Case of pediatric acquired chronic hepatocerebral degeneration.
Acquired chronic hepatocerebral degeneration is a central nervous system disorder secondary to several conditions related to hepatic dysfunction. Clinical features of acquired chronic hepatocerebral degeneration include a hyperkinetic extrapyramidal syndrome, neuropsychiatric symptoms, or both. We present for the first time a pediatric case of acquired chronic hepatocerebral degeneration secondary to endstage biliary disease. The pediatric phenotype of acquired chronic hepatocerebral degeneration is presented, and the differential diagnosis in regard to Wilson's disease and management alternatives are discussed.
Papapetropoulos
Spiridon
S
Division of Movement Disorders, Department of Neurology, Miller School of Medicine, University of Miami, Miami, Florida 33136, USA. spapapetropoulos@med.miami.edu
Tzakis
Andreas
A
Sengun
Cenk
C
Reddy
Chakradhar
C
Boukas
Konstantinos
K
Zitser
Jennifer
J
Singer
Carlos
C
D000293:Adolescent; D001284:Atrophy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D001480:Basal Ganglia Diseases / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D001659:Biliary Tract / Q000473:pathology / Q000503:physiopathology; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000503:physiopathology; D002908:Chronic Disease; D003072:Cognition Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005917:Globus Pallidus / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D006501:Hepatic Encephalopathy / Q000209:etiology / Q000503:physiopathology*; D006527:Hepatolenticular Degeneration / Q000175:diagnosis; D006801:Humans; D008099:Liver / Q000473:pathology / Q000503:physiopathology; D017093:Liver Failure / Q000150:complications / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008345:Manganese / Q000378:metabolism; D019965:Neurocognitive Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D017211:Treatment Failure; D014815:Vitamins / Q000627:therapeutic use
D014815:Vitamins; D008345:Manganese