Table 2.
Demographic, Clinical, and Imaging Features of the NWD Subgroups
| Active NWD | Stable NWD | p Value | Drug-naive NWD | Non–drug-naive NWD | p Value | |
| n | 22 | 19 | — | 10 | 31 | — |
| Age, y | 25.0 (20.8–29.0) | 31.0 (21.0–37.0) | 0.091a | 23.0 (19.8–28.8) | 29.0 (24.0–36.0) | 0.066a |
| Sex, male/female | 19/3 | 11/8 | 0.04b | 7/3 | 23/8 | 0.795b |
| MMSE | 29.0 (28.0–30.0) | 29.0 (28.0–30.0) | 0.863a | 29.0 (28.0–30.0) | 29.0 (28.0–30.0) | 0.589a |
| Disease duration, y | 1.4 (0.9–3.0) | 10.0 (4.0–15.0) | <0.001a | 1.3 (0.9–3.0) | 5.0 (2.0–13.0) | 0.009a |
| Treatment duration of chelating agents and/or zinc salts, y | 0.2 (0.0–0.7) | 6.0 (3.0–15.0) | <0.001a | 0.0 (0.0–0.0) | 3.0 (0.7–13.0) | <0.001a |
| Treatment | ||||||
| Chelating agents (d-penicillamine, dimercaptosuccinic acid) | 10 (45.5) | 17 (89.5) | 0.003b | 0 (0.0) | 27 (87.1) | <0.001b |
| Zinc salts | 11 (50.0) | 15 (78.9) | 0.055b | 0 (0.0) | 26 (83.9) | <0.001b |
| GAS for WD | 18.0 (13.0–26.0) | 11.0 (9.0–13.0) | <0.001a | 18.5 (14.5–26.8) | 12.0 (9.0–17.0) | 0.028a |
| UWDRS | 36.0 (15.5–46.5) | 10.0 (5.0–15.0) | <0.001a | 33.0 (18.5–50.0) | 14.0 (8.0–36.0) | 0.031a |
| Kayser-Fleischer ring | 21 (95.5) | 15 (78.9) | 0.107b | 9 (90.0) | 27 (87.1) | 0.807b |
| Serum ceruloplasmin, mg/L | 32.0 (22.9–48.6) | 36.1 (25.9–63.2) | 0.553a | 31.2 (20.5–39.8) | 36.0 (26.5–64.9) | 0.325a |
| Hyperintense globus pallidus rim sign | 22 (100.0) | 16 (84.2) | 0.053b | 10 (100.0) | 28 (90.3) | 0.307b |
| T2/SWI hypointensity | 22 (100.0) | 18 (94.7) | 0.276b | 10 (100.0) | 30 (96.8) | 0.565b |
Abbreviations: GAS for WD = Global Assessment Scale for Wilson disease; MMSE = Mini-Mental State Examination; NWD = neurologic Wilson disease; SWI = susceptibility-weighted imaging; UWDRS = Unified Wilson's Disease Rating Scale.
a
Mann-Whitney U test.
b
χ2 test.