单词 | Cerebral Pseudoscleroses |
释义 | Cerebral Pseudoscleroses《英文msh词典》Cerebral Pseudoscleroses [入口词] Cerebral Pseudoscleroses [主题词] Hepatolenticular Degeneration [英文释义] 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) |
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