Myoclonic Epilepsy Beginning in Infancy or Early Childhood Medication

Updated: Oct 02, 2020
  • Author: Michael C Kruer, MD; Chief Editor: Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA  more...
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Medication

Medication Summary

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

Go to Antiepileptic Drugs for complete information on this topic.

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Antiepileptic Drugs

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.

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.

Clobazam (ONFI)

Benzodiazepine indicated for adjunctive treatment of seizures associated with Lennox-Gastaut syndrome in children aged 2 years or older.

Stiripentol (Diacomit)

Allylic alcohol that is unrelated to other anticonvulsants. The precise anticonvulsant effect in humans is unknown. Possible mechanisms of action include direct effects mediated through the GABA-A receptor and indirect effects involving inhibition of cytochrome P450 activity with resulting increase in blood levels of clobazam and its active metabolite. It is indicated for treatment of seizures associated with Dravet syndrome in patients aged 2 years or older who are taking clobazam. There are no clinical data to support the use of stiripentol as monotherapy in Dravet syndrome.

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.

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.

Cannabidiol (Epidiolex)

Purified formulation of cannabidiol indicated for treatment of seizures associated with Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), or tuberouis sclerosis complex (TSC) in patients aged 1 year or older. Cannabidiol is a structurally novel anticonvulsant and the exact mechanism by which it produces anticonvulsant effects is unknown. It does not appear to exert its anticonvulsant effects through CB1 receptors, nor through voltage-gated sodium channels.

Fenfluramine (Fintepla)

Fenfluramine and its metabolite, norfenfluramine, increase extracellular levels of serotonin through interaction with serotonin transporter proteins, and exhibit agonist activity at serotonin 5HT-2 receptors. The precise mechanism of action for the treatment of seizures associated with Dravet syndrome is unknown. It is indicated for treatment of seizures associated with Dravet syndrome in patients aged 2 years and older.

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