Incidence
Age
Sex
Geography
Aetiology	- a chronc neuropsychiatric syndrome secondary to chronic liver disease - due to portosystemic shunting - either spontaneous or iatrogenic (e.g. TIPS) - blood bypasses liver, metabolites pass directly to brain - may be due to ammonia (also alters CNS neurotransmitter balance), FFAs, mercaptans, false neurotransmitters e.g. octopamine, activation of GABA system - high plasma levels of aromatic aas, reduced branched-chain aas - ammonia is produced by breakdown of protein by intestinal bacteria
Presentation	Precipitating factors - high dietary protein - GI haemorrhage - constipation - infection, including spontaneous bacterial peritonitis - fluid and electrolyte disturbance (2º to diuretic therapy, paracentesis) - drugs (any CNS depressant) - portosystemic shunts, TIPS - any surgical procedure - progressive liver damage - development of HCC History - there is often a precipitating factor - disorder of personality, mood and intellect - may fluctuate - reversal of normal sleep rhythm - nausea, vomiting, weakness Examination - patient appears irritated and confused, with slurred speech - convulsions and coma occur later on - hyperventilation and pyrexia - foetor hepaticus - asterixis - coarse flapping tremor when hands are outstretched with wrists hyperextended - constructional apraxia (5-pointed star test) - decreased mental function (serial sevens test; trail-making test) - clinical diagnosis
Investigations	LFTs - chronic liver disease U+Es EEG - decrease in frequency of a-waves to d-waves Visual evoked responses Arterial blood ammonia - occasionally useful, but rarely available
Macro
Micro
Staging
Serum markers
Management	- aim is to restrict protein intake and sterilize the bowel Immediate - identify and remove any possible precipitating cause - purgation and enemas e.g. lactulose 10-30 mL tds, lactilol 30 g daily - protein-free diet, with adequate calories - may need to be given via NG tube - antibiotics e.g. neomycin 1g qds po, metronidazole 200 mg qds - stop or reduce diuretic therapy - correct any electrolyte imbalance - i.v. fluids as necessary - treat any infection - flumazenil can produce a transitory improvement Long term - increased protein in diet to limit of tolerance (20-50g daily) - lactulose 10-30 mL tds (prevent constipation) - avoid precipitating factors
Prognosis	- very poor prognosis when acute PSE seen in FHF (high mortality of underlying disease) - chronic PSE has the same prognosis as the underlying liver disease
Complications

Hepatobiliary medicine

Main Page