ArticleJournal of medical case reports2018
Valproate-induced hyperammonemia - uncovering an underlying inherited metabolic disorder: a case report.
Article in Journal of medical case reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 22 citations in OpenAlex.
- Emerging neurological and cognitive symptoms in patients with late-onset ornithine transcarbamylase deficiency: a narrative review.Metabolic brain disease · 2026Review
- Article
- Fatal ornithine transcarbamylase deficiency presentation postresection of fourth ventricle ependymoma: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Article
- Amino Acid Metabolism and Immune Dysfunction in Urea Cycle Disorders: T and B Cell Perspectives.Journal of inherited metabolic disease · 2025Article
- Unusual case of sodium valproate-induced hyperammonaemia encephalopathy.BMJ case reports · 2023Article
- Valproate overdose leading to hyperammonaemic encephalopathy.BMJ case reports · 2023Article
- Hyperammonemia in patients receiving valproic acid in the hospital setting: A retrospective review.The mental health clinician · 2021Article
- Status epilepticus secondary to hyperammonaemia: a late presentation of an undiagnosed urea cycle defect.BMJ case reports · 2021Article
- Comparative study on the efficacy and safety of low-dose sodium valproateTranslational pediatrics · 2020Article
- Article
- The Story of Ammonia in Liver Disease: An Unraveling Continuum.Journal of clinical and experimental hepatologyReview
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSodium valproate is a commonly used anticonvulsant. It is widely recognized that valproate can cause hyperammonemia, particularly in people with underlying liver disease. Patients with urea cycle disorders are genetically predisposed to this adverse event and can develop severe hyperammonemia if given valproate. This can occur even if liver functions tests and plasma concentration of valproate are normal, highlighting the importance of checking ammonia levels in any patient presenting with encephalopathy. Specific treatment for hyperammonemia must be implemented promptly. CASE PRESENTATION: A 22-year-old white British man with a history of epilepsy post head trauma presented with subacute encephalopathy 4 weeks after the introduction of sodium valproate. His ammonia levels were not checked until 48 hours into his presentation and were found to be elevated. He initially responded to treatment of his hyperammonemia and the raised levels were attributed to sodium valproate. However, as his ammonia levels continued to rise, further investigation led to a diagnosis of ornithine transcarbamylase deficiency.
conclusionsOrnithine transcarbamylase deficiency is the most common of the urea cycle disorders. This case highlights both the importance of checking ammonia levels early and considering the diagnosis of this X-linked disorder in patients with raised ammonia, as these have implications both for the patient's acute and further management, and for family screening.
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