NEW ORLEANS – The long-term social outcome for children with symptomatic generalized epilepsy is usually disappointing, with only 6% having normal intelligence and becoming seizure-free off medication and financially and socially independent.
“Twenty years after the onset of symptomatic generalized epilepsy, the outcome is usually death or complete dependence,” Carol Camfield, M.D., said at the annual meeting of the American Epilepsy Society. “Only a tiny fraction of these children are seizure-free and independent.”
Dr. Camfield of Dalhousie University, Halifax, Nova Scotia, examined outcomes for the 75 children with symptomatic generalized epilepsy (SGE) who were included in the Nova Scotia Childhood Epilepsy Cohort. The cohort includes 692 children diagnosed with epilepsy from 1977 to 1985. The outcomes assessment took place in 2003.
About 11% of the cohort had SGE. Of these 75 children, 45 (60%) developed seizures before 1 year of age. Undefined SGE was present in 28 (37%), 32 (43%) had West's syndrome, 4 (5%) had Lennox-Gastaut syndrome, and 9 (12%) had myoclonic astatic epilepsy. One child had Dravet's syndrome, and one child had partial complex seizures.
By the end of the follow-up period, 25% of the group (19) had died. The mean age at death was 11.6 years; the mean duration of epilepsy was 10.5 years. Of the 56 children who survived, the mean follow-up was more than 19 years.
Of the cohort of 75 patients, 25% were in remission off antiepileptic drugs, 5% were in remission on antiepileptic drugs, 8% were having seizures on antiepileptic drugs