Psychiatric Aspects of Wilson's Disease (Hepatolenticular Degeneration): Results of Psychometric Tests During Long-Term Therapy

Goldstein, Norman; Ewert, Josephine C.; Randall, Raymond V.; Gross, John B.

doi:10.1176/ajp.124.11.1555

Cited by 53 publications

(22 citation statements)

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“…KF rings are not exclusive to Wilson's disease and can uncommonly be found in other obstructive liver disease and primary biliary cirrhosis. 34 Sunflower cataracts are seen less commonly than KF rings, present in about17%. 34 Sunflower cataract do not impair vision, cannot be seen with the unaided eye or with an ophthalmoscope and require slit-lamp evaluation for detection.…”

Section: Ophthalmologic Manifestationsmentioning

confidence: 99%

“…34 Sunflower cataracts are seen less commonly than KF rings, present in about17%. 34 Sunflower cataract do not impair vision, cannot be seen with the unaided eye or with an ophthalmoscope and require slit-lamp evaluation for detection. With treatment, KF rings and sunflower cataract become less prominent and can gradually disappear.…”

Section: Ophthalmologic Manifestationsmentioning

confidence: 99%

“…With treatment, KF rings and sunflower cataract become less prominent and can gradually disappear. 34 Other less common ophthalmologic findings include slowing of saccades, impaired up gaze, and strabismus. …”

Section: Ophthalmologic Manifestationsmentioning

confidence: 99%

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Neurologic Wilsons Disease and its Management - An Update

2014

Bangladesh J Child Health

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Wilsons disease is an inborn error of copper metabolism caused by mutations in the ATP7B gene. The disease has an autosomal recessive mode of inheritance and is characterized by excessive copper deposition, predominantly in the liver and brain. Clinical manifestations of neurologic Wilsons disease include variable combinations of dysarthria, dystonia, tremor, and choreoathetosis. Among neurodegenerative disorders. WD is preventable and treatable if they are diagnosed treated early. Untreated case of Wilsons disease progresses to severe neurologic disability and death, while those adequately treated have normal life spans. The traditional treatment for WD is based on copper chelation with agents such as D-penicillamine, but use of this drug has been questioned because of reported side effects. The use of agents such as Zinec, trientine and ammonium tetrathiomolybdate has been advocated, although results of long-term trials are awaited. This review focuses on the neurologic features of Wilsons disease and treatment options. DOI: http://dx.doi.org/10.3329/bjch.v37i3.18620 Bangladesh J Child Health 2013; Vol.37(3): 158-169

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Section: Ophthalmologic Manifestationsmentioning

confidence: 99%

Section: Ophthalmologic Manifestationsmentioning

confidence: 99%

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Neurologic Wilsons Disease and its Management - An Update

2014

Bangladesh J Child Health

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“…CT and MRI show variable evidence of amelioration of brain lesions (usually basal ganglia lesions) after onset of treatment Rothfus et al, 1988). Some studies have reported slight to moderate improvements in intellectual functioning after treatment (Goldstein et al, 1968;Rosselli et al, 1987), although the lack of control for practice effects in these studies may have confounded the results. Some patients show an initial (and sometimes irreversible) deterioration upon initiation of pharmacotherapy, in spite of excretion of copper from the body.…”

Section: Wilson's Diseasementioning

confidence: 99%

Cognitive deficits related to major organ failure: The potential role of neuropsychological testing

Farmer

1994

Neuropsychol Rev

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Until recently, little attention has been paid to the possibility of cognitive deficits in patients with disease or failure of major organs such as the liver, kidney, or heart. However, there is a growing awareness that major organ failure often has neuropsychological sequelae. These sequelae may at times be quite subtle and not detectable under gross examination. Nevertheless, even subtle deficits may have a major impact on adherence to medical regimens, psychosocial adjustment, and quality of life of patients. Neuropsychological assessment has a potentially valuable role to play both in research and in clinical work. It can be useful in adding to our knowledge of the cognitive effects of various types, severity and duration of major organ disease, as well as sequelae associated with treatment. It also is a potentially valuable clinical tool for identifying cognitive deficits that will affect the quality of life and probability of survival for organ failure patients.

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“…There are a few publications addressing psychiatric manifestations of WD as the predominant feature [1][2][3][4][5][6][7][8]. The present study describes 15 patients with WD who had significant psychiatric co-morbidity at onset that posed diagnostic and therapeutic challenges.…”

Section: Introductionmentioning

confidence: 99%

Dominant psychiatric manifestations in Wilson's disease: A diagnostic and therapeutic challenge!

Srinivas¹,

Sinha

Taly

et al. 2008

Journal of the Neurological Sciences

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Psychiatric Aspects of Wilson's Disease (Hepatolenticular Degeneration): Results of Psychometric Tests During Long-Term Therapy

Cited by 53 publications

References 2 publications

Neurologic Wilsons Disease and its Management - An Update

Neurologic Wilsons Disease and its Management - An Update

Cognitive deficits related to major organ failure: The potential role of neuropsychological testing

Dominant psychiatric manifestations in Wilson's disease: A diagnostic and therapeutic challenge!

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Psychiatric Aspects of Wilson's Disease (Hepatolenticular Degeneration): Results of Psychometric Tests During Long-Term Therapy

Cited by 53 publications

References 2 publications

Neurologic Wilsons Disease and its Management - An Update

Neurologic Wilsons Disease and its Management - An Update

Cognitive deficits related to major organ failure: The potential role of neuropsychological testing

Dominant psychiatric manifestations in Wilson's disease: A diagnostic and therapeutic challenge!

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Product

Resources

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Neurologic Wilsons Disease and its Management - An Update

Neurologic Wilsons Disease and its Management - An Update