Wilson's disease


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Related to Wilson's disease: Huntington's disease

Wilson's disease

 [wil´sunz]
a rare progressive disease, inherited as an autosomal recessive trait and due to a defect in metabolism of copper, with accumulation of copper in the liver, brain, kidney, cornea, and other tissues. Characteristics include cirrhosis of the liver and degenerative changes in the brain, particularly the basal ganglia. Liver disease is the most likely manifestation in children; neurologic disease is most common in young adults. The characteristic ophthalmic feature is a pigmented ring (Kayser-Fleischer ring) at the outer margin of the cornea. Called also hepatolenticular degeneration.

Wilson's disease

Neurology An AR disorder of copper metabolism characterized by accumulation of copper in the brain, liver, and other organs Clinical Cirrhosis, degeneration of basal ganglia and neurologic deterioration with involuntary movement, tremors, muscular rigidity, spastic contractures, psychiatric defects, dysphagia, Kayser-Fleischer ring. See Ceruloplasmin, Copper.

Wilson's disease

A rare genetic disorder in which copper accumulates in the body, especially in the liver and brain, causing CIRRHOSIS and brain damage. There are behaviour abnormalities and personality changes, writhing movements of the limbs (athetoid movements), muscle rigidity and shortening, dementia and, in untreated cases, death. If diagnosed early, often by the observation of a ring of greenish-brown discoloration in each cornea (KAYSER-FLEISCHER RING), the condition may be controlled with a diet low in copper and the use of the drug d-penicillamine, which binds copper into a form which is excreted in the urine. Also known as hepato-lenticular degeneration. (Samuel Alexander Kinnier Wilson, 1878–1937, American-born English neurologist).

Wilson's disease

An inborn defect of copper metabolism in which free copper may be deposited in a variety of areas of the body. Deposits in the brain can cause tremor and other symptoms of Parkinson's disease.

Patient discussion about Wilson's disease

Q. Hi this is Wilson; I have a daughter, who is 5. She has frequent nose bleeds when she is behaving badly. Hi this is Bell; I have a daughter, who is 5. She has frequent nose bleeds when she is behaving badly. She has been screened by her school for ADHD and I haven’t had a chance to consult a doctor for her screening and exam for ADHD. I thought that her nose bleeds cause due to her behavior? I need help.

A. I don’t have an idea about this. But I think that you should first discuss these issues with your doctor. Also mention to the doctor if she has any problems with sleeping, like only sleeping 6 hours a night. Try with occupational Therapists which help children and adults with small motor issues like handwriting and also with sensory issues like learning how to calm down. Early intervention is always best.

More discussions about Wilson's disease
References in periodicals archive ?
Wilson's disease asociated with delusional disorder.
Hiejima et al., "Acute lymphoblastic leukemia in a girl with Wilson's disease," Pediatrics International, vol.
Penicillamine challenge test in the diagnosis of Wilson's disease. Mymen singh Med J 2014; 23: 489-95.
Wilson's disease and other neurological copper disorders.
Ferenci in his mammoth research also concludes that there has been no single large randomised controlled trial that establishes a consensus on Wilson's disease diagnosis [12].
Millonig, "Fulminant liver failure in Wilson's disease with histologic features of postinfantile giant cell hepatitis; cytomegalovirus as the trigger for both?" European Journal of Gastroenterology & Hepatology, vol.
Synovial copper deposition as a possible explanation of arthropathy in Wilson's disease. Bull Hosp Jt Dis 1993;52:46-9.
The most common neuro-radiological finding of Wilson's disease on MR is bilaterally symmetric T2/FLAIR hyperintensity in the putamina (70%), caudate nuclei (60%), ventrolateral thalami (55-60%), and midbrain (50%) (31-41).
Aim: Wilson's disease is an autosomal recessive disorder of copper metabolism which leads to copper overload in different tissues of the body.
The main manifestations of Wilson's disease (WD) are hepatic dysfunction and a broad spectrum of movement disorders with parkinsonian, dystonic, ataxic, and choreatic characteristics.
Valeant Pharmaceuticals quadrupled the price for Cuprimine, a drug required for treatment of Wilson's disease. For the patient with Medicare, the price for a month's supply jumped overnight from $9000 to $35,000, with the patient's out-of-pocket expense being $1800.