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

Weise, Sebastian; Syrbe, Steffen; Preuß, Matthias; Bertsche, Astrid; Merkenschlager, Andreas; Bernhard, Matthias K.

doi:10.1186/s40064-015-1057-9

Cited by 5 publications

(8 citation statements)

References 17 publications

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“…Se han descrito casos en los que la asociación de varios antiepilépticos como el TPM y el VPA induce hiperamoniemia con mayor frecuencia (8,(12)(13)(14). El TPM inhibe a la anhidrasa carbónica elevando los niveles de amonio y disminuyendo la síntesis de urea.…”

Section: Discussionunclassified

Estudio Multicéntrico Español: Niños Con Hiperamoniemia No Asociada a Errores Innatos Del Metabolismo

et al. 2017

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ResumenIntroducción: la encefalopatía hiperamoniémica inducida por causas diferentes a los errores innatos del metabolismo es una complicación relativamente infrecuente, pero muy grave. Objetivos: conocer las características de un episodio de hiperamoniemia secundaria, tratar de discernir las causas desencadenantes, cómo se llega al diagnóstico del episodio y cómo se desarrolla la actuación terapéutica. Métodos: es un estudio multicéntrico retrospectivo de casos pediátricos con hiperamoniemia no relacionada con errores innatos del metabolismo, llevado a cabo en hospitales españoles. Resultados: fueron seleccionados 19 pacientes y en un 47% el episodio de hiperamoniemia apareció en menores de un año, que fueron diagnosticados mayoritariamente con dos o más síntomas. La clínica más frecuente fue la de alteración del nivel de conciencia tipo intoxicación, seguida de las crisis convulsivas, sumando en ambas hasta 14 pacientes con alguna de ellas o las dos. Doce de los 19 pacientes utilizaban más de dos fármacos antiepilépticos de forma habitual. Todos los niños recibieron tratamiento con restricción proteica (n: 10), quelantes (n: 10) y/o ácido carglúmico (n: 12) para el tratamiento de la hiperamoniemia. Conclusiones: este estudio sugiere que la hiperamoniemia secundaria puede estar infradiagnosticada y solo se detecta cuando aparece una sintomatología grave. Parecen ser pacientes de riesgo aquellos que reciben fármacos antiepilépticos o aquellos críticos con una alimentación restringida o un metabolismo elevado. La respuesta con tratamiento específi co es adecuada pero debe ser precoz para evitar las secuelas neurológicas de esta entidad. AbstractIntroduction: The hyperammonemic encephalopathy induced by causes different from inborn errors of metabolism is a relatively uncommon but severe complication. Objectives: To study the characteristics of a secondary hyperammonemia episode to discern the triggering causes to get to the diagnosis, and the development in the therapeutic intervention. Methods: A multicenter retrospective study of children with hyperammonemia unrelated to inborn errors of metabolism, conducted in Spanish hospitals. Results: Nineteen patients were selected; hyperammonemia developed in infants under one year old in 47% of them, being diagnosed mostly with two or more symptoms. The most common clinical fi nding was an altered consciousness level similar to that of intoxication symptoms, followed by seizures. These clinical symptoms were present in 14 patients, with one of them or both. Twelve of the 19 patients were in treatment with more than two antiepileptic drugs routinely. All children were treated with protein restriction (n: 10), scavengers (n: 10) and/or carglumic acid (n: 12) for the treatment of hyperammonemia. Conclusions: This study suggests that secondary hyperammonemia could be underdiagnosed because it is only detected when severe symptoms appear. Risk seems to be higher in those patients receiving antiepileptic drugs or those critically ill with a restricted diet or incremented met...

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Section: Discussionunclassified

Estudio Multicéntrico Español: Niños Con Hiperamoniemia No Asociada a Errores Innatos Del Metabolismo

et al. 2017

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“…Regarding the external validity, correlation coefficients were significant and appropriate all sub-domains of QOLIE-31 with SF-36 as well. Therefore, future researchers should not limit themselves to the western scales,7,21 but should also consider specific cultural factors.…”

Section: Discussionmentioning

confidence: 99%

“…The aged samples replied to the 31 items of QOLIE-31. The QOLIE-31 questionnaire mainly consists of a 31-item disability/symptom scale regarding epilepsy that was investigated by authors and literature reviews 2,4,7,12,13,20,22,24,27,29. It was developed in three version i.e.…”

