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hepatolenticular degeneration
Used for:
cerebral pseudosclerosis
Used for:
familial hepatitis
Used for:
Gowers' chorea
Used for:
hepatocerebral degeneration
Used for:
Kinnier-Wilson disease
Used for:
neurohepatic degeneration
Used for:
Westphal pseudosclerosis
Synonym for:
Westphal Strumpell disease
Synonym for:
Wilson's disease
Broader Terms:
inborn metal metabolism disorder
Broader Terms:
liver disorder
Related Terms:
autosomal recessive trait
Scope Note:
rare autosomal recessive disease characterized by the deposition of copper in the brain, liver, cornea and other organs; clinical features include liver cirrhosis, liver failure, splenomegaly, tremor, bradykinesia, dysarthria, chorea, muscle rigidity, Kayser-Fleischer rings (pigmented corneal lesions), ataxia and intellectual deterioration; hepatic dysfunction may precede neurologic dysfunction by several years.
Term Number:
1849-4349
Send your comments to: Melody Lowe