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Hepatocerebral degeneration - Symptom

Alternative Names

Chronic acquired (Non-Wilsonian) hepatocerebral degeneration

Symptoms:

Symptoms may include:

  • Difficulty walking
  • Impaired intellectual function
  • Jaundice
  • Muscle spasm (myoclonus)
  • Rigidity
  • Shaking of arms, head (tremor)
  • Twitching
  • Uncontrolled body movements (chorea)
  • Unsteady walking (ataxia)

Signs and tests:

Signs include:

  • Coma
  • Fluid in the abdomen that causes swelling (ascites)
  • Gastrointestinal bleeding from enlarged veins in the food pipe (esophageal varices)

A nervous system (neurological) examination may show signs of:

  • Dementia
  • Involuntary movements
  • Walking instability

Laboratory tests may show a high ammonia level in the bloodstream and abnormal liver function.

Other tests may include:

  • Reviewed last on: 7/15/2008
  • Luc Jasmin, MD, PhD, Department of Neurosurgery and Gene Therapeutics Research Institute, Cedars-Sinai Medical Center, Los Angeles, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Wijdicks EF, Wiesner RH. Acquired (non-Wilsonian) hepatocerebral degeneration: complex management decisions. Liver Transpl. 2003 Sep;9(9):993-4.

Burkhard PR, Delavelle J, Du Pasquier R, Spahr L. Chronic parkinsonism associated with cirrhosis: a distinct subset of acquired hepatocerebral degeneration. Arch Neurol. 2003 Apr;60(4):521-8.

Ferrante MA. Endogenous Metabolic Disorders. In: Goetz CG. Goetz: Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 38.

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