Abstracts

Rationale: Rational: Juvenile Myoclonic Epilepsy (JME) typically begins in adolescence and is thought to be life-long. Studies on the clinical course have been cross-sectional or longitudinal with referred patients. We studied the long term evolution of JME using a population-based approach. Methods: Methods: From the Nova Scotia Childhood Epilepsy data we identified all children who developed JME in Nova Scotia between 1977-85 and then re-contacted them in 2006-2008. Hospital records were also reviewed. JME was diagnosed when a neurologically normal patient had generalized tonic-clonic (GTC) seizures plus myoclonus (± absence) and an EEG with normal background plus bursts of irregular, typical or fast generalized spike and wave. Results: Results: Of the 692 children with new onset epilepsy in Nova Scotia, 24 (3.5%) had JME. Age of first seizure was 11 ± 4 years. We contacted 22/24 (92%) (17 women, 5 men) ≥20 years later (mean follow up 25.8 ± 2.4 years, range 20-30) at an average age of 37 ± 4.8 years. All were treated with AEDs and 6 had a terminal remission from all seizure types ≥ 5 years. 16/22 (73%) became seizure free and attempted to discontinue medication. By the end of follow-up, 10 (45%) no longer were treated with AEDs. Four were treated for a mean of 6.5 years (range 4-10 years), then discontinued AEDs and remained completely seizure-free for additional 16 years (range 10.8 - 23 years). Three discontinued AEDs but continued for 18-22 years with non-intrusive myoclonus only. Three discontinued AEDs but continued with rare GTCs + myoclonus. Only 1 had intractable epilepsy at the end of follow up. Convulsive status epilepticus was surprisingly common, occurring in 8/22 (36%) - in 1 patient at the onset and in 7, 1-25 years later. Through the entire course, 6 had ≤10 GTCs, 4 had 11-20, 8 had 21-100 and 4 had >100. Remarkably only 4 had injuries from seizures (laceration, broken teeth, fracture, concussion, shoulder dislocation). 70-80% reported that they were “very satisfied” with their health, work, friendships and social life (Likert scales). 18/22 (82%) graduated from high school and 14/22 (64%) are currently employed. For the 8 unemployed, 5 are supported financially by their spouse and 3 are dependent on the state. Fourteen live with a spouse or partner, 6 live alone. Seven were treated with anti-depressant medications. Ten of the women had ≥1 pregnancy, all with living children; 3 men fathered a living child. For 11/13 of these parents, ≥1 pregnancy occurred outside of a stable relationship. Conclusions: Conclusions: Our sample size is modest but the long follow up and population-based sampling are unique. Apparently all seizure types in JME will resolve in 18%. As well for 14%, only myoclonus persists for ~20 years after stopping AEDs. Therefore, for about one-third of people with JME, troublesome seizures vanish and AED treatment is no longer needed. Depression and social impulsiveness complicate the lives of many but the overall outcome is generally favorable for the majority with good or complete seizure control and reasonable life satisfaction.