Table.
Clinical Characteristics of CD in WD Patients.
| no. | Age (yo) | Sex | Age at Dx. (yo) | Medication | Neurological sign | Electrophysiological finding | MRI finding | Laboratory test | References | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Zn (mg/day) | Other (mg/day) | M | S | R | Neuropathy | Myelopathy | Dorsal cord lesion | Serum tCu (µg/dL) | Serum CPN (mg/dL) | Calculated serum fCu (µg/dL) | Urine Cu (µg/24h) | Serum Zn (µg/dL) | |||||
| Normal values | 68-128 | 21-37 | 10-15 | 4.2-33.0 | 80-130 | ||||||||||||
| 1 | 41 | M | 25 | 275 | + | + | N | N/A | + | - | Low | Low | N/A | N/A | N/A | (2) | |
| 2 | 43 | M | 15 | 170 | T; 900 | + | - | N | + | - | - | 3 | <10 N/A | 108 | 251 | (3) | |
| 3 | 51 | F | 27 | 480 | + | + | ↓ | + | + | - | 5 | N/A | <5 | 20 | N/A | (4) | |
| 4 | 37 | F | 21 | 180 | - | - | - | - | + | + | <5 | 0.92 | <2.1 | 11 | 474 | (5) | |
| 5 | 46 | F | 41 | 180 | N/A | N/A | N/A | N/A | N/A | N/A | <5 | 0.5 | <3.4 | 6 | 192 | (5) | |
| 6 | 18 | F | 12 | 180 | N/A | N/A | N/A | - | + | N/A | 7 | 0.9 | 4.2 | 12 | 247 | (5) | |
| 7 | 56 | F | 18 | 150 | - | + | ↑ | N/A | + | + | 3 | Low | N/A | Low | 179 | (6) | |
| 8 | 44 | F | 29 | 160 | - | + | ↑ | N/A | N/A | + | 3 | 8 | 0 | 7.4 | 311 | (7) | |
| 9 | 17 | F | 17 | 225 | + | + | ↑ | + | N/A | + | N/A | N/A | N/A | Low | N/A | (8) | |
| 10 | 56 | M | 25 | 480 | N/A | N/A | N/A | N/A | N/A | N/A | 5 | N/A | N/A | N/A | N/A | (9) | |
| 11 | 36 | M | 20 | 150 | DP; 600 | - | + | N | + | N/A | + | 13.3 | 3.0 | 3.9 | 40.5 | 96.1 | (10) |
| 12 | 57 | F | 20 | 150 | DP; 1000 | + | + | ↓ | + | + | + | 11 | 5.5 | 0 | 74 | 127 | *** |
No.: number, Dx: diagnosis, F: female, M: male, yo: years old, tCu: total copper, fCu: free copper, Zn: zinc, T: trientine, DP: D-penicillamine, CPN: ceruloplasmin, M: motor symptoms, S: sensory impairment, R: tendon reflex, N: normal, N/A: not available, MRI: magnetic resonance imaging
fCu(µg/dL)=tCu(mg/dL)-3.15(mg/µg)×CPN(mg/dL). We set fCu as 0 when it is calculated to be a negative value.
***Current case.