Valproate-induced Hyperammonemic Encephalopathy

Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that can occur in people with normal liver-associated enzyme levels, and despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl suffering from absence seizures, who co...

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Main Authors: Hsiao-Feng Chou, Rei-Cheng Yang, Cheng Ying Chen, Yuh-Jyh Jong
Format: Article
Language:English
Published: Elsevier 2008-10-01
Series:Pediatrics and Neonatology
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S1875957209600103
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author Hsiao-Feng Chou
Rei-Cheng Yang
Cheng Ying Chen
Yuh-Jyh Jong
author_facet Hsiao-Feng Chou
Rei-Cheng Yang
Cheng Ying Chen
Yuh-Jyh Jong
author_sort Hsiao-Feng Chou
collection DOAJ
description Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that can occur in people with normal liver-associated enzyme levels, and despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl suffering from absence seizures, who complained of progressive dizziness and general malaise several days after restarting valproate. She developed vomiting and decreased consciousness after 3 weeks of valproate use. She had a serum ammonia level five times higher than the upper normal limit, normal liver-associated enzymes, and a supra-therapeutic valproate level. Electroencephalography (EEG) showed continuous generalized slowing. Tandem mass spectrometry analysis revealed carnitine deficiency. Her consciousness improved after emergent hemodialysis. Her ammonia level and EEG also became normal. Possible mechanisms, risk factors and treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.
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spelling doaj.art-c778b34ed40c43e49a9a8d20f0b1a0092022-12-21T19:05:51ZengElsevierPediatrics and Neonatology1875-95722008-10-0149520120410.1016/S1875-9572(09)60010-3Valproate-induced Hyperammonemic EncephalopathyHsiao-Feng Chou0Rei-Cheng Yang1Cheng Ying Chen2Yuh-Jyh Jong3Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanDepartment of Nephrology, Tian-Sheng Memorial Hospital, Pingtung, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanValproate-induced hyperammonemic encephalopathy is an unusual but serious complication that can occur in people with normal liver-associated enzyme levels, and despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl suffering from absence seizures, who complained of progressive dizziness and general malaise several days after restarting valproate. She developed vomiting and decreased consciousness after 3 weeks of valproate use. She had a serum ammonia level five times higher than the upper normal limit, normal liver-associated enzymes, and a supra-therapeutic valproate level. Electroencephalography (EEG) showed continuous generalized slowing. Tandem mass spectrometry analysis revealed carnitine deficiency. Her consciousness improved after emergent hemodialysis. Her ammonia level and EEG also became normal. Possible mechanisms, risk factors and treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.http://www.sciencedirect.com/science/article/pii/S1875957209600103encephalopathyhyperammonemiavalproate
spellingShingle Hsiao-Feng Chou
Rei-Cheng Yang
Cheng Ying Chen
Yuh-Jyh Jong
Valproate-induced Hyperammonemic Encephalopathy
Pediatrics and Neonatology
encephalopathy
hyperammonemia
valproate
title Valproate-induced Hyperammonemic Encephalopathy
title_full Valproate-induced Hyperammonemic Encephalopathy
title_fullStr Valproate-induced Hyperammonemic Encephalopathy
title_full_unstemmed Valproate-induced Hyperammonemic Encephalopathy
title_short Valproate-induced Hyperammonemic Encephalopathy
title_sort valproate induced hyperammonemic encephalopathy
topic encephalopathy
hyperammonemia
valproate
url http://www.sciencedirect.com/science/article/pii/S1875957209600103
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AT reichengyang valproateinducedhyperammonemicencephalopathy
AT chengyingchen valproateinducedhyperammonemicencephalopathy
AT yuhjyhjong valproateinducedhyperammonemicencephalopathy