An acute encephalopathy with hepatic dysfunction stemming from mitochondrial damage.
Etiology remains unknown, but viral infections, exogenous toxins, drugs, and inborn errors of metabolism have been implicated. Aspirin is classically associated but data are lacking for a definitive cause-effect relationship.
Usually presents after a preceding viral infection with acute onset of profuse vomiting and altered mental status ranging from a personality change to coma.
Laboratory findings include elevated ammonia, ALT/AST, and prolonged PT, without an increase in bilirubin. Liver biopsy shows steatosis without significant inflammation.
Treatment includes intensive supportive care, correction of metabolic abnormalities, and control of intracranial pressure.
Prognosis is generally good, and liver function returns to normal. However, encephalopathy can result in permanent neurologic sequelae.
- altered mental status
- abnormal pupillary response
- diminished pain response
- absence of, or minimal, jaundice and scleral icterus
1st Tests To Order
- serum electrolytes
- serum glucose
- serum ammonia
- urine/serum toxicology
- blood gas
Other Tests to Consider
- cranial CT
- LP with CSF analysis
- liver biopsy
- metabolic testing
- supportive care
- with fluid overload
- with stage 2 disease or greater
- intensive monitoring
- with hypotension
- intravenous fluids ± vasopressors
- with hypoglycemia
- intravenous dextrose
- with acidosis: pH <7.0
- slow intravenous bicarbonate infusion
- with hyperammonemia
- sodium phenylacetate/sodium benzoate
- with raised intracranial pressure (ICP)
- ICP-lowering regimen
- with seizures
- with coagulopathy
- fresh frozen plasma and/or platelet transfusion