Multifactorial non-cirrhotic hyperammonaemic encephalopathy

BMJ Case Rep. 2018 Mar 9:2018:bcr2017223245. doi: 10.1136/bcr-2017-223245.

Abstract

A 51-year-old female presented with acute confusion associated with a non-specific headache and lethargy. The patient's history included bipolar disorder on valproate and recent travel to northern Vietnam. The patient was subsequently found to have hyperammonaemia as well as a urinary tract infection and bacteraemia with Klebsiellapneumoniae The patient was presumed to have a multifactorial non-cirrhotic hyperammonaemic encephalopathy due to a combination of a urinary tract infection and bacteraemia with K. pneumoniae, a urease-producing bacteria, and also valproate use, a medication known to interfere with ammonia elimination. The patient's treatment included supportive care, ceasing valproate, empiric then rationalised antibiotics, N-acetylcysteine and L-carnitine. We present a case of non-cirrhotic hyperammonaemic encephalopathy and explain why it is multifactorial in origin.

Keywords: adult intensive care; neurology; unwanted effects / adverse reactions; urinary tract infections.

Publication types

  • Case Reports

MeSH terms

  • Acetylcysteine / administration & dosage
  • Acetylcysteine / therapeutic use
  • Aftercare
  • Anti-Bacterial Agents / administration & dosage
  • Anti-Bacterial Agents / therapeutic use
  • Bacteremia / diagnosis
  • Bacteremia / drug therapy
  • Bacteremia / microbiology
  • Carnitine / administration & dosage
  • Carnitine / therapeutic use
  • Female
  • Free Radical Scavengers / therapeutic use
  • Humans
  • Hyperammonemia / chemically induced
  • Hyperammonemia / diagnosis*
  • Klebsiella pneumoniae / isolation & purification
  • Magnetic Resonance Imaging / methods
  • Middle Aged
  • Neurotoxicity Syndromes / diagnosis*
  • Neurotoxicity Syndromes / diagnostic imaging
  • Treatment Outcome
  • Urinary Tract Infections / complications
  • Urinary Tract Infections / diagnosis*
  • Urinary Tract Infections / drug therapy
  • Urinary Tract Infections / microbiology
  • Valproic Acid / adverse effects*

Substances

  • Anti-Bacterial Agents
  • Free Radical Scavengers
  • Valproic Acid
  • Carnitine
  • Acetylcysteine