | 52.38 | 40.27 ± 8.09 | |||
| Bilateral | 6 | 28.57 | 37.37 ± 8.22 | |||
| Eye involvement | None | 10 | 47.62 | 44.98 ± 6.42 | −0.58 | 0.005 |
| Unilateral | 9 | 42.86 | 38.17 ± 6.87 | |||
| Bilateral | 2 | 9.52 | 31.65 ± 9.97 | |||
Abbreviations:
Neuro-QoL = quality of life in neurological disorders
SWS = Sturge-Weber syndrome
A higher T-score represents more cognitive function Neuro-QoL, and it is standardized to have a standard deviation of 10 with 50 representing the mean value in the reference population (see text for description). An increased extent of skin, eye, and total SWS involvement correlated with lower cognitive function Neuro-QoL. Extent of brain involvement was not significantly associated with cognitive function Neuro-QoL.