Nonmotor Manifestations of Wilson's Disease

Biswas, Samar; Paul, Neelanjana; Das, Shyamal Kumar

doi:10.1016/bs.irn.2017.04.010

Cited by 15 publications

(8 citation statements)

References 55 publications

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“…Even though psychiatric disturbances have been reported in 50% to 70% of Wilson's disease (WD) patients, < 10% showed psychosis, suggesting that this manifestation is not a hallmark of the disease . On the other hand, psychotic symptoms at disease onset may be present in 35% of patients, with an interval between onset of psychosis and diagnosis of WD of 2.4 years . This poses an important differential diagnosis with schizophrenia.…”

Section: Resultsmentioning

confidence: 99%

“…179,[181][182][183] On the other hand, psychotic symptoms at disease onset may be present in 35% of patients, 180,184 with an interval between onset of psychosis and diagnosis of WD of 2.4 years. 185 This poses an important differential diagnosis with schizophrenia. In most cases in which psychotic symptoms were the first manifestation of WD, these were paranoid delusions and hallucinations.…”

Section: Wilson's Diseasementioning

confidence: 99%

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Nosology and Phenomenology of Psychosis in Movement Disorders

Rossi

Farcy

Starkstein

et al. 2020

Movement Disord Clin Pract

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Background Background: Psychotic symptoms, such as delusions and hallucinations, are part of the clinical picture of several conditions presenting movement disorders. Phenomenology and epidemiology of psychosis in Parkinson's disease have received wide attention; however, the presence of psychosis in other movement disorders is, comparatively, less well known. Objectives Objectives: To review psychotic symptoms present in different movement disorders. Methods Methods: A comprehensive and structured literature search was performed to identify and analyze data on patients with movement disorders and comorbid psychosis.

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Section: Resultsmentioning

confidence: 99%

Section: Wilson's Diseasementioning

confidence: 99%

Nosology and Phenomenology of Psychosis in Movement Disorders

Rossi

Farcy

Starkstein

et al. 2020

Movement Disord Clin Pract

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“…WD is now known to be more prevalent than once believed, particularly because of identification of ATP7B as a causative gene [3]. Indeed, because abnormal biliary excretion of copper owing to ATP7B gene mutation is a primary etiology of WD, liver abnormalities are the most common initial manifestation, and 40-70% patients diagnosed with WD exhibit liver lesions [4]. In such cases, the coexistence of asymptomatic and unexplained cirrhosis is a key feature in diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin a nephropathy as the first clinical presentation of Wilson disease: a case report and literature review

Zhang

Geng

et al. 2021

BMC Gastroenterol

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Background Wilson disease (WD) is a rare genetic disorder of copper metabolism. Differences in copper tissue accumulation lead to various clinical manifestations, including some atypical presentations. The complex clinical features of WD make diagnosis challenging, delaying the best chance for treatment. Case presentation We report a case of a 26-year-old man with nephritis-range proteinuria and elevated serum creatinine. The renal pathology indicated immunoglobulin A (IgA) nephropathy and tubular injury, which was inconsistent with glomerular lesions. Cirrhosis was also detected by imaging examination. Considering both kidney injury and liver damage, WD was suspected. Based on results showing abnormal copper metabolism, corneal Kayser–Fleischer rings, and genetic disorders in the ATP7B gene, the patient was finally diagnosed with WD. After treatment with oral penicillamine, zinc sulfate and losartan, the patient showed alleviation of both WD and nephropathy after 3 years of follow-up. He maintained a good quality of daily life. Conclusion This case highlights that unexplained neurological and liver symptoms in patients with IgA nephropathy can be clues for WD.

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“…Regardless of mutation type in the ATP7B gene, the impaired P-type ATPase leads to copper accumulation in multiple organs with fatal consequences if untreated (53). Typically liver, but also brain, nerves and other tissues are targeted by copper deposition and copper toxicity is manifested through failure of these organs (54)(55)(56). Formation of Kayser-Fleischer rings, copper deposits in the cornea, are visual manifestations of Wilson disease in its developed phase and the deposits are clearly visible in the eyes of patients through a detailed examination using slit lamps (57,58 termined disorder, the manifestation and progression depends on the fact whether the mutations are homozygous or heterozygous (59).…”

Section: Wilson and Menkes Disease As Genetically Determined Disordermentioning

confidence: 99%

Copper and copper nanoparticles toxicity and their impact on basic functions in the body

Pohanka¹

2019

BLL

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Copper is a biogenic metal having multiple functions in basic processes in organisms and it is common in all kingdoms of life. Limited intake of copper is a problem; however, doses of copper exceeding the recommended alimentary source are problematic as well and toxicity is soon manifested. Impact of copper nanoparticles on human health is another serious issue taken into consideration in this review. Regarding to copper toxicity, neurodegenerative disorders including Alzheimer and Parkinson diseases are suspected to be linked to copper toxicity or copper can contribute to their progression. Wilson and Menke diseases are also described as examples of copper intolerance. This paper is focused on the description, literature survey and discussion of the current knowledge about copper and copper nanoparticles toxicity and their involvement in various pathological processes (Tab. 6, Fig. 2, Ref. 171).

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Nonmotor Manifestations of Wilson's Disease

Cited by 15 publications

References 55 publications

Nosology and Phenomenology of Psychosis in Movement Disorders

Nosology and Phenomenology of Psychosis in Movement Disorders

Immunoglobulin a nephropathy as the first clinical presentation of Wilson disease: a case report and literature review

Copper and copper nanoparticles toxicity and their impact on basic functions in the body

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