Valproate-induced Hyperammonemic Encephalopathy Caused by Free Carnitine Deficiency in a Patient with Bipolar Disorder: A Case Report
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Valproate is commonly used in various psychiatric and neurologic settings and is usually well tolerated. We present the case of a 26-year-old man who developed encephalopathy after valproate treatment. Ten days after initiating valproate therapy, he presented with a sudden decreased level of consciousness, disorientation, seizures, and eventually coma. Notably, the serum ammonia level was elevated to 392 μmol/l with no hepatic dysfunction on laboratory tests. He did not have a urea cycle-associated enzyme deficiency on tandem mass spectrometry. However, the free carnitine level was low (5 μmol/l), and this was suspected of being caused by the valproate treatment. Due to the possibility of valproate-induced hyperammonemic encephalopathy, the valproate was discontinued, and the patient was treated with L-carnitine 50 mg/kg per day. He recovered completely within 3 days of L-carnitine treatment, and the free carnitine level was within normal limits (53.5 μmol/l) 5 months thereafter. Clinicians should consider valproate-induced hyperammonemic encephalopathy in patients who present with any mental changes or atypical psychiatric symptoms while on valproate.
Abstract
Valproate is commonly used in various psychiatric and neurologic settings and is usually well tolerated. We present the case of a 26-year-old man who developed encephalopathy after valproate treatment. Ten days after initiating valproate therapy, he presented with a sudden decreased level of consciousness, disorientation, seizures, and eventually coma. Notably, the serum ammonia level was elevated to 392 μmol/l with no hepatic dysfunction on laboratory tests. He did not have a urea cycle-associated enzyme deficiency on tandem mass spectrometry. However, the free carnitine level was low (5 μmol/l), and this was suspected of being caused by the valproate treatment. Due to the possibility of valproate-induced hyperammonemic encephalopathy, the valproate was discontinued, and the patient was treated with L-carnitine 50 mg/kg per day. He recovered completely within 3 days of L-carnitine treatment, and the free carnitine level was within normal limits (53.5 μmol/l) 5 months thereafter. Clinicians should consider valproate-induced hyperammonemic encephalopathy in patients who present with any mental changes or atypical psychiatric symptoms while on valproate.
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