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Related Concept Videos

Hepatic Encephalopathy01:29

Hepatic Encephalopathy

DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic shunting—including...
Urea Cycle01:23

Urea Cycle

The urea cycle describes how liver cells convert ammonia to urea. Ammonia is a toxic waste product of protein catabolism. Land animals must convert ammonia into the less toxic urea which can be safely eliminated by the kidneys through urine. Marine animals excrete ammonia directly, and the surrounding water dilutes the ammonia to safe levels.
Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Overview of Protein Metabolism01:21

Overview of Protein Metabolism

Proteins are broken down into amino acids during digestion. Unlike fats and carbohydrates, which are stored for later use, proteins are not. Instead, amino acids are either used to produce ATP through oxidation or contribute to the creation of new proteins for the growth and repair of the body. Any surplus amino acids from the diet are converted into glucose or triglycerides rather than excreted.
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...

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Related Experiment Video

Updated: Jul 9, 2026

Quantification of Coenzyme A in Cells and Tissues
08:51

Quantification of Coenzyme A in Cells and Tissues

Published on: September 27, 2019

Hyperammonemic encephalopathy caused by carnitine deficiency.

Berkeley N Limketkai1, Stephen D Zucker

  • 1Department of Internal Medicine, University of Cincinnati, Cincinnati, OH 45267-0595, USA. berkeley.limketkai@gmail.com

Journal of General Internal Medicine
|December 15, 2007
PubMed
Summary

Carnitine deficiency can cause severe hyperammonemia and encephalopathy. Supplementation with carnitine effectively treated a patient with refractory hyperammonemia, highlighting its crucial role in fatty acid metabolism.

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Area of Science:

  • Biochemistry
  • Metabolic Disorders
  • Neurology

Background:

  • Carnitine is vital for fatty acid metabolism and energy production.
  • Carnitine deficiency can lead to impaired fatty acid oxidation.
  • Rarely, carnitine deficiency may manifest as hyperammonemia and encephalopathy.

Observation:

  • A 35-year-old woman presented with acute mental status changes, asterixis, and muscle weakness.
  • Her ammonia level was significantly elevated (276 microg/dL).
  • Standard ammonia-reducing treatments were ineffective.

Findings:

  • The patient was severely malnourished with critically low carnitine levels.
  • Carnitine supplementation led to normalization of ammonia levels.
  • The patient's neurological status and mental state returned to baseline.

Implications:

  • This case underscores the importance of considering carnitine deficiency in unexplained hyperammonemia.
  • Carnitine supplementation offers a potentially life-saving treatment for such cases.
  • Early diagnosis and intervention in carnitine deficiency can prevent severe neurological complications.