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CSF Ornithine

Body Fluids

Cerebrospinal fluid ornithineCSF amino acid: ornithineCSF Orn

Review status

Currently under review

Pending specialist review and validation.

What it shows

The CSF Ornithine test measures the amount of the amino acid ornithine in your cerebrospinal fluid, the clear liquid that bathes your brain and spinal cord. Ornithine is part of the urea cycle, the pathway your body uses to process nitrogen and remove ammonia.

Measuring ornithine directly in CSF helps your care team understand how the brain and nervous system are handling amino acids. This test is often ordered together with other CSF amino acids and compared with blood and urine testing when there is concern for a metabolic condition or unexplained neurologic symptoms.

Why it matters

Abnormal CSF ornithine levels can be a clue to inherited metabolic disorders that affect nitrogen handling or amino acid metabolism, such as urea cycle defects or ornithine aminotransferase deficiency. Changes may also reflect liver dysfunction, nutritional issues, or problems with transport of amino acids between blood and brain.

Your clinician may order this test if you have unexplained seizures, developmental concerns, altered mental status, or vision problems suggestive of certain rare conditions. Results can guide next steps, such as targeted genetic testing, nutrition changes, or specific treatments aimed at lowering ammonia and stabilizing brain function.

Understanding your results

Your result is interpreted alongside your symptoms, examination, imaging, and other laboratory tests such as plasma and urine amino acids, ammonia, liver panel, and the broader CSF amino acid profile. Small deviations may be due to sample handling or individual variation, so a repeat sample or parallel blood testing may be recommended if findings are unexpected.

If results suggest an inherited metabolic condition, your clinician may refer you to a metabolic specialist for confirmatory testing and counseling. Management can include diet adjustments, medications that support nitrogen removal, careful monitoring during illness, and follow-up testing to track stability over time.

Reference ranges

29 umol/L
All sexes
0 days – 150 years

Reference intervals vary by laboratory, analyzer, methodology, population, and units. The ranges shown here are for education only. Always interpret your results against the reference interval printed on your own lab report.

Factors that could impact CSF Ornithine

  • Sample handling and timing

    CSF should be collected cleanly, kept cold, and processed or frozen promptly. Delays, warm temperatures, or prolonged storage can alter amino acid concentrations and lead to misleading results.

  • Blood contamination of CSF

    A traumatic tap or bleeding into the CSF can introduce blood amino acids, distorting the true CSF ornithine level. Laboratories often assess for red blood cells to judge sample integrity.

  • Diet and catabolic state

    High protein intake, fasting, fever, or recent illness can shift amino acid patterns. Your clinician will interpret CSF ornithine in the context of your recent diet and overall health.

  • Medications and supplements

    Agents that affect nitrogen metabolism, such as valproate, or supplements like arginine or ornithine, can influence results. Provide a full list of medicines and over-the-counter products.

  • Liver and kidney function

    Liver disease can impair ammonia detoxification and alter amino acids, while kidney dysfunction can change clearance. Related blood tests help clarify whether results reflect systemic issues.

  • Neurologic and inflammatory conditions

    Meningitis, encephalitis, or disruption of the blood-brain barrier can change CSF amino acid levels. Clinical context and other CSF tests are needed for accurate interpretation.

2026

References

  1. McGill University Health Centre. (2006, September 13). CSF Ornithine (Task CD 693419). Laboratory reference ranges.
  2. Häberle, J., Burlina, A., Chakrapani, A., Dixon, M., Karall, D., Lindner, M., Martins, E., et al. (2019). Suggested guidelines for the diagnosis and management of urea cycle disorders. Journal of Inherited Metabolic Disease, 42(6), 1192–1230.
  3. Vockley, J., Burton, B. K., Berry, G. T., Longo, N., Phillips, J., Sanchez-Valle, A., Chapman, K. A., & Summar, M. L. (2014). Acute management of hyperammonemia in urea cycle disorders: A consensus guideline of the Urea Cycle Disorders Consortium. Molecular Genetics and Metabolism, 110(4), 446–454.