Portosystemic Encephalopathy

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Portosystemic Encephalopathy


Pathology

  • fibrosis blocks portal vein → portal hypertension
  • collaterals develop
    • umbilical
    • left renal
    • anal
  • venous drainage of gut bypass first pass metabolism of liver
  • ? ammonia

Precipitating factors

  • increased dietary proteins
  • GI haemorrhage (large protein meal)
  • Constipation
  • Infection
  • Fluid and electrolyte disturbance
    • diuretic therapy
    • paracentesis
  • Drugs
    • CNS depressants
  • TIPS
  • Surgery
  • Progressive liver damage
  • Development of Hepatocellular Carcinoma

History

Acute onset

  • precipitating factor
  • increasing drowsiness and confusion

Chronic onset

  • history from relative
  • mood disorder
  • personality change
  • intellectual deficit
  • reversal of normal sleep rhythm
  • nausea
  • vomiting
  • weakness
  • convulsions
  • coma

Examination

  • irritable
  • confused
  • disorientated
  • slow, slurred speech
  • hyperventilation
  • pyrexia
  • fetor hepaticus
  • asterixis
  • constructional apraxia
  • reduced mental function
    • trail time increased
    • serial sevens

Investigations

  • LFTs confirm presence of liver disease
  • EEG
    • alpha waves (8-13 Hz ) decreased in frequency to delta waves (1.5-3 Hz)
  • Visual evoked responses
  • Arterial ammonia (if available)

Management

Acutely

  • remove precipitants
  • purgatives / enemas
  • lactulose (30ml/day)
    • reduces colonic pH → reduced ammonia absorption
  • protein-free diet
  • antibiotics
    • neomycin
  • stop / reduce diuretic therapy
  • correct electrolyte imbalance
  • IV fluids
  • Treat infection
  • Flumazenil

Chronically

  • increase protein to level of tolerance
  • lactulose
  • avoid precipitants
    • narcotic drugs
    • over-diuresis

Prognosis

  • variable, dependent on underlying disease
 

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