Case of pediatric acquired chronic hepatocerebral degeneration

Pediatr Neurol. 2008 Jan;38(1):67-70. doi: 10.1016/j.pediatrneurol.2007.09.010.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Atrophy / etiology
  • Atrophy / pathology
  • Atrophy / physiopathology
  • Basal Ganglia Diseases / etiology
  • Basal Ganglia Diseases / pathology
  • Basal Ganglia Diseases / physiopathology*
  • Biliary Tract / pathology
  • Biliary Tract / physiopathology
  • Brain / pathology
  • Brain / physiopathology
  • Cholestasis, Intrahepatic / complications
  • Cholestasis, Intrahepatic / physiopathology
  • Chronic Disease
  • Cognition Disorders / etiology
  • Cognition Disorders / pathology
  • Cognition Disorders / physiopathology
  • Diagnosis, Differential
  • Disease Progression
  • Fatal Outcome
  • Female
  • Globus Pallidus / metabolism
  • Globus Pallidus / pathology
  • Globus Pallidus / physiopathology
  • Hepatic Encephalopathy / etiology
  • Hepatic Encephalopathy / physiopathology*
  • Hepatolenticular Degeneration / diagnosis
  • Humans
  • Liver / pathology
  • Liver / physiopathology
  • Liver Failure / complications
  • Liver Failure / physiopathology*
  • Magnetic Resonance Imaging
  • Manganese / metabolism
  • Neurocognitive Disorders / etiology
  • Neurocognitive Disorders / pathology
  • Neurocognitive Disorders / physiopathology*
  • Treatment Failure
  • Vitamins / therapeutic use

Substances

  • Vitamins
  • Manganese