Quality of Life and Psychiatric Symptoms in Wilson’s Disease: the Relevance of Bipolar Disorders

Abstract

Introduction:Wilson’s disease is an inherited disorder caused by a gene located on chromosome 13, which involved copper transportation across cell membranes. The disease can cause a reduced incorporation of copper into ceruloplasmin resulting in accumulation of this metal in the liver, central nervous system, kidneys and other organs. The objective is to define the frequencies of psychiatric disorders in WD, the amount of impairment of Quality of Life [QoL] in patients with WD and the relevance of the psychiatric disorders in the QoL of people suffering by WD.Methods:This is a systematic review. The search of the significant articles was carried out in PubMed using specific key words.Results:Such other neurological diseases, WD is characterized by chronic course and need of treatments, impairment of functional outcomes and high frequency of psychiatric symptoms, although a specific association between Bipolar Disorders and WD was recently found. Despite this, since today few studies are carried on WD patients’ quality of life related to psychiatric symptoms. Some new reports showed a link between presence of Bipolar Disorders diagnosis, cerebral damage and low Qol.Conclusion:Prospective studies on large cohorts are required to establish the effective impact of psychiatric disorders comorbidity, particularly Bipolar Disorders, on quality of life in WD and to clarify the causal link between brain damage, psychiatric disorders and worsening of QoL.

Encephalopathy in Wilson Disease: Copper Toxicity or Liver Failure?

“…32 In up to 25% of WD patients psychiatric symptoms may be the initial disease presentation. The most common psychiatric symptoms in patients with WD are personality disorders (46-71%) appearing as abnormal behavior, irritability and/or aggressiveness.…”

“…Their complaints can have multiple origins and may reflect affective or somatic problems, as well as a neurotic personality, which are unrelated to primary cognition dysfunction. 32 In individuals with excellent premorbid intelligence and high education, the possible actual decline in some cognitive domains, might be masked by different strategies used by these patients to compensate any deterioration. It has been documented that depression levels assessed in participants by psychiatrists were significantly higher than those in patients' self-rating.…”

Major depression caused by Wilson's disease

“…[1][2][3][4][5] It is caused by an autosomal recessive genetic alteration, located in chromosome 13, 1 and has an incidence of approximately 1 in every 30,000-40,000 births. 5 It generally manifests between the ages of 11 and 25 years, with hepatic symptoms, although other organs may be affected.…”

“…[1][2][3][4][5] It is caused by an autosomal recessive genetic alteration, located in chromosome 13, 1 and has an incidence of approximately 1 in every 30,000-40,000 births. 5 It generally manifests between the ages of 11 and 25 years, with hepatic symptoms, although other organs may be affected. 1 In 10% of cases, psychiatric disorders manifest concomitantly with the first symptoms and signs, 3,5 although they usually tend to appear later, and may present as anxiety, mood disorders, or full-blown psychosis.…”

“…5 It generally manifests between the ages of 11 and 25 years, with hepatic symptoms, although other organs may be affected. 1 In 10% of cases, psychiatric disorders manifest concomitantly with the first symptoms and signs, 3,5 although they usually tend to appear later, and may present as anxiety, mood disorders, or full-blown psychosis. 5 The diagnosis can be made by a thorough history and positive liver biopsy.…”