Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0233565 (bradykinesia)
 2,352 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Portal-systemic encephalopathy may be seen with hyperammonemia that complicates chronic liver disease. We report an unusual case of reversible parkinsonism associated with hyperammonemia and portal vein thrombosis. An active 90-year-old male developed motor slowing and resting hand tremor over 6 months. Examination showed asterixis, bradykinesia, cogwheel rigidity, rest tremor, and a parkinsonian gait. Serum venous ammonia was elevated at 145 microM. The next day, the patient became comatose and serum ammonia was 178 microM. With lactulose therapy, serum ammonia level normalized and examination showed only minimal parkinsonism after 1 week. An abdominal CT scan identified portal vein thrombosis with porto-systemic shunting that reversed after 7 months of treatment. Examination 2 years later showed no signs of parkinsonism. Parkinsonism can dominate the clinical picture of patients with hyperammonemia before the onset of encephalopathy.
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PMID:Reversible parkinsonism and hyperammonemia associated with portal vein thrombosis. 1124 May 69

Acquired (non-Wilsonian) hepatocerebral degeneration is a rare irreversible neurological syndrome that occurs in patients with chronic liver disease associated with multiple metabolic insults. Van Woerkom was the first to describe acquired hepatocerebral degeneration in 1914 followed by the landmark article by Victor et al in 1965. Multiple bouts of hepatic coma are the only known risk factors that trigger this devastating neurodegenerative disease with features suggesting toxic exposure to the brain. Clinically and pathologically the disorder is similar to Wilson's disease although subtle differences in immunostaining of glial fibrillar acidic protein have been documented. Acquired hepatocerebral degeneration occurs in 0.8-2% of patients with cirrhosis. As acquired hepatocerebral degeneration is relatively rare, we are reporting one such case from our hospital in a 50-year-old male patient who had long-standing portal systemic shunt and presented with progressive cognitive decline, bradykinesia, tremors and bilateral extrapyramidal signs.
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PMID:Acquired hepatocerebral degeneration. 2378 Jul 67