Neurologic Wilson's disease

@article{Lorincz2010NeurologicWD,
  title={Neurologic Wilson's disease},
  author={Matthew T. Lorincz},
  journal={Annals of the New York Academy of Sciences},
  year={2010},
  volume={1184}
}
  • Matthew T. Lorincz
  • Published 1 January 2010
  • Medicine
  • Annals of the New York Academy of Sciences
Despite a long history, Wilson's disease, an autosomal recessive disease caused by mutations in the ATP7B gene, remains a commonly misdiagnosed import disease. Mutations in ATP7B result in abnormal copper metabolism and subsequent toxic accumulation of copper. Clinical manifestations of neurologic Wilson's disease include variable combinations of dysarthria, dystonia, tremor, and choreoathetosis. Among neurodegenerative diseases, it is unusual in that misdiagnosis and delay in treatment are… 
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TLDR
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TLDR
The drugs, also named as anticopper agents, are commonly used in clinics including D-penicillamine, trientine, sodium dimercaptosuccinate, dimerc aptosuccinic acid, zinc and tetrathiomolybdate are reviewed.
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TLDR
The aim of this chapter is to present a detailed description of the neurologic features of WD, including their evaluation, together with relevant ophthalmologic examinations, brain neuroimaging, and other laboratory measurements that show the extent of the involvement of the nervous system.
Symptomatic treatment of neurologic symptoms in Wilson disease.
TLDR
The aim of this chapter is to summarize possible treatments of neurologic symptoms in WD based on the presently available medical literature.
The hidden face of Wilson's disease.
TLDR
A new biological marker called 'relative exchangeable copper' (REC) facilitates diagnosis and familial screening and patient monitoring is important to ensure treatment adherence, efficacy and tolerability, and to detect rare complications such as copper deficiency induced by chronic copper chelation and hepatocarcinoma in patients with cirrhosis.
Wilson's disease masquerading as rheumatoid arthritis
TLDR
A case of Wilson's disease is presented that had a long prodrome of polyarthritis mimicking rheumatoid arthritis, without any concomitant hepatic or neuro-psychiatric manifestations.
Neurological Wilson Disease
TLDR
This review discusses possible neurological symptoms of Wilson disease, its clinical assessment, and differential diagnosis as well as tests that can lead to its diagnosis and treatment.
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References

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Wilson’s Disease: A Great Masquerader
Background: Wilson’s disease (WD) is a treatable autosomal recessive metabolic disorder which could lead to protean hepatic or neurologic manifestations. WD could mimic many neurologic disorders and
Worsening of neurologic syndrome in patients with Wilson's disease with initial penicillamine therapy.
TLDR
A patient with Wilson's disease is described who presented with neurologic disease, was treated with D-penicillamine, and suffered sudden neurologic deterioration coincident with therapy, suggesting that the cause of this distressing syndrome occurs frequently.
Late‐onset Wilson's disease with neurological involvement in the absence of Kayser‐Fleischer rings
TLDR
In patients with chronic, degenerative neurological disease, particularly when associated with hepatic dysfunction, the diagnosis of Wilson's disease should be considered regardless of age at onset or the absence of Kyser‐Fleischer rings on slit lamp examination.
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  • Medicine
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TLDR
The experience shows OLT is a life-saving procedure in patients with end-stage Wilson's disease and is associated with excellent long-term survival, and the neurological manifestation of the disease can improve significantly after OLT.
Wilson's disease. Psychiatric symptoms in 195 cases.
TLDR
The most common psychiatric features were abnormal behavior and personality change, although depression and cognitive impairment were also rated frequently, and many of the psychopathologic features seem to have an organic basis.
Wilson's disease: the problem of delayed diagnosis.
TLDR
The earliest symptoms and signs of neurological Wilson's disease were dysarthria or difficulty with the hands, or often both, and a "pseudosclerotic" picture was much more common in adults but dystonic and choreic symptoms were seen more often in children.
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TLDR
Oral zinc therapy, used according to the regimen, may now be considered in the treatment of patients with penicillamine intolerance, however, it is premature to convert patients to zinc therapy if they tolerate penicillsamine well.
Wilson disease in septuagenarian siblings: Raising the bar for diagnosis
TLDR
It is suggested that WD must be considered at all ages in patients with hepatic disease, neurological disease, or psychiatric symptoms, as well as environmental and extragenic factors are pivotal determinants of disease phenotype.
Wilson's disease and epilepsy.
TLDR
The relationship between Wilson's disease and epilepsy is explored, both in the literature and in a series of 200 cases of Wilson’s disease, with prognosis of seizures comparable with the best quoted figures for idiopathic epilepsy.
Wilson's disease: A longitudinal study of psychiatric symptoms
TLDR
Psychiatric cases whose psychiatric symptoms persisted to F2 differed from those who responded, in particular showing more dysarthria, incongruous behavior, and hepatic symptoms.
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