Table 1.
Characteristic | Without early neurological deterioration (n = 51) | With early neurological deterioration (n = 10) | P value |
---|---|---|---|
Gender, male, n (%) | 29 (56.8%) | 3 (30.0%) | 0.170 |
Age at onset, mean ± SD (years) | 28.1 ± 10.9 | 30.5 ± 11.6 | 0.575 |
Age at diagnosis, mean ± SD (years) | 31.2 ± 11.1 | 36.2 ± 14.3 | 0.267 |
Neurological form of WD (n = 36) | 26 (50.9%) | 10 (100%)** | 0.003 |
Hepatic form of WD (n = 18) | 18 (35.2%) | 0 (0%)** | 0.026 |
Pre-symptomatic form of WD (n = 7) | 7 (13.7%) | 0 (0%) | 0.587 |
Diagnostic delay*, mean ± SD (years) | 2.4 ± 3.6 | 5.7 ± 9.2 | 0.088 |
d-penicillamine, n (%) | 22 (43.2%) | 6 (60%) | 0.327 |
Zinc sulphate, n (%) | 29 (56.8%) | 4 (40%) | 0.327 |
UWDRS part II score, mean (range) | 2.0 (0–38) | 4.3 (0–16)** | 0.033 |
UWDRS part III score, mean (range) | 9.3 (0–96) | 21.5 (2–41)** | 0.002 |
MRI acute toxicity score, mean (range) | 1.5 (0–9) | 3.3 (0–11) | 0.071 |
MRI chronic damage score, mean (range) | 1.4 (0–10) | 3.2 (0–5)** | 0.006 |
MRI total score, mean (range) | 3.0 (0–12) | 6.5 (0–13)** | 0.020 |
Serum ceruloplasmin concentration, mean ± SD (mg/dL) | 13.0 ± 6.6 | 11.9 ± 6.7 | 0.718 |
Urinary copper excretion, mean ± SD (μg/24 h) | 373.5 ± 1220.0 | 195.9 ± 184.0 | 0.640 |
sNfL concentration, mean ± SD (pg/mL) | 27.2 ± 62.7 | 33.2 ± 23.5** | 0.006 |
*The time between first symptoms onset and disease diagnosis
**Denotes statistically significance
MRI magnetic resonance imaging, SD standard deviation, sNfL serum neuro-filament light chain, UWDRS Unified Wilson’s Disease Rating Scale