Wilson Disease - Mayo Clinic Proceedings 2012;1:61. doi: 10.4103/2277-9175.100182. Removing a sample of liver tissue for testing (biopsy). In 1965, Wilson tried LSD for the first time, and shortly afterward began to have auditory hallucinations, a common symptom of schizophrenia. An official website of the United States government. A 35-year-old unmarried Iranian woman presented to the consulting psychiatrist in the emergency room after a suicide attempt due to commanding auditory hallucination. FOIA Spyridi S, Diakogiannis I, Michaelides M, Sokolaki S, Iacovides A, Kaprinis G. Delusional disorder and alcohol abuse in a patient with Wilson's disease. Wilson's disease (WD) or hepatolenticular degeneration is an inherited neurodegenerative disorder of copper metabolism (autosomal recessive, chromosome13). Psychiatry Clin Neurosci. Find out more about saving to your Kindle. On the basis of clinical manifestations and laboratory findings, a diagnosis of Wilson's disease was made. sharing sensitive information, make sure youre on a federal [16] A case series documenting disturbance of circadian rhythm of temperature, pulse, and blood pressure in Wilson's disease described two cases of schizophrenia like psychosis. Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML. Accessibility The presented case of WD has several specific manifestations that distinguish it from most other patients with WD. The psychiatric presentations of Wilson's disease. Psychiatric manifestations are common with Wilson's disease and occur in 20-100% of the cases. The patient is the second child of a five children family and the parents are fourth degree relatives. Although he has treated with antipsychotics, he had periods of remissions and relapses and never was symptoms free. Bidaki R, Zarei M, Mirhosseini SM, Moghadami S, Hejrati M, Kohnavard M, Shariati B. Adv Biomed Res. [6] Furthermore, many persons with WD will have psychiatric manifestations develop after diagnosis and initiation of management or after relapses.[8]. 2017 Dec 18;7(12):1290. doi: 10.1038/s41398-017-0027-0. conditionsPrivacy policy. To save content items to your account, Traditional or atypical antipsychotics have been used with the overall warning that these patients are very sensitive to antipsychotics and prone to develop multiple extrapyramidal side effects. Over the next few days, the psychotic symptoms became more pervasive and systematized; following which he was found to keep all his belonging under lock and key. In Wechsler test for adults (WAIS-III) mild mental retardation (verbal IQ = 60, full scale = 69) was detected. Wilson disease is a rare genetic disorder found in children in which large amounts of copper build up in the liver and brain. FOIA Akil M, Brewer GJ. Wilson's disease is one of the few neurogenetic diseases that can be treated. It prevents your body from getting rid of extra copper in your system. However, the diagnosis of Wilson's disease was made after 12 years of the first episode of psychosis. It has been shown that early detection and treatment of WD by Penicillamine therapy could improve the psychosis without the need of treating with neuroleptic medications.[9]. The https:// ensures that you are connecting to the Ophthalmological examination demonstrated the initial development of KayserFleischer (KF) rings in the clear anterior segment of the cornea. Department of Psychiatry, Psychiatry and Behavioral Sciences Research Center, Addiction Institute, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran. Krstic D, Antonijevic J, piric . Atypical case of Wilson's disease with psychotic onset, low 24 hour urine copper and the absence of Kayser-Fleischer rings. Brain. Drugs and zinc supplements. Inclusion in an NLM database does not imply endorsement of, or agreement with, Delusional disorder and schizophrenia-like psychosis are rare forms of psychiatric presentation. Wilson's disease, also known as hepatolenticular degeneration and progressive lenticular degeneration, is a rare genetic disorder that causes copper poisoning in the body. The association of hepatolenticular degeneration with schizophrenia. Similar cases have been described elsewhere, but to the best of our knowledge, this case represents the longest period over which a psychotic disorder was diagnosed and treated without uncovering the underlying cause of the symptoms (19 years). Nurses said that she had hysterical movements such as pseudoseizure and voluntary falling in the ward (the falling, in retrospect, was probably involuntary rather than voluntary). Cooper JD, Ozcan S, Gardner RM, Rustogi N, Wicks S, van Rees GF, Leweke FM, Dalman C, Karlsson H, Bahn S. Transl Psychiatry. FOIA 05 August 2011. Further, when diagnosed with psychosis associated with Wilson's disease, patients should be treated with low dose antipsychotics with close monitoring for extrapyramidal side effects. Mismanagement of Wilson's disease as psychotic disorder Saint-Laurent M. Schizophrenia and Wilson's disease. Jeon B, Kim