Table 1. Characteristics of Neurological WD patients, Non-Neurological WD patients and healthy controls.
Neurologic WD | Non Neurologic WD | Controls | p-value | |||||
---|---|---|---|---|---|---|---|---|
N = 10 | N = 9 | N = 20 | ||||||
Mean (SD) | Mean (SD) | Mean (SD) | ||||||
Age at onset (years) | 19.3 (3.5) | 10.44 (4.53) | - | p = .0002 | ||||
Age at time of the study (years) | 33.2 (12.73) | 26.33 (5.61) | 29.5 (9.18) | ns | ||||
Gender (M/F) | 4/6 | 2/7 | 6/14 | ns | ||||
Education (years) | 12.3 (2.41) | 13.44 (2.3) | 13.7 (8.46) | ns | ||||
Modified Rankin Scale score (score 0–6) | 1.1 (0.74) | 0 (0) | - | - | ||||
[range] | [0–3] | [0] | ||||||
Specific treatments of the disease (number of patients) | ||||||||
D-penicillamine / Trientine / Zinc | 5 / 2 / 0 | 4 / 1 / 1 | - | - | ||||
Liver transplant (hepatic/neurological signs) | 3 (2/1) | 3 (3/0) | - | - | ||||
Other treatments | ||||||||
Anxiolytics / Antidepressants / Antipsychotics | 3 / 1 / 1 | 0 / 0 / 0 | - | - | ||||
Slip lamp examination | 8/10 | 9/9 | - | - | ||||
(number of Kayser-Fleischer ring at time of diagnosis) | 6 with KF ring | 2 with KF ring | ||||||
Brain MRI (number of patients) | 10/10 | 8/9 | - | - | ||||
10 with signs | 0 with signs | |||||||
Neuropsychological profile | ||||||||
Estimated IQ—FNART | 109.3 (7.41) | 109.22 (5.36) | - | ns | ||||
Total recall score—Word list WMS III | 33.67 (6.48) | 38.14 (4.78) | - | ns | ||||
Position discrimination—VOSP | 28.6 (8.13) | 26.22 (9.34) | - | ns | ||||
Number location—VOSP | 10 (0) | 10 (0) | - | ns | ||||
GZ score—D2 | 417.2 (50.59) | 477.44 (98.77) | - | ns | ||||
KL score—D2 | 166 (19.66) | 185.78 (47.81) | - | ns | ||||
Response time A-B—TMT | 42.2 (30.12) | 23.78 (7.98) | - | p = .07 |
FNART: French-National Adult Reading Test, WMS: Wechsler Memory Scale, VOSP: Visual Object and Space Perception Battery, TMT: Trail Making Test