Hepatic encephalopathy

Hepatic encephalopathy (HE), also known as portosystemic encephalopathy (PSE), refers to a spectrum of neuropsychiatric abnormalities occurring in patients with liver dysfunction and portal hypertension. It results from exposure of the brain to excessive amounts of ammonia

Hepatic encephalopathy (HE) specifically refers to an encephalopathy occurring in patients with acute liver failure, a portosystemic shunt, or chronic liver disease. HE is generally regarded as a reversible condition, although the long-term sequelae are being further studied 11.

Hepatic encephalopathy may be subclassified according to severity and pattern (acute episode, recurrent, or persistent) 11

Chronic neurological damage ascribed to repeated episodes of hepatic encephalopathy is usually termed acquired hepatocerebral degeneration, which is discussed separately.

For the purposes of this article, we use the terms separately and discussion here is limited to acute hepatic encephalopathy.

The vast majority of patients have portosystemic shunts in the setting of cirrhosis, either from the development of spontaneous shunting or as a result of transjugular intrahepatic portosystemic shunting (TIPS) 7. The clinical spectrum can rarely manifest in individuals who have portosystemic bypass without any associated intrinsic hepatocellular disease. The broader term "portosystemic encephalopathy" can be used for this reason.

The clinical manifestations of hepatic encephalopathy range widely from chronic episodic subclinical neurological dysfunction to acute fulminant neurological impairment, coma and death 4.

In the majority of patients, a superimposed precipitating cause rather than worsening of hepatocellular function can be identified (particularly in acute situations). Such precipitants include:

  • infection (most common): 50% 4
  • excessive nitrogen-containing intestinal load
  • reduced nitrogen excretion
    • constipation
    • renal failure
  • metabolic or drug interactions
    • hyponatremia or hypokalemia (e.g. from diuretics)
    • sedatives

In hepatic encephalopathy, due to portosystemic shunting, ammonia from the digestive system or from elsewhere, which is usually metabolized by the liver reaches the systemic circulation. Although the exact mechanism of neurotoxicity has not been elucidated, ammonia is taken up by the brain and has been shown to be toxic to both astrocytes and neurons 4.

MR imaging features of acute hyperammonemia include:

  • T2/FLAIR
    • mild
      • symmetric high signal within the insula (most common), thalamus, and posterior limbs of the internal capsule, and cingulate gyrus 5-7
      • often reversible with therapy 5,6
    • severe
      • diffuse cortical edema and hyperintensity
      • perirolandic and occipital regions are typically spared
  • DWI
    • similar distribution to T2/FLAIR
    • reverse with appropriate therapy
  • SWI
    • nearly one-half have microhemorrhages of white matter or cortex 8
      • number and presence of microhemorrhages do not affect the outcome
  • MR spectroscopy
    • may show an elevated glutamine/glutamate peak coupled with decreased myoinositol and choline signals on proton MR spectroscopy 1,7

As the vast majority of cases of acute hepatic encephalopathy will be encountered in patients with established and chronic liver disease, chronic findings of acquired hepatocerebral degeneration are also usually present.

Acute hepatic encephalopathy can be rapidly fatal, while chronic hepatic encephalopathy tends to be a more indolent process. Identifying and treating precipitating cause is important, and leads to resolution in up to 80% of patients 4.

Treatment usually entails the careful cessation of any offending agents (e.g. toxins, chemotherapeutic agents, antiepileptic agents), treating contributing conditions (e.g. sepsis, GI bleeding), nutritional support (including the use of lactulose), and in some cases hemofiltration to remove excess ammonia.

Notably, the extent of severity on FLAIR and DWI correlates with the plasma ammonia level 5. More severe cases of diffuse cortical involvement (particularly on DWI) can be reversible but are more likely to be fatal, indicating the need for prompt therapy to treat the hyperammonemia in acute hepatic encephalopathy 5.

The differential diagnosis includes other causes of hyperammonemic encephalopathy 6,10

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Article information

rID: 21635
Synonyms or Alternate Spellings:
  • Hepatic encephalopathy (HE)
  • Portosystemic encephalopathy (PSE)
  • Hyperammonemic encephalopathy
  • Portosystemic encephalopathy
  • Hyperammonaemic encephalopathy

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