JM, Jeong JM, Kim KM, Chang YS, Lee DS, et al. Amin R, Hasan R, Saha A, Hossain AR, Kahhar MA. The early presentations were psychiatric symptoms and epileptic seizures. ScienceDirect is a registered trademark of Elsevier B.V. Schizophrenia-like psychosis and delusional disorder are rare forms of psychiatric manifestations of WD. 2014 Jan-Feb;36(1):53-62. doi: 10.1016/j.genhosppsych.2013.08.007. FOIA Early recognition and prompt institution of treatment can delay the development of future complications of the disease and improve the long term outcome of the patient. Amin R, Hasan R, Saha A, Hossain AR, Kahhar MA. Normally, your liver gets rid of extra copper by sending it out in bile. 2008 Mar 15;266(1-2):104-8. doi: 10.1016/j.jns.2007.09.009. Inclusion in an NLM database does not imply endorsement of, or agreement with, Twelve years after his first psychiatric hospitalization, it was discovered that he was suffering from Wilson's disease. Serum copper level and total urine copper level were also estimated, reports of which were consistent with a diagnosis of Wilson's disease. Psychiatric manifestations in Wilson's disease: possibilities and difficulties for treatment. 2018 Jul;8(7):199-211. doi: 10.1177/2045125318759461. A Case Report of Misdiagnosis of Psychotic Symptoms Predominant Wilson's Disease. WD was diagnosed after laboratory tests; furthermore, the patient received a diagnosis of the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition Axis I psychotic disorder due to WD, with delusions. @kindle.com emails can be delivered even when you are not connected to wi-fi, but note that service fees apply. Find out more about the Kindle Personal Document Service. 8600 Rockville Pike The patient was referred to the general hospital for monitoring of her medical problem. Despite discontinuing his use of LSD, Wilson continued to hear voices, until . At the age of 17 years, the first set of severe psychiatric signs and symptoms surfaces, including negativism, commanding auditory hallucinations, persecutory delusions, and impaired verbal communication. [2] Copper is a trace element that acts as a cofactor for multiple enzymes . A prominent symptom of Wilson's disease is "sunflower eyes" or sunflower . In retrospect, the clinical picture was atypical, notably with an important neurologic involvement mainly parkinsonism almost uncontrollable and aggravated with neuroleptics. Patients with psychiatric symptoms with clues such as history of jaundice, family history of neuropsychiatric disorders, and sensitivity to typical/atypical antipsychotics should be evaluated for WD to avoid delay in diagnosis and associated morbidity. [29,30] Another case report described the development of akinetic-rigid syndrome with use of risperidone. An interdisciplinary approach with good collaboration of psychiatrists and neurologists is crucial for WD because early diagnosis and management without delay is an important for good prognosis. Chapter 27. Wilson's disease (WD) is an inherited autosomal recessive disorder that affects copper metabolism. In this admission, the laboratory results for liver function testing were as follows: total bilirubin = 2.4 mg/dl, direct bilirubin = 1.0 mg/dl, aspartate aminotransferase = 11 IU/L, and alanine aminotransferase = 31 U/L. Polymorphism of psychiatric manifestations in Wilson s disease- Romanian. An interdisciplinary approach with good collaboration of psychiatrists and neurologists is crucial for WD because early diagnosis and management without delay is an important for good prognosis. Wilson's disease - Wikipedia This Clinical Practice Guideline (CPG) has been developed to assist physicians and other healthcare providers in the diagnosis and management of patients with Wilson's disease. Wilson's disease. As is evident from the literature, Wilson's disease must be considered as a possibility in patients who develop psychosis at a young age and the patients must be adequately investigated for the same, which should include ultrasound abdomen, serum ceruloplasmin levels, serum copper level and total urine copper level. The gold standard for WD diagnosis is by liver biopsy with quantitative copper evaluation (copper values >3.1 mol/g of dry liver weight). Before It is caused by mutations in a gene on chromosome 13 that encodes ATP 7B, an adenosine triphosphatase involved in copper transportation across cell membranes [1, 2]. supportTerms and 1 The clinical symptoms are a result of organ dysfunction due to the direct or indirect effects of copper accumulation. Before Presentations with psychotic symptoms have been described less frequently. However, this did not lead to abatement of psychotic symptoms and required use of tab risperidone 1 mg/day, with which, within a week the psychotic symptoms resolved, and he was discharged from the hospital. Schizophrenia-like psychosis and delusional disorder are rare forms of psychiatric manifestations of WD. Numbers from liver function testing were as follows: aspartate aminotransferase = 14 IU/L (reference range, 837 IU/L), alanine aminotransferase = 8U/L (reference range, 837 IU/L). The diagnostic approach to patients with WD may be challenging and is based on a complex set of clinical findings that derive from patient history, physical examination, as well as laboratory and imaging testing. Indian J Psychiatry. Unable to load your collection due to an error, Unable to load your delegates due to an error. [6] The prevalence of psychiatric disorders in patients with WD varies wildly (major depressive disorder, 4%47%; psychosis, 1.4%11.5%). 