Section: Methodsmentioning

confidence: 99%

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Psychometric Properties of the Persian Version of the Quality of Life in Epilepsy Inventory in the Later Life

et al. 2016

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Background and PurposeThe reports indicate on the incidence of seizure disorder about 1.5 per cent of the normal elderly population. The Quality of Life in Epilepsy Inventory (QOLIE-31) has been pervasive simple tool to screen seizure in the busy neurophysiological settings and monitoring. It was constructed as self-administered tool in two formats, 89 and 31-items. To the reliability and validity of the QOLIE-31 across older adults in the southwest Iran and discuss its role in the detection of health-related quality of elderly patients with epilepsy.MethodsAbout 73 older adults (mean age = 66.3 ± 1.71) were sampled from the eight hospitals and caring centres. They replied to the QOLIE-31. External and criterion validity was calculated by correlation to the SF-36 questionnaire, to check and validate the epilepsy specific dimensions. The QOLIE-31 includes seven subscales: overall quality of life, seizure worry, emotional well-being, energy/fatigue, cognitive, medication effects, and social function.ResultsThere was significant difference within sample groups regarding main variables (p < 0.05). The coefficients of Cronbach’s alpha (α= 0.76), convergent validity (0.81), divergent validity (−0.21), external validity with overall score of SF-36 (0.87), and criterion validity (0.78) were estimated, which were significant at p < 0.01. The exploratory factor analysis demonstrated that the QOLIE-31 is organized into six factors, which clarifies 92 per cent of the scale’s variance. Second-order confirmatory factor analysis pointed out that the factor is well matched up onto a principal factor. Consequently, the 6-factors model was well appropriate for the data by the fit index techniques for adjusting the scale (AGFI = 0.94, GFI = 0.96, RMSEA = 0.003, IFI = 0.90, NFI = 0.95, CFI = 0.95).ConclusionsThe results pointed to the well-adjusted reliability and psychometric properties of the QOLIE-31 and its usefulness for the relevant studies as well.

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“…However, although VPA is highly effective in controlling seizure in children, it also has several adverse effects such as fatigue, gastrointestinal disturbances, weight gain, muscle tremors, hair loss, thrombocytopenia, and an increase in hepatic enzymes. 1,2 Other than that, VPA also increases blood ammonia concentrations (hyperammonemia) in adults [3][4][5][6][7] and children [8][9][10][11][12][13][14][15] . The actual increases in blood ammonia concentrations depend on the dose of VPA 4,8 and the use of long-term therapy with VPA 5 .…”

Section: Introductionmentioning

confidence: 99%

High Blood Ammonia Levels Associated with Long-term Valproic Acids Therapy in Epileptic Children

Sidiartha

Suwarba

Wati

et al. 2020

Mol Cell Biomed Sci

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Background: Valproic acid is an effective drug for controlling seizure in children with epilepsy and it is usually used for treatment as long as two years or more. Blood ammonia level often increased in epileptic children who were treated with long-term valproic acid. The study was conducted to determine the relationship between blood ammonia level with valproic acid therapy in epileptic children.Materials and Methods: This is an observational study with cross-sectional approach. The subjects were 64 children with epilepsy, average age of 6.2 years old. Subjects were 33 boys and 31 girls. Blood ammonia level was examined using enzymatic glutamate dehydrogenase. Subjects were divided into 2 therapeutic groups based on the duration, doses and combination therapy of valproic acid. Subjects were recruited from Pediatric Neurology Clinic, Sanglah General Hospital, Bali, Indonesia, from May to December 2017. Comparison of blood ammonia level between groups were analyzed using an Independent t-test with significances if the p<0.05. Results: A significant difference of blood ammonia level was found between subjects who were treated with valproic acid less than 2 years and more than 2 years (45.7±16.4 mmol/L vs. 70.9±43.6 mmol/L; p=0.032). However, significant difference was not found between the groups according to the doses and combination therapy (p=0.450 and p=0.647, respectively).Conclusion: Blood ammonia level was significantly higher in epileptic children who used long-term valproic acid, hence it was recommended to check the blood ammonia level routinely.Keywords: ammonia, epilepsy, valproic, children

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

Cited by 5 publications

References 17 publications

Estudio Multicéntrico Español: Niños Con Hiperamoniemia No Asociada a Errores Innatos Del Metabolismo

Estudio Multicéntrico Español: Niños Con Hiperamoniemia No Asociada a Errores Innatos Del Metabolismo

Psychometric Properties of the Persian Version of the Quality of Life in Epilepsy Inventory in the Later Life

High Blood Ammonia Levels Associated with Long-term Valproic Acids Therapy in Epileptic Children

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