Neurohepatic Degenerations
《英文msh詞典》Neurohepatic Degenerations
[入口詞] Neurohepatic Degenerations
[主題詞] Hepatolenticular Degeneration
[英文釋義] 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)