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01-06-2010 | Original Paper

Generalized seizures aggravated by levetiracetam in an adult patient with phenylketonuria

Authors: Nese Dericioglu, Serap Saygi

Published in: Metabolic Brain Disease | Issue 2/2010

Abstract

Phenylketonuria (PKU) is one of the most common inherited metabolic disorders, which is characterized by excessive accumulation of phenylalanine (Phe) in tissues. Generalized seizures occur in 25% of the patients. Little is known about seizures and their treatment in adult PKU patients, and information with newer antiepileptic drugs is lacking. Here we report an adult patient who developed generalized seizures later in life, despite strict dietary control, and whose seizures were aggravated by levetiracetam (LEV). Convulsions ceased after discontinuation of LEV and the patient has been seizure free on topiramate 125 mg/day.

Brenton DP, Pietz J (2000) Adult care in phenylketonuria and hyperphenylalaninemia: the relevance of neurological abnormalities. Eur J Pediatr 159(Suppl 2):S114–S120CrossRefPubMed

Gross PT, Berlow S, Schuett VET, Fariello RG (1981) EEG in phenylketonuria. Attempt to establish clinical importance of EEG changes. Arch Neurol 38(2):122–126PubMed

Hanley B (2004) Adult Phenylketonuria. Am J Med 117:590–595CrossRefPubMed

Lin CM, Thajeb P (2007) Valproic acid aggravates epilepsy due to MELAS in a patient with an A3243G mutation of mitochondrial DNA. Metab Brain Dis 22(1):105–109CrossRefPubMed

Lynch BA, Lambeng N, Nocka K, Kensel-Hammes P, Bajjalieh SM, Matagne A, Fuks B (2004) The synaptic vesicle protein SV2A is the binding site for the antiepileptic drug levetiracetam. Proc Natl Acad Sci USA 101(26):9861–9866CrossRefPubMed

Martynyuk AE, Ucar DA, Yang DD, Norman WM, Carney PR, Dennis DM, Laipis PJ (2007) Epilepsy in phenylketonuria: a complex dependence on serum phenylalanine levels. Epilepsia 48(6):1143–1150CrossRefPubMed

Szücs A, Clemens Z, Jakus R, Rasonyi G, Fabo D, Hollo A, Barcs G, Kelemen A, Janszky J (2008) The risk of paradoxical levetiracetam effect is increased in mentally retarded patients. Epilepsia 49(7):1174–1179CrossRefPubMed

Thompson AJ, Tillotson S, Smith I, Kendall B, More SG, Brenton DP (1993) Brain MRI changes in phenylketonuria. Association with dietary status. Brain 116:811–821CrossRefPubMed

Villasana D, Butler IJ, Williams JC, Roongata SM (1989) Neurological deterioration in adult phenylketonuria. J Inherit Metab Dis 12:451–457CrossRefPubMed

Walter JH, White F, Wraith JE, Jenkins JP, Wilson BP (1997) Complete reversal of moderate/severe brain MRI abnormalities in a patient with classical phenylketonuria. J Inherit Metab Dis 20:367–369CrossRefPubMed

Williams RA, Mamotte CDS, Burnett JR (2008) Phenylketonuria: an inborn error of phenylalanine metabolism. Clin Biochem Rev 29:31–41PubMed

Zhang SX, Yu WM, Wang GZ (1995) An electroencephalogram analysis of 94 phenylketonuria patients. J Clin Electroencephalogr 4(3):139–141

Zhongshu Z, Weiming Y, Yukio F, Cheng L, Ning Z, Zhixing W (2001) Clinical analysis of West syndrome associated with phenylketonuria. Brain Dev 23:552–557CrossRefPubMed

Title: Generalized seizures aggravated by levetiracetam in an adult patient with phenylketonuria
Authors: Nese Dericioglu
Serap Saygi
Publication date: 01-06-2010
Publisher: Springer US
Published in: Metabolic Brain Disease / Issue 2/2010
Print ISSN: 0885-7490
Electronic ISSN: 1573-7365
DOI: https://doi.org/10.1007/s11011-010-9197-7

At a glance: The STEP trials

Springer Medicine

Generalized seizures aggravated by levetiracetam in an adult patient with phenylketonuria

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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