Atypical Presentation of Neuropsychiatric Variant of Wilson’s Disease and Clinical Improvement with Elemental Zinc Monotherapy
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https://doi.org/10.54169/ijocp.v3i02.81Keywords:
Wilson's disease, zinc, monotherapy, extrapyramidal symptoms, copperDimensions Badge
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Copyright (c) 2023 Simranjit Kaur, Utkarsh Kumar Tripathi, Abhishek Chakladar, Zaid Ahmed, Gaurav Verma, Alexander Martin Alphonse, Ankan Paul

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Wilson’s disease has been referred to as “the great masquerader” because it has a plethora of clinical manifestations based on the organ system involved.1 Current understanding is that neuropsychiatric WD develops after years of subclinical hepatic dysfunction.1 Neurologic dysfunction typically begins at approximately 20 years of age but can present earlier or later.2 In WD, the predominant neurologic manifestations (60%) are dysarthria, tremor and ataxia, followed by dystonia (15%) and parkinsonism (11%).3,4 In WD, pure psychiatric presentations are typically seen in patients in their teens. However, symptoms are often non-specific and frequently misdiagnosed as behavioral problems.5 Therefore, in many cases, when the clinical suspicion is low, the diagnosis of Wilson’s disease is often delayed or missed.Abstract
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Sean Cleymaet, Katsuko Nagayoshi, Edward Gettings & Justin Faden (2019): A review and update on the diagnosis and treatment of neuropsychiatric Wilson disease, Expert Review of Neurotherapeutics, DOI: 10.1080/14737175.2019.1645009 Merle U, Schaefer M, Ferenci P, et al. Clinical presentation, diagnosis and long-term outcome of Wilson’s disease: a cohort study. Gut. 2007; 56:115– 120. Członkowska A, Litwin T, Dzieżyc K, et al. Characteristics of a newly diagnosed Polish cohort of patients with neurological manifestations of Wilson disease evaluated with the Unified Wilson’s Disease Rating Scale. BMC Neurol. 2018; 18:34. Hedera P. Wilson’s disease: A master of disguise. Parkinsonism Relat. Disord. 2019. Carta M, Mura G, Sorbello O, et al. Quality of Life and Psychiatric Symptoms in Wilson’s Disease: the Relevance of Bipolar Disorders. Clin. Pract. Epidemiol. Ment. Health. 2012; 8:102–109. Sinha S, Taly AB, Ravishankar S, et al. Wilson’s disease: cranial MRI observations and clinical correlation. Neuroradiology. 2006; 48:613–621. Roberts EA, Schilsky ML. Diagnosis and treatment of Wilson disease: An update. Hepatology. 2008; 47:2089–2111. Brewer GJ, Dick RD, Johnson VD, et al. Treatment of Wilson’s disease with zinc: XV long-term follow-up studies. J. Lab. Clin. Med. 1998; 132:264– 278. Linn FHH, Houwen RHJ, van Hattum J, et al. Long-term exclusive zinc monotherapy in symptomatic Wilson disease: Experience in 17 patients. Hepatology. 2009;50:1442–1452
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