Treatment of Acute Psychosis from Wilson's Disease

Primary Treatment Approach

The American Association for the Study of Liver Diseases recommends D-Penicillamine or trientine as the first-line treatment for symptomatic patients with Wilson's disease presenting with psychosis 1, 2
Chelation therapy with D-Penicillamine or trientine promotes urinary excretion of copper, addressing the root cause of neuropsychiatric symptoms in patients with Wilson's disease 2, 3
Initial dosing of chelation therapy should be carefully monitored as neurological symptoms may worsen in 10-50% of patients during the early phase of treatment 2, 3

Tetrathiomolybdate is an experimental chelating agent that may be preferable for patients with neuropsychiatric symptoms as it appears less likely to cause neurological deterioration during initial treatment 4, 5

Atypical antipsychotics are preferred over typical antipsychotics due to lower risk of extrapyramidal side effects in patients with Wilson's disease 6
Low doses of atypical antipsychotics should be used initially (e.g., risperidone 2 mg/day or olanzapine 7.5-10 mg/day) 6

Antioxidants, particularly vitamin E, may have a role as adjunctive treatment as serum and hepatic vitamin E levels are often low in Wilson's disease 1, 7
Dietary modifications to reduce copper intake should be implemented, especially during the first year of treatment 1, 4

Frequent monitoring is essential during the initial phase of treatment, including laboratory testing and physical examinations 8, 9
24-hour urinary copper excretion should be monitored to assess treatment efficacy and compliance 9
Target values for patients on chelation therapy should be 200-500 μg/day (3-8 μmol/day) 9

Treatment must never be terminated indefinitely, even if symptoms resolve, as interruption of treatment has resulted in fulminant hepatic failure and severe neuropsychiatric deterioration 1, 4, 10
Liver transplantation may be considered for patients with severe neuropsychiatric disease that doesn't respond to medical therapy 8, 7

After adequate treatment with a chelator, stable patients may be transitioned to zinc maintenance therapy 1, 4
Zinc is more selective for removing copper than penicillamine or trientine and is associated with fewer side effects 1, 4