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. 2022 Sep 13;123(3):917–925. doi: 10.1007/s13760-022-02091-z

Table 1.

The impact of selected baseline clinical and laboratory parameters on early neurological deterioration

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