J Korean Child Neurol Soc Search

CLOSE


Journal of the Korean Child Neurology Society 2003;11(2):256-261.
Published online November 30, 2003.
The Clinical Analysis of Children Who Increase Urine gamma-hydroxybutyric Acid with Seizure Disorder.
Eung Seok Kim, Chang Bum Koh, Eun Joo Bae, Hong Jin Lee, Won Il Park, Kyoung Ja Lee
Department of Pediatrics, College of Medicine, Hallym University, Chuncheon, Korea. beetlbum@nownuri.net
Abstract
PURPOSE
This study was performed to analyse urine gamma-hydroxybutyric acid(GHB) in children with seizures, and to investigate the pattern of seizures and neurologic abnormalities in children related with gamma-hydroxybutyric aciduria. METHODS: We reviewed retrospectively medical records of children who admitted to our hospital with seizures between August 1. 2001 and February 28. 2003. We compared urine GHB levels with controls, and also analyzed the clinical features of patients who showed increased urine GHB. RESULTS: The mean urine GHB was 1.7+/-1.6 mmol/mol cr in febrile seizures, 1.8+/-2.5 mmol/mol cr in non-febrile seizures, and 1.8+/-2.0 mmol/mol cr in controls. Compared with control group, there was no significant difference in urine GHB levels(P>0.05). In 8 of 64 children with seizures, GHB levels increased above 2 standard deviation of normal controls. The types of seizure in children who showed increased urine GHB were generalized tonic clonic seizure in 3 patients, complex partial seizure in 2 patients, febrile seizure in 2 patients, and benign Rolandic epilepsy in 1 patient. 3 patients showed neurologic abnormalities, 4 patients showed electroencephalographic abnormalities, and 2 patients of 6 patients who performed brain imaging study showed brain imaging abnormalities. CONCLUSION: Children with gamma-hydroxybutyric aciduria should be suspected succinic semialdehyde dehydrogenase deficiency as a cause of underlying disease.
Key Words: gamma-hydroxybutyric acid, Febrile seizure, Non-febrile seizure, gamma-hydroxybutyric aciduria
TOOLS
Share :
Facebook Twitter Linked In Google+ Line it
METRICS Graph View
  • 51 View
  • 0 Download
Related articles in Ann Child Neurol

The Clinical Features of Children with Developmental Language Disorder.2001 May;9(1)



ABOUT
ARTICLE CATEGORY

Browse all articles >

BROWSE ARTICLES
EDITORIAL POLICY
AUTHOR INFORMATION
Editorial Office
50-1, Yonsei-ro, Seodaemun-gu, Seoul 03722, Korea
Tel: +82-2-2019-3350    Fax: +82-2-2019-4881    E-mail: editor@annchildneurol.org                

Copyright © 2019 by Korean Child Neurology Society. All rights reserved.

Developed in M2community

Close layer
prev next