2007;33:92432. Dening TR. Therefore, he has started to take antipsychotic medications including haloperidol 0.5 mg/day, chlordiazepoxide 25 mg/day, thioridazine 110 mg/day, and piracetam 800 mg daily was prescribed for him but without resulting in any significant changes in his symptoms and he had several periods of partial remissions and relapses. Psychiatric Manifestations in Wilson's Disease: A Cross-Sectional Psychiatric manifestations may precede the diagnosis of WD and other symptoms related to neurological or hepatic impairment. Copyright [1,2] The most common psychiatric manifestations in patients with Wilson's disease include sudden and changeable mood swings, anger outbursts, personality changes, depression, anxiety and cognitive impairment. Misidentification of Wilson Disease as Schizophrenia (1998 - PubMed She was eventually admitted to the Intensive Care Unit after a suicide attempt, in which she ingested alkali-based detergent. Wilson's Disease. Thrombocytopenia was present (plt: 44,000, 39,000, 12,000/mm3). Unauthorized use of these marks is strictly prohibited. Careers. Inclusion in an NLM database does not imply endorsement of, or agreement with, Current treatments cannot fix the copper metabolism. Wilson SK. The present observation found that early onset of psychiatric presentation and seizures is common in WD with white matter lesions in frontal lobes. [2], Psychiatric manifestation is strongly associated with WD and may precede neurological signs and symptoms. (Log in options will check for institutional or personal access. Accessibility 1912;34:295509. In the initial description of 12 cases of the disease, Wilson described presence of schizophrenia-like psychosis in two of his cases. But too much copper is toxic. The diagnosis made at that time was paranoid schizophrenia. The neuropsychiatry of Wilson's disease: A review. J Neuropsychiatry Clin Neurosci. Careers. Wilson disease is a metabolic disorder involving copper metabolism and is associated with abnormal liver functions. Psychiatric aspects of Wilson disease: A review. He had psychomotor agitation, was anxious and fearful. . HHS Vulnerability Disclosure, Help Dominant psychiatric manifestations in Wilson's disease: A diagnostic and therapeutic challenge! Total loading time: 0 Bethesda, MD 20894, Web Policies Harrison's Principles of Internal Medicine. It occurs more frequently in males and usually between the ages of 6 and 20 years. Wilson's disease (WD) is a rare, . Wilson's disease is an autosomal recessive genetic disorder that is caused by the mutation of the ATP7B gene. Epub 2013 Oct 9. Such a delay is particularly tragic as favorable outcomes depend on early discovery;[9] the patient died after her suicidal attempt that was spurred by auditory hallucinations. -. Accessibility cookies. Wilson's Disease Symptoms & Treatment | Children's Pittsburgh The patient was diagnosed with DSM-IV Axis I psychotic disorder due to WD, with delusions. and transmitted securely. [14] Other studies have also reported low prevalence of psychosis, viz., 1 out of 31,[15] 8 out of 71 cases,[16] 3 cases of organic delusional disorder out of 45 patients,[4] 2 out of 195 cases[5] and 2 out of 42 cases. [4,5] However, the prevalence of psychosis and catatonia is higher in patients with neurological manifestations of Wilson's disease.[6]. Reduced biliary excretion of copper results in its accumulation in the liver and other tissues (eg, brain . Symptoms may progress rapidly, especially in younger patients, but are more often gradual in development with periods of remission and exacerbation. Unusual mental symptoms in a case of hepatolenticular degeneration. HHS Vulnerability Disclosure, Help 8600 Rockville Pike Genetic testing. Saint-Laurent M. Schizophrenia and Wilson's disease. At the first movement neurological symptoms were misinterpreted as side effect of haloperidol, but consulted neurologist suggested additional diagnostic procedure which confirmed WD. Schizophrenia is a serious mental disorder in which people interpret reality abnormally. Journal of Psychiatry Romanian Journal of Psychiatry. Finally, 14 researches were entered into the study. It rarely manifests after 40 years of age. Schizophrenia may result in some combination of hallucinations, delusions, and extremely disordered thinking and behavior that impairs daily functioning, and can be disabling. J Neurosci Rural Pract. Progressive lenticular degeneration: A familial nervous disease associated with cirrhosis of liver. Diagnosis of Wilson's disease: an experience over three decades [11] Table 3 provides an overview of the case reports about this issue. A review of the literature and case report. No significant improvement in her condition occurred; because of this, other antipsychotic therapies (including clozapine) were used over a period of almost 19 years; she was admitted to eleven different psychiatric wards during this time, with different diagnoses emerging [Table 1]. He expressed delusion of reference and persecution. Wichowicz HM, Cubaa WJ, Sawek J. Wilson's disease associated with delusional disorder. [9] This results in abnormalities of copper metabolism and accumulation of copper in various tissues. National Library of Medicine Feature Flags: { [43], Co-occurrence of schizophrenic-like symptoms with WD is rare, and there are just a few case reports of WD with this type of psychiatric presentation. Indian J Psychol Med. Wilson's disease. The lack of recognition of these signs and symptoms as being attributable to WD often leads to delays in diagnosis and management. eCollection 2017. Wilson Disease - Disorders of Nutrition - The Merck Manuals Nineteen years after her first symptoms, it was discovered that the patient was suffering from WD. Federal government websites often end in .gov or .mil. Copper deposition occurs in hepatic parenchymal cells, the brain, the periphery of the iris, and the kidney. 2007 Dec;33(6):924-32. doi: 10.1016/j.encep.2006.08.009. PMC [8] The study which was conducted by Wichowicz and et al., demonstrates the importance of considering WD diagnosis in psychotic patients because of uncommon psychiatric manifestations of WD. Wilson's Disease: Symptoms, Causes, Treatment, and Outlook - WebMD 8600 Rockville Pike official website and that any information you provide is encrypted Patient's EEG was reported normal. -. doi: 10.1136/gpsych-2019-100066. government site. After six months of follow up, a significant improvement with his psychotic symptoms was observed. Wilson's Disease: Risk Factors, Causes, & Symptoms - Healthline Dening TR, Berrios GE. The psychiatric presentations of Wilson's disease. In Wilson's case, heavy amounts of drug abuse in the form of marijuana and LSD caused a significant amount of brain damage. Disclaimer. [5], In this case report, the patient with Wilson's disease presented by psychosis symptoms and were treated mistaken as a schizophrenic patient. 2003;78:1126-36. Keywords: The lack of recognition of these signs and symptoms as being attributable to WD often leads to delays in diagnosis and management. FOIA Gen Hosp Psychiatry. In Wilson disease, a rare hereditary disorder, the liver does not excrete excess copper into the bile as it normally does, resulting in accumulation of copper in the liver and liver damage. Epub 2013 Oct 9. Wilson's disease (WD) is a neurodegenerative disorder due to copper metabolism. In view of the clinical picture, a diagnosis of organic delusional disorder (F06.2) due to Wilson's disease was considered. Wilson's disease (WD) or hepatolenticular degeneration is an inherited neurodegenerative disorder of copper metabolism (autosomal recessive, chromosome13). 2019 Apr;7(Suppl 2):S59. The psychiatric features were usually misinterpreted as schizophrenia-like disease and the diagnosis was delayed, but their magnetic resonance imaging (MRI) showed asymmetric cerebral white matter lesions in the frontal lobe which revealed the diagnosis. The bane of a silent illness: When Wilson's disease takes its course. Dening TR, Berrios GE. The psychiatric symptoms improved with fewer neuroleptics than during the 12 previous years. We searched Google Scholar, Ovid, PsycINFO, CINHAL, and PubMed databases from 1985 to 2015. Finally, the abnormalities in his laboratory tests was considered as the side effect of some of his anti-psychotic medications like Clozapine which were discontinued at the time of diagnosis and patient was discharged from the hospital after ten days of therapy. Comprehensive text book of psychiatry. In addition to the above-mentioned symptoms, the child became very agitated and disinhibited. 2019 Jul 16;32(3):e100066. WD should be thought of in young patients presenting with acute psychosis, behavioural changes and features akin to schizophrenia. Before cookies. Dominant psychiatric manifestations in Wilson's disease: a diagnostic and therapeutic challenge! Note you can select to save to either the @free.kindle.com or @kindle.com variations. The https:// ensures that you are connecting to the 1989;46:112634. } This site needs JavaScript to work properly. on the Manage Your Content and Devices page of your Amazon account. Grover S, Sarkar S, Jhanda S, Chawla Y. Psychosis in an adolescent with Wilson's disease: A case report and review of the literature. Higher levels of serum copper in schizophrenic patients treated with depot neuroleptics.
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