Psychiatric manifestations in Wilson’s disease: possibilities and difficulties for treatment

Abstract

Wilson’s disease (WD) is an inherited metabolic disorder related to disturbances of copper metabolism, and predominantly presents with liver and neuropsychiatric symptoms. In most cases it can be successfully treated with anti-copper agents, and both liver function and neuropsychiatric symptoms typically improve. Treatment guidelines for WD include recommendations for anti-copper treatment as well as for the treatment of liver failure symptoms. Recently, recommendations for treatment of the neurological symptoms of WD have also been proposed. Although most WD patients present with psychiatric symptoms at some stage of the disease, currently there are no guidelines for the treatment of the psychiatric manifestations. Treatment of the psychiatric symptoms of WD is often guided by general psychiatric experience, which typically glosses over the specificity of WD, and can result in severe neurological and/or hepatic complications. Here we review and discuss the possible treatments available for the mood disturbances, psychosis, behavioral and cognitive disorders that can occur in WD, as well as their efficacy.

Regional morphometric abnormalities and clinical relevance in Wilson's disease

“…In previous studies, a variety of psychiatric symptoms have been reported in WD, including emotional lability, mood disorders, and behavioral and personality changes. The amygdala and hippocampus play important roles in emotional processes, learning, and memory.…”

“…Wilson's disease (WD) is a progressive autosomal‐recessive genetic disorder of copper metabolism, with principally hepatic, neurological, and psychiatric manifestations. The neurological symptoms of WD develop in almost 40% to 50% of patients and are characterized by involuntary movements, speech disturbances, dysphagia, autonomic dysfunction, and gait and balance disturbances . Up to 30% of WD patients have manifested with psychiatric symptoms at the time of diagnosis .…”

Late-Onset Wilson's Disease

“…These symptoms may precede the hepatic and neurological symptomatology by a significant period of time. Other psychiatric manifestations include personality changes (antisocial behavior, irritability, and disinhibition), mood disorders (bipolar disorders, manic and hypomanic syndrome, depression, suicidal attempts), psychosis, anorexia, dyssomnias, sexual dysfunction, and cognitive impairment (7)(8)(9).…”

Wilson disease in children and adolescents

“…Psychiatric problems may not be recognised as a symptom of WD. They are common in adults with a quarter having symptoms at presentation and almost all developing symptoms over the course of the disease 19–21…”