Valproate-induced hyperammonemia - uncovering an underlying inherited metabolic disorder: a case report

Journal of Medical Case Reports
Shaine MehtaRobin Lachmann

Abstract

Sodium 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. 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. Ornithine transcarbamylase deficiency is the most common of the urea cycle disorders. This case highlights both the importance of checking ammonia ...Continue Reading

References

May 23, 1998·Journal of Neurology, Neurosurgery, and Psychiatry·M OechsnerA Kohlschütter
Aug 1, 1998·Journal of Inherited Metabolic Disease·M TuchmanN M Allewell
Aug 12, 2009·Hepatology International·Ophir D KleinSeymour Packman
Jun 2, 2011·Case Reports in Medicine·Surjit TarafdarMatt Doogue
May 9, 2012·Journal of Korean Medical Science·Dae Eun ChoiKi Ryang Na

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Citations

Aug 14, 2020·Curēus·Faraaz ZafarAhsen U Ahmed
Jul 29, 2021·The Mental Health Clinician·Tressa McMorrisAmanda Bernardini

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