title: Psychological presentations without hepatic involvement in Wilson disease.
abstract: Wilson disease is an autosomal recessive inborn error of copper metabolism that leads to neurologic symptoms and variable degrees of hepatic damage. The most common characteristic signs clinically are liver disease, psychiatric disease, neurologic disease, or a combination of these. Early recognition by means of clinical signs and an early initiation of therapy using chelators or zinc-salts are essential for a good outcome and prognosis. This report describes a male suffering from Wilson disease who exhibited an unusual presentation that included psychological manifestations without hepatic involvement. He was initially treated for attention-deficit hyperactivity disorder and a seizure disorder until brain imaging established the diagnosis of Wilson disease.
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Chang Gung Children's Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. lincgh@adm.cgmh.org.tw
authors.forename: Jainn-Jim
authors.initials: JJ
authors.lastname: Lin
authors.forename: Kuang-Lin
authors.initials: KL
authors.lastname: Lin
authors.forename: Huei-Shyong
authors.initials: HS
authors.lastname: Wang
authors.forename: Mun-Ching
authors.initials: MC
authors.lastname: Wong
mesh_terms: D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis / Q000188:drug therapy / Q000523:psychology*; D001921:Brain / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D002648:Child; D003300:Copper / Q000652:urine; D003937:Diagnosis, Differential; D004292:Dominance, Cerebral / Q000502:physiology; D004359:Drug Therapy, Combination; D004828:Epilepsies, Partial / Q000175:diagnosis / Q000188:drug therapy / Q000523:psychology*; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000188:drug therapy / Q000523:psychology*; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000523:psychology*; D008111:Liver Function Tests; D008279:Magnetic Resonance Imaging; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use; D011889:Rage / Q000187:drug effects / Q000502:physiology*; D025101:Vitamin B 6 / Q000627:therapeutic use; D017967:Zinc Compounds / Q000627:therapeutic use
chemical_list: D017967:Zinc Compounds; D003300:Copper; D025101:Vitamin B 6; D010396:Penicillamine