Pronounced reversible hyperammonemic encephalopathy associated with combined valproate鈥搕opiramate therapy in a 7-year-old girl

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• 作者：Sebastian Weise ; Steffen Syrbe ; Matthias Preuss ; Astrid Bertsche…
• 关键词：Topiramate ; Valproate ; Encephalopathy ; Glutamine ; Hyperammonemia
• 刊名：SpringerPlus
• 出版年：2015
• 出版时间：December 2015
• 年：2015
• 卷：4
• 期：1
• 全文大小：716KB
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• 作者单位：Sebastian Weise (1)
  Steffen Syrbe (1)
  Matthias Preuss (1) (2)
  Astrid Bertsche (1)
  Andreas Merkenschlager (1)
  Matthias K Bernhard (1)
  
  1. Department of Women and Child Health, University Hospital for Children and Adolescents, Liebigstr. 20a, 04103, Leipzig, Germany
  2. Department of Neurosurgery, Pediatric Neurosurgery, University Hospital Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany
  
• 刊物类别：Science, general;
• 刊物主题：Science, general;
• 出版者：Springer International Publishing
• ISSN：2193-1801

文摘

Valproate is one of the most frequently used anticonvulsive drugs in children and adults. Valproate is a generally well tolerated medication. However, encephalopathy with or without hyperammonemia is one of its rare adverse events. We present a 7-year-old girl who suffered from epilepsy with generalized tonic鈥揷lonic seizures and absence epilepsy. She was initially treated with topiramate. Methylprednisolone pulse therapy and long-term therapy with valproate were initiated due to an increase of seizure frequency. At day 5 of therapy, a further increase of seizure frequency was observed followed by lethargy and somnolence. Liver enzymes remained within normal range, but ammonia serum levels increased to a maximum of 544 mmol/l. Discontinuing valproate and starting potassium-benzoate and sodium-phenylbutyrate improved the clinical condition and ammonia serum levels. Haemodialysis was not required. Cranial magnetic resonance imaging ruled out brain edema. The patient was further on successfully treated with a combination of both, topiramate and levetiracetam. Seizures did not recur and development was normal until now (3 years later). To the best of our knowledge, we observed the highest ammonia serum levels ever reported in valproate-induced hyperammonemia with a complete remission of the subsequent encephalopathy. Topiramate might increase the risk of valproate-induced encephalopathy by carbonic anhydrase inhibition. Keywords Topiramate Valproate Encephalopathy Glutamine Hyperammonemia