title: Carnitine deficiency presenting with encephalopathy and hyperammonemia in a patient receiving chronic enteral tube feeding: a case report.
abstract: BACKGROUND
  Carnitine is an essential cofactor in mitochondrial fatty acid oxidation. Carnitine deficiency results in accumulation of non-oxidized fatty acyl-coenzyme A molecules, and this inhibits intra-mitochondrial degradation of ammonia. Hyperammonemia may lead to encephalopathy. This scenario has been previously reported.
  
  
  METHODS
  We report the case of a 47-year-old Caucasian man who had sustained a remote motor vehicle accident injury and relied on long-term tube feeding with a commercial product that wascarnitine-free. He was also on phenytoin therapy for control of his chronic seizures. He developed significant acute psychological and behavioral changes superimposed on his chronic neurological impairment. His ammonia level was found to be elevated at 75 to 100μmol/L (normal <35μmol/L). Phenytoin was found to be at a supra-therapeutic level of 143μmol/L (therapeutic range 40-80μmol/L). After adjusting the dose of phenytoin, other pharmacological and hepatic causes of his hyperammonemia and subacute encephalopathy were excluded. His carnitine levels were found to be low. After initiating carnitine supplementation at 500mg twice daily, the patient's mental status improved, and his ammonia level improved to 53-60μmol/L.
  
  
  CONCLUSIONS
  This case illustrates the importance of avoiding carnitine deficiency and anti-convulsant toxicity in tube-fed patients encountered in hospital wards and nursing homes. These patients should have their carnitine levels assessed regularly, and supplementation should be provided as necessary. Manufacturers of enteral feeds and formulas should consider adding carnitine to their product lines.
authors: Department of Medicine, University of British Columbia, 10th Floor, 2775 Laurel Street, Vancouver, BC V5Z 1M9, Canada. Sandra.Sirrs@vch.ca.
authors: Peter
authors: P
authors: Ling
authors: Douglas J
authors: DJ
authors: Lee
authors: Eric M
authors: EM
authors: Yoshida
authors: Sandra
authors: S
authors: Sirrs