Background <p>Wilson’s disease is a rare autosomal recessive disorder of copper metabolism, characterized by copper accumulation in many tissues, especially in the liver and the brain.</p> Pathophysiology <p>In the brain, pathological changes mainly involve the basal ganglia, explaining the high prevalence of movement disorders and psychiatric symptoms in Wilson’s disease patients.</p> Diagnosis <p>Neuropsychiatric symptoms usually appear at around 20&#xa0;years of age, but the age of presentation range is very broad. Brain magnetic resonance imaging may detect intensity signal abnormalities in the basal ganglia, thalamus and/or brainstem in Wilson’s disease patients with or without neuropsychiatric symptoms.</p> Treatment <p>Neuro-Wilson’s disease treatment includes medications leading to negative copper body balance, as well as drugs for symptomatic treatment of neuropsychiatric symptoms.</p> Conclusion <p>Wilson’s disease includes a wide spectrum of phenotypic presentations, thus making diagnosis difficult. Since neuropsychiatric symptoms may represent the first clinical manifestation of Wilson’s disease, early diagnosis is essential to start prompt treatment.</p>