Juvenile Myoclonic Epilepsy Treatment & Management

  • Author: Elizabeth Carroll, DO; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Jun 8, 2011
 

Approach Considerations

Medical therapy with anticonvulsants typically is needed (see Medication). Avoidance of precipitating events such as alcohol use and sleep deprivation may be useful but is not sufficient to control the seizures of juvenile myoclonic epilepsy (JME).

Go to Antiepileptic Drugs and Epilepsy and Seizures for complete information on these topics.

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Anticonvulsant Therapy

The selection of antiepileptic drugs for the treatment of JME depends on several factors, including the patient’s comorbidities, preferences, previous history of adverse events, and gender.

The US Food and Drug Administration (FDA) has not approved any anticonvulsant solely for the treatment of JME. In 2006, the FDA approved the adjunctive use of levetiracetam for the treatment of JME. Divalproex sodium has been approved as adjunctive therapy for patients with multiple seizure types that include absence seizures. However, many patients with JME do not have absence seizures.

In most patients with JME, seizures are well controlled with monotherapy. Valproic acid has been considered the treatment of choice for JME for many years, but epileptologists are increasingly using other choices as first-line therapies. Approximately 80% of patients with JME become seizure free with valproate monotherapy. Several studies using lamotrigine, topiramate, levetiracetam, and zonisamide have shown similar efficacy to that achieved with divalproex sodium, and in some cases better tolerability.

Levetiracetam is useful for the treatment of myoclonic seizures.[42, 43] Noachtar et al demonstrated in a randomized, double-blinded, placebo-controlled trial that levetiracetam adjunctive therapy reduced all seizure types and myoclonic seizures in patients with JME.[44] Meta-analysis of 2 randomized controlled trials affirm that JME is highly responsive to treatment with levetiracetam.[45] Small, uncontrolled studies of levetiracetam monotherapy in JME suggest efficacy and tolerability.[46, 47]

Lamotrigine may also be useful in the treatment of JME. This agent is ideal for patients who cannot tolerate the adverse effects of valproate (eg, weight gain, tremor, stomach upset, and hair loss). In some patients, lamotrigine monotherapy controls seizures completely. However, recent evidence indicates that lamotrigine may exacerbate myoclonic jerks.

Data from a recent open-label study suggested that lamotrigine was better tolerated than valproate, with similar efficacy.[48] A European expert opinion study showed that lamotrigine was first-line choice for JME in adolescent females, whereas valproate was the first-line choice in adolescent males.[49]

Topiramate has been useful in the treatment of primary generalized seizures; it may effectively prevent the seizures of JME.[50]

Findings from an open-label study also suggested that zonisamide might be effective and well tolerated in patients with JME.[51]

In general, low doses of appropriate anticonvulsants are needed to successfully treat JME. Although treatment with phenytoin, carbamazepine, or phenobarbital may control some seizure components of JME (typically at high doses), these drugs may increase seizure frequency (eg, myoclonic exacerbation with carbamazepine) and occasionally precipitate new seizure types, such as absence seizures. However, they may be used in combination if the patient’s condition does not respond to other drugs.[52]

Clonazepam is often used during seizure exacerbations in patients with JME; however, it is inadequate as long-term treatment.

A patient’s medication should rarely be changed because he or she is not having seizures. In medical school, physicians are taught to treat patients and not serum concentrations. A low-dose requirement is not unusual; in fact, the great majority of patients with JME need relatively low levels of anticonvulsants to achieve adequate seizure control (as long as it is an appropriate medication for the syndrome).

A valproic-acid serum concentration of less than 20 mcg/mL is certainly a concern, and the patient’s spouse or other observer should be interviewed for confirmatory evidence that the patient is not having seizures.

Special considerations in pregnant women

Most of the time, when a neurologist examines a pregnant woman with epilepsy, it is after the first 6 weeks of gestation (ie, after the neural tube normally closes). That is one of the reasons why all women of childbearing age who are taking anticonvulsants should also take folic acid 1 mg/d.

In general, most epileptologists believe that the anticonvulsants that help that patient the most should be continued during pregnancy. Frequent monitoring of drug levels is recommended, as pregnancy induces clinically significant changes in drug metabolism, clearance, and volume of distribution. Women with JME are no different from other women who need to take anticonvulsants.

A great majority of children born to women taking anticonvulsant monotherapy are healthy. Valproic acid and divalproex sodium clearly pose a recognized risk of neural-tube defects (category D) that is higher than the risk associated with older anticonvulsants. Evidence suggests that supplementation with folic acid may decrease this risk.

