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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:

MRI of the head
EEG (may show general slowing of brain waves)
CT scan of the head

Liver anatomy

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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