Hepatolenticular Degeneration
| Definition: |
A 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. (From Adams et al., Principles of Neurology, 6th ed pp969-71) |
| Notes: |
lenticular refers to the lenticular nucleus in the brain |
| Also Called: |
Hepatic Form of Wilson Disease |
Hepatolenticular Degeneration Definitions and Terms
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