Gender differences in Wilson's disease.

Background: Wilson's disease (WD) is an inherited disorder of copper metabolism. Although well documented in many disorders, gender hasn't been directly addressed in WD; therefore, our aim was to evaluate gender differences in WD.

Methods: We analyzed data on 627 consecutive WD patients entered into our registry (1958-2010).

Results: We observed a male predominance in our population of WD patients (327 males vs. 290 females; p<0.05). At disease diagnosis, 510/627 patients were symptomatic, most patients had the neuropsychiatric WD form (345/510; p<0.01). The neuropsychiatric form occurred predominantly in men versus women (209/278 vs. 136/232; p<0.01), especially the rigidity-tremor (71/111 vs. 40/111; p<0.05), rigidity (23/33 vs. 10/33; p=0.07) and psychiatric forms (46/71 vs. 25/71; p=0.06). The hepatic form occurred more frequently in women (96/165 vs. 69/165; p<0.01) and women developed the neuropsychiatric form 2 years later than men (29.4 vs. 27.1; p<0.05).

Conclusions: According to our findings, the neuropsychiatric form of WD is predominant at diagnosis in both genders. The hepatic form of WD occurs more frequently in women, and women develop the neuropsychiatric form of disease almost 2 years later than men. We speculate these differences may be due to the protective effect of estrogens and iron metabolism differences.