Myoclonic Epilepsy Beginning in Infancy or Early Childhood Medication

Author: Michael C Kruer, MD; Chief Editor: Amy Kao, MD more...

Updated: Dec 8, 2011

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Medication Summary
Antiepileptic Drugs
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References

Medication Summary

The long-term goals of pharmacotherapy are to reduce morbidity and prevent complications.

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Class Summary

These agents prevent seizure recurrence and terminate clinical and electrographic seizure activity. Patients with the benign form of myoclonic epilepsy often respond very well to first-line AEDs.

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Valproic acid (Depakote, Depakene, Depacon, Stavzor)

Valproic acid is chemically unrelated to other drugs that treat seizure disorders. Although its mechanism of action not established, its activity may be related to increased brain levels of gamma-aminobutyric acid (GABA), or enhanced GABA action. Valproate also may potentiate postsynaptic GABA responses, affect potassium channels, or have direct membrane-stabilizing effect.

The use of valproic acid in young children (younger than 2 y) is associated with an increased risk of hepatotoxicity. Hepatotoxicity is estimated to occur in fewer than 1 in 250 children treated.

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Clonazepam (Klonopin)

Clonazepam facilitates inhibitory GABA neurotransmission and other inhibitory transmitters. It is useful in immediate control of seizures, but it may be associated with relatively rapid loss of efficacy against seizures.

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Ethosuximide (Zarontin)

The mechanism of action of ethosuximide is based on reducing current in T-type calcium channels found on thalamic neurons. Spike-and-wave patterns during petit mal seizures are thought to be initiated in thalamocortical relays by activation of these channels. Ethosuximide is used as adjunctive medication to valproic acid if that medication has failed to control seizures.

Contributor Information and Disclosures

Author

Michael C Kruer, MD Assistant Professor, Departments of Pediatrics and Neurosciences, Sanford School of Medicine, University of South Dakota; Physician in Pediatric Neurology and Neurogenetics, Sanford Children's Specialty Clinic, Sanford Children's Hospital

Michael C Kruer, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Society of Human Genetics, Child Neurology Society, and Society for Neuroscience

Disclosure: EMD/Serono Grant/research funds Grant PI

Coauthor(s)

Raj D Sheth, MD Professor, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic

Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, and Child Neurology Society

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

James J Riviello Jr, MD George Peterkin Endowed Chair in Pediatrics, Professor of Pediatrics, Section of Neurology and Developmental Neuroscience, Professor of Neurology, Peter Kellaway Section of Neurophysiology, Baylor College of Medicine; Chief of Neurophysiology, Director of the Epilepsy and Neurophysiology Program, Texas Children's Hospital

James J Riviello Jr, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Up To Date Royalty Section Editor

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

References

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Djukic A, Lado FA, Shinnar S, Moshé SL. Are early myoclonic encephalopathy (EME) and the Ohtahara syndrome (EIEE) independent of each other?. Epilepsy Res. Aug 2006;70 Suppl 1:S68-76. [Medline].
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Cohen BH, Chinnery PF, Copeland WC. POLG-Related Disorders. In: Pagon RA, Bird TD, Dolan CR, Stephens K, eds. GeneReviews [Internet]. University of Washington, Seattle; 1993-2010 Mar 16.
Crompton DE, Berkovic SF. The borderland of epilepsy: clinical and molecular features of phenomena that mimic epileptic seizures. Lancet Neurol. Apr 2009;8(4):370-81. [Medline].
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Wallace SJ. Myoclonus and epilepsy in childhood: a review of treatment with valproate, ethosuximide, lamotrigine and zonisamide. Epilepsy Res. Jan 1998;29(2):147-54. [Medline].
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