Juvenile Myoclonic Epilepsy

Introduction: Juvenile myoclonic epilepsy (JME) is often regarded as a common epilepsy syndrome in which seizures are relatively easy to control and drug resistance should raise the suspicion about incorrect diagnosis, inappropriate lifestyle, noncompliance or inadequate/insufficient treatment; Nevertheless, true resistance to adequate drugs is not uncommon and is found in 15.5-37% of JME series of tertiary epilepsy centers. Prognostic factors for refractoriness: JME is a heterogeneous condition. Age of onset is slightly higher in the group with easy to control seizures. Non - classic JME phenotypes are more prevalent in drug resistant cases. A recent meta - analysis suggested six variables as prognostic factory for refractoriness including, having three seizure types, non - classic clinical presentation, childhood absence epilepsy / absence in general evolving to JME, psychiatric comorbidities, earlier age at seizure onset and praxis - induced seizures. Treatment options: at the present time Valproate (VPA) is the first choice antiepileptic drug (AED) in men with JME. The next AED choices if VPA fails to control the seizures or is poorly tolerated will be VPA and Lamotrigine (LTG)in combination, Levetriacetam (LEV)LTG or Topiramate. If all mentioned AEDs fail to control the seizures Zonisamide either as monotherapy or add on drug may be an effective option.Evidence suggests that VPA should not be given to women of childbearing age. The safest AEDs in terms of teratogenicity are probably LEV and LTG respectively. When dealing with refractory JME it may be well to remember the possible benefits of older therapies such a Primidon, phenobrbital, Colobazam and Clonazepam. Acetazolamide and Prampanel are other alterative choices in pharmacoresistant JME patients. It is important to mention that some AEDs may aggravates seizures in these patients.Vagal nerve stimulation may be considered as a last resort in pharmacoresistant cases, Conclusion: JME is a heterogeneous epilepsy syndrome incorporating different subtypes; as a result, it will be characterized by different outcomes. Future prospective studies will be needed in this challenging population to optimize the management of intractable cases and to test the reliability and genealizability of above mentioned prognostic factors.