Neurologic Wilson's disease

  title={Neurologic Wilson's disease},
  author={Matthew T. Lorincz},
  journal={Annals of the New York Academy of Sciences},
  • 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… 

122 Wilson ’ s Disease

This review discusses the epidemiology, genetics, clinical features, etiopathophysiology, diagnostic tests, and treatment of Wilson’s disease.

Wilson Disease

The case of a 23 year old male, presenting with psychiatric symptoms (depressions, insomnia) which progressed despite pshyciatric treatment, which was diagnosed with Wilson disease by genetic testing.

Wilson's disease: Case report from Maharashtra

In young adults, neuropsychiatric symptoms predominate and include dystonia, tremor, personality changes, and cognitive impairments secondary to copper accumulation in the central nervous system.

Wilson disease: neurologic features.

Current Drug Managements of Wilson's Disease: From West to East

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.

Symptomatic treatment of neurologic symptoms in Wilson disease.

Neurological Wilson Disease

Neurologic Wilson disease: case series on a diagnostic and therapeutic emergency

Two cases of neurologic Wilson disease with progressive movement disorder and Kayser-Fleischer rings with low serum copper, low ceruloplasmin, and increased 24-hour urine copper against a background of normal transaminases are presented.



Wilson's disease

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.

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

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.

Liver transplantation for Wilson's disease: a single-center experience.

  • B. EghtesadN. Nezakatgoo J. Rakela
  • Medicine
    Liver transplantation and surgery : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
  • 1999
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.

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.

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.

Oral zinc therapy for Wilson's disease.

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

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: A longitudinal study of psychiatric symptoms