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Myoclonic Epilepsy Beginning in Infancy or Early Childhood Treatment & Management

  • Author: Michael C Kruer, MD; Chief Editor: Amy Kao, MD  more...
 
Updated: Mar 14, 2016
 

Approach Considerations

The mainstays of medical therapy for myoclonic epilepsy are valproic acid (sodium valproate), ethosuximide, or benzodiazepines (clonazepam or clobazam).[11] . A number of different antiepileptic medications may be efficacious, although phenobarbital, lamotrigine, vigabatrin, and carbamazepine may worsen the seizures in some cases.[12] Combination therapy with valproic acid and benzodiazepines is often helpful. Stiripentol may have a niche role in treating Dravet syndrome.[11, 13] In 2016, an experimental cannabis-based drug successfully treated children with Dravet syndrome. Results of the 120-patient trial showed patients taking the drug achieved a median reduction in monthly convulsive seizures of 39% compared with a reduction on placebo of 13%.[14] More Phase III trials are forthcoming.

Adrenocorticotropic hormone (ACTH), steroids, and immunoglobulins have been tried but have not shown conclusive benefit.

 

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Antiepileptic Medication

Patients with benign forms of myoclonic epilepsy often respond well to valproic acid or clonazepam. Either one of the drugs may be started; if both fail independently, they may be combined. The duration of treatment is tailored on an individual basis but is usually approximately 5 years. Second-line medications include ethosuximide, zonisamide, and topiramate.

Treatment for the more severe myoclonic epilepsies is more difficult and similar to the approach used for Lennox-Gastaut syndrome. For example, patients with Dravet syndrome typically have medically refractory epilepsy and often require polytherapy.[15]

Go to Antiepileptic Drugs for complete information on this topic.

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Dietary Modification and Activity Restrictions

The ketogenic diet may be useful in children with particularly refractory epilepsy.[16] This diet should be instituted carefully, paying particular attention to the possibility of dehydration.

Caution should be used in children with drop attacks, as they may fall and injure themselves. A helmet can be protective. Routine seizure precautions are applicable.

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Consultations and Long-Term Monitoring

Patients should be evaluated by a pediatric neurologist. If dysmorphic features are present, a genetic evaluation may be useful.

Serial EEGs often are required to ensure that patients are responding to treatment and that subclinical seizures are not occurring. Typical EEG findings in responsive patients include the disappearance of polyspike and wave activity and other associated epileptiform discharges.

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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, Society for Neuroscience

Disclosure: Nothing to disclose.

Coauthor(s)

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

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, 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 Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

Acknowledgements

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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  2. Djukic A, Lado FA, Shinnar S, Moshé SL. Are early myoclonic encephalopathy (EME) and the Ohtahara syndrome (EIEE) independent of each other?. Epilepsy Res. 2006 Aug. 70 Suppl 1:S68-76. [Medline].

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  14. Hirschler, B. Cannabis drug succeeds in epilepsy, doubling value of GW Pharma. Reuters. March 14, 2016. Available at http://www.reuters.com/article/us-gw-pharma-cannabis-idUSKCN0WG16Z.

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