Experience is limited with the newer anticonvulsants, including lamotrigine (category C), levetiracetam (category C), and topiramate (category C). Laboratory data indicate some teratogenicity with topiramate, but the effect in humans is unknown. Animal studies have revealed no evidence of teratogenicity related to lamotrigine. Lamotrigine is a weak folic-acid antagonist in the gut; therefore, folic-acid supplementation is required in women of childbearing age taking this drug.

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Surgical Care

Surgical treatment is not indicated, because JME is a primary generalized epilepsy. Some uncontrolled studies have suggested that vagal nerve stimulation might be helpful for patients with intractable seizures of primary generalized onset, such as JME.

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Modification of Activity

Seizure precautions include warnings about unpredictable lapses of consciousness due to seizures during a variety of activities, including the following:

  • Driving vehicles or flying aircraft
  • Immersing oneself in water (eg, for baths, swimming, and other purposes)
  • Being at heights (eg, on roofs, scaffolds, and ladders)
  • Using fire (eg, on stoves, in ovens, in open fires)
  • Using power tools (eg, drills and saws)

Such precautions, including restrictions on driving, must be observed until seizures that impair consciousness are controlled (ie, the patient is seizure free) for the recommended period—typically 3-12 months, though the length varies from state to state in the United States. Patients with seizures cannot have a commercial driving license until they complete a seizure-free period of 5 years. In addition, patients with seizures are not permitted to fly aircraft.

Studies have shown that patients with JME experience decreases in quality of life similar to those experienced by patients with other epileptic syndromes.[53]

Patients with suspected seizures manifesting as lapses of consciousness during wakefulness should be educated and warned about seizure precautions. Documenting on the patient’s chart that driving and occupational hazards for people with seizures were discussed is helpful. Physicians should be aware of state regulations regarding driving, which vary considerably among states and nations.

Warnings should be tailored to each specific patient, and they should include factors such as seizure control, time of the occurrence of seizures, medication compliance, and the patient’s occupation, among other concerns.

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Consultations

JME is rarely diagnosed in the primary care setting. Most often, an epileptologist diagnoses the condition after several years of inadequate treatment with medications such as carbamazepine or phenytoin.

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Contributor Information and Disclosures
Author

Elizabeth Carroll, DO  Resident Physician, Department of Neurology, University of South Florida College of Medicine

Elizabeth Carroll, DO is a member of the following medical societies: American Academy of Neurology, American Osteopathic Association, and Florida Osteopathic Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Jose E Cavazos, MD, PhD, FAAN  Associate Professor with Tenure, Departments of Neurology, Pharmacology, and Physiology, Program Director of the Clinical Neurophysiology Fellowship, University of Texas School of Medicine at San Antonio; Co-Director, South Texas Comprehensive Epilepsy Center, University Hospital System; Director of the San Antonio Veterans Affairs Epilepsy Center of Excellence and Neurodiagnostic Centers, Audie L Murphy Veterans Affairs Medical Center

Jose E Cavazos, MD, PhD, FAAN is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, and American Neurological Association

Disclosure: GXC Global, Inc. Intellectual property rights Medical Director - company is to develop a seizure detecting device. No conflict with any of the eMedicine articles that I wrote or edited.

Mark Spitz, MD  Professor, Department of Neurology, University of Colorado Health Sciences Center

Mark Spitz, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, and American Epilepsy Society

Disclosure: pfizer Honoraria Speaking and teaching; ucb Honoraria Speaking and teaching; lumdbeck Honoraria Consulting

Specialty Editor Board

Ramon Diaz-Arrastia, MD, PhD  Professor, Department of Neurology, University of Texas Southwestern Medical Center at Dallas, Southwestern Medical School; Director, North Texas TBI Research Center, Comprehensive Epilepsy Center, Parkland Memorial Hospital

Ramon Diaz-Arrastia, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, New York Academy of Sciences, and Phi Beta Kappa

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

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Findings in a man with a history of generalized tonic-clonic seizures (mostly nocturnal) and myoclonic jerks (mostly in the morning) since the age of 14 years. Carbamazepine exacerbated his myoclonic seizures. Sleep-deprived EEG was digitally recorded and then plotted by using an analog paper machine. The patient was getting drowsy when this burst of polyspike and slow wave was recorded.
 
 
 
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