Psychiatric symptoms as late onset of Wilson's disease: neuroradiological findings, clinical features and treatment

Abstract

We describe a case of Wilson's disease with late psychiatric onset. Major depressive disorder was the first clinical manifestation at the age of 38 years. After pharmacotherapy with antidepressive agents, a manic episode was observed. Extrapyramidal hand tremor and micrography were the first neurological signs. Emotional lability occurred during worsening of extrapyramidal signs. Diagnosis was based on urinary and serum copper levels, ceruloplasmin serum level, Kayser-Fleischer ring, and liver biopsy that detected cirrhosis. Magnetic resonance imaging revealed basal ganglia hyperintensity on T1-weighted images, and hypodensity in the central part and hyperintensity in the peripheral part of the lentiform nucleus on T2-weighted images. Hyperintensity on T2-weighted images was also observed in the dorsal part of the midbrain. 123I-iodobenzamide single photon emission computed tomography (IBZM-SPECT) detected a normal distribution of the drug in the brain, with better signal in the right side and deficit of D2-dopaminergic receptors in the basal ganglia. Abnormal manganese erythrocyte level was observed. Treatment was based on penicillamine, zinc salts, low-copper diet, antidepressant agents, interpersonal psychotherapy and neurorehabilitation.

Contribution to the Data on Copper Concentration in Blood and Urine in Patients with Wilson’s Disease and in Normal Subjects

“…Among many methods of diagnosis are the following: determination of total serum copper, free serum copper, and serum ceruloplasmin, as well as copper in biopsy liver samples [13]. Gitlin [4] proposes haplotype or genetic mutation analysis when a proband mutation is identified.…”

The degree of depression in Hamilton rating scale is correlated with the density of presynaptic serotonin transporters in 23 patients with Wilson's disease

“…Psychiatric diseases, especially depression and per- [1,4,10]. Some 20 percent of patients undergo psychiatric treatment before specific chelation therapy begins.…”

Personality changes in a patient with Wilson’s disease

“…Psychiatric and behavioral abnormalities may be seen in 30 to 100% of the patients and these are initial symptoms in two thirds of cases (12)(13)(14). Personality changes and aggression are the most common symptoms and are followed by depression.…”