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a University
Hospital of Wales: Department of Gastroenterology, b Department of Surgery, c Department of Neurology, d Department
of Paediatrics
Correspondence to: Dr N D Hawkes, Department of Gastroenterology, University Hospital of Wales, Heath Park, Cardiff CF14 4XN, UK neilhawkes{at}doctors.org.uk
Submitted 21 August
2000;
Accepted 15 February 2001
The clinical syndrome of encephalopathy is most often
encountered in the context of decompensated liver disease and the
diagnosis is usually clear cut. Non-hepatic causes of encephalopathy
are rarer and tend to present to a wide range of medical specialties with variable and episodic symptoms. Delay can result in the
development of potentially life threatening complications, such as
seizures and coma.
Early recognition is vital. A history of similar episodes or clinical
risk factors and early assessment of blood ammonia levels help
establish the diagnosis. In addition to adequate supportive care,
investigation of the underlying cause of the hyperammonaemia is
essential and its reversal, where possible, will often result in
complete recovery. Detection of an unborn error of metabolism should
lead to the initiation of appropriate maintenance therapy and genetic counselling.
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