Common Clinical Practice

When clear triggering factors such as sleep deprivation, photic stimulation or excessive alcohol intake have been identified, specific measures to avoid them should be adopted by the patient, as in JME [41]. The pharmacological sensitivity is probably the same as in JME, with VPA being the first-line treatment, together with LTG, TPM and possibly LVT. Zonisamide may be another option in the future.

TREATMENT OF JUVENILE MYOCLONIC EPILEPSY ILAE Guidelines (Newly Diagnosed Epilepsy)

Despite being a relatively common syndrome, there are no randomized trials reporting efficacy or effectiveness as a primary outcome measure in newly diagnosed JME. VPA has long been regarded as the drug of choice to treat this condition.

'In the absence of class I, class II and class III RCTs for patients with JME, class IV studies suggest that CZP, LTG, LVT, TPM, VPA and ZNS may have some efficacy for patients with newly diagnosed JME. Among these AEDs, no clear first-choice AED exists for initial monotherapy in patients with newly diagnosed or untreated JME based solely on efficacy or effectiveness. Selection of the initial AED therapy for a patient with newly diagnosed JME requires integration of patient-specific, AED-specific, and nation-specific variables that can affect overall response to therapy.' [10]

AAN Guidelines (New AEDs)

No syndrome-specific recommendation is made for JME. SODIUM VALPROATE

In spite of the lack of controlled studies of VPA on JME, this drug continues to be the choice of treatment for JME. In special populations such as women of childbearing age, VPA may be less suitable as the first option because of its potential teratogenicity [2, 3].

Evidence for VPA effectiveness in the treatment of JME mainly consists of case series that have demonstrated good responses, with seizure-free rates ranging from 54% to 93% [5054]. Some studies have reported that seizures in some patients can be controlled with VPA doses as low as 500 mg daily [55, 56], showing that some patients with IGE are sensitive to a small dose of an effective medication. This may be important when considering the teratogenic potential of VPA, as a dose effect has been observed in some studies [3, 57]. Another study tried to show a dose-response relationship with VPA. Sixteen patients with JME were given two different doses of VPA (1000 mg or 2000 mg daily) in a randomized, double-blind, crossover study [58]. Each patient spent 6 months taking the high or low dose before switching, and there was no significant difference in seizure frequency between the two doses [12].

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