Hyperammonemic encephalopathy due to essential amino acid hyperalimentation

Area of Science:

• Clinical Nutrition
• Neurology
• Metabolic Disorders

Background:

• Parenteral nutrition often utilizes essential amino acids for patient support.
• Hyperammonemia is a known complication of certain metabolic disturbances.
• Encephalopathy presents with altered mental status and neurological deficits.

Observation:

• A case of hyperammonemic encephalopathy occurred in an adult patient receiving essential amino acids intravenously.
• Clinical presentation included elevated cerebrospinal fluid glutamine and serum ammonia.
• No other drug or metabolic etiology for the encephalopathy was identified.

Findings:

• The patient's encephalopathy and hyperammonemia resolved after discontinuing the essential amino acid infusion.
• This suggests a direct link between the amino acid formulation and the observed toxicity.
• Elevated ammonia levels indicate impaired urea cycle function or increased ammonia production.

Implications:

• Serum ammonia monitoring is recommended for patients receiving essential amino acid infusions.
• Clinicians should consider alternative nutritional support if ammonia levels remain high or toxicity develops.
• This case highlights the importance of vigilant monitoring in parenteral nutrition therapy.