Abstracts

The long term social outcome is unknown for children who develop symptomatic generalized epilepsies (SGE). The seizures in SGE are typically thought to be refractory and mental handicap is frequent [ndash] features that suggest that social outcome will be poor. We used the Nova Scotia population-based childhood epilepsy cohort to identify all children in Nova Scotia who developed SGE between 1977 and 1985. They were followed up in 2003-4. ILAE syndromes included were Lennox Gastaut, West, Myoclonic-astatic(MAE) and SGE undefined ([gt]1 generalized seizure types including myoclonus, tonic, atonic/akinetic + slow spike wave or multifocal EEG). Social outcome was assessed using a semi-structured interview with patients and/or caretakers. 76 children developed SGE, 11% of all childhood epilepsy. Syndromes at onset were: SGE undefined 28 (37%), West 32 (42%), Lennox 4, MAE 9, other 3. During follow up, 19 died between ages 1.5 and 20 years (7/19 died [lt]10 years old). Six (8%) were lost to follow up. For the 51 survivors, the average age of seizure onset was [lt]2 years (1month -12 years) and follow up averaged 20 [plusmn] 6.5 years. Twenty-seven (47%) survivors had intractable seizures at the end of follow up. During the entire course 25 (40%) never had an entire year seizure free and 25 never attempted to discontinue AEDS. By the end of follow up, 21 (38%) had a terminal remission of [gt]4 years and 18 (32%) no longer received AEDs. For social outcome we focused on the 46 patients older than 18 years at the end of follow up (average age 23[plusmn]3 years). 48% were unable to walk and 35(76%) were mentally handicapped. Assessment of social outcome indicated that only 3 were living independently, although 9 were judged potentially capable. 57% still lived at home and 35% lived in group homes or institutions. Financial dependency was marked [ndash] complete dependence on parents or state in 86%. Only 5 earned enough money to support themselves. 60% required assistance with all aspects of care and 38% were spoon or tube fed. Functional reading literacy was achieved by 22% and 40% had some safety literacy. 12 (26%) were judged capable of managing their own finances. 20% were considered socially isolated, although only 8/45 (18%) had their greatest social interaction in normal community activities. Current health was considered satisfactory by 93%. None of the 19 women had become pregnant. Overall only 3 (6%) (all normally intelligent) were seizure-free, off AEDs, living independently and financially independent. The social outcome of children with SGE 20 years after diagnosis is usually disappointing. Most are mentally handicapped and/or highly dependent with ongoing frequent seizures. 25% die and only a tiny percent are seizure free and independent. (Supported by Epilepsy Nova Scotia)