Symptoms:

Symptoms may include:

• Tremor (shaking of arms, head)
• Ataxia (unsteady walking)
• Difficulty walking
• Chorea (uncontrolled body movements)
• Myoclonus (spasm of muscles)
• Impairment of intellectual function
• Coma
• Jaundice
• Rigidity
• Gastrointestinal bleeding from esophageal varices (enlarged veins in the food pipe)
• Ascites (fluid in the abdomen, causing swelling)

Signs and tests:

A thorough neurological examination may show signs of dementia, involuntary movements, and walking instability. Laboratory tests may show a high ammonia level in the bloodstream and abnormal liver function test results.

A head CT scan or a cranial MRI may not show any abnormalities, or may show non-specific changes in the basal ganglia region of the brain. An EEG test may show general slowing of brain waves, which is consistent with an abnormal metabolic (chemical breakdown) process.