mean age at diagnosis was 20 years (range 7-54 years) with 38 patients presenting due to liver involvement, 13 due to neuropsychiatric disease, 4 with both and 8 as a result of family screening. Of those presenting with liver involvement, 43% had asymptomatic liver disease with incidentally identified abnormal liver blood tests. In patients with a neuropsychiatric presentation, 95% had neurological signs while 53% had psychiatric symptoms. The mean delay between onset of symptoms and diagnosis

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... as 2 years (max. 8 years). During disease management, 31 patients received one type of drug treatment while 32 received more than one. In total, 48 patients received Penicillamine; of these, 11 were noncompliant to treatment, 16 experienced adverse effects and 21 stopped treatment. The most common adverse effect was skin changes (38% of patients). In patients who stopped Penicillamine, 67% did so due to adverse effects. Trientine was given to 25 patients (1 noncompliant, 2 with adverse effects and 6 stopped treatment) while Zinc was given to 22 patients (4 noncompliant, 3 with adverse effects and 8 stopped treatment). At latest follow-up (mean 10.2 years), 92% of patients with symptomatic liver disease, 87% with neurological disease and 89% with psychiatric symptoms had clinically improved or were stable. In patients with no psychiatric involvement at diagnosis, 26% developed new psychiatric symptoms which was greater than new symptomatic liver or neurological disease. Four patients underwent liver transplant, 3 due to decompensated cirrhosis and 1 due to acute liver failure. There were 3 deaths, of which 2 were due to liver-related complications. In this cohort, 7 patients had 12 pregnancies; 10 were on Penicillamine and 2 were on Zinc therapy throughout the pregnancy. Of those pregnancies on Penicillamine, 8 were normal, 1 was a miscarriage and 1 was a termination. Both pregnancies on Zinc were normal. Conclusions The majority of patients with Wilson's disease who present primarily with liver involvement are asymptomatic with incidentally identified abnormal liver blood tests. New psychiatric symptoms commonly develop after diagnosis and may warrant more proactive psychological input.