Myoclonic Epilepsy Beginning in Infancy or Early Childhood Treatment & Management

  • Author: Michael C Kruer, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: Dec 8, 2011
 

Approach Considerations

The mainstays of medical therapy for myoclonic epilepsy are valproic acid (sodium valproate), ethosuximide, or benzodiazepines (clonazepam or clobazam).[10] . A number of different antiepileptic medications may be efficacious, although phenobarbital, lamotrigine, vigabatrin, and carbamazepine may worsen the seizures in some cases.[11] Combination therapy with valproic acid and benzodiazepines is often helpful. Stiripentol may have a niche role in treating Dravet syndrome.[10]

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.

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.[12] 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, 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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  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. Aug 2006;70 Suppl 1:S68-76. [Medline].

  3. Kelley SA, Kossoff EH. Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress. Dev Med Child Neurol. Nov 2010;52(11):988-93. [Medline].

  4. Dravet C. The core Dravet syndrome phenotype. Epilepsia. Apr 2011;52 Suppl 2:3-9. [Medline].

  5. Michoulas A, Farrell K. Medical management of Lennox-Gastaut syndrome. CNS Drugs. May 1 2010;24(5):363-74. [Medline].

  6. Shahwan A, Farrell M, Delanty N. Progressive myoclonic epilepsies: a review of genetic and therapeutic aspects. Lancet Neurol. Apr 2005;4(4):239-48. [Medline].

  7. 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.

  8. 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].

  9. Korff CM, Nordli DR Jr. Epilepsy syndromes in infancy. Pediatr Neurol. Apr 2006;34(4):253-63. [Medline].

  10. 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].

  11. Andrade DM, Hamani C, Minassian BA. Treatment options for epileptic myoclonus and epilepsy syndromes associated with myoclonus. Expert Opin Pharmacother. Jul 2009;10(10):1549-60. [Medline].

  12. Wheless JW, Sankar R. Treatment strategies for myoclonic seizures and epilepsy syndromes with myoclonic seizures. Epilepsia. 2003;44 Suppl 11:27-37. [Medline].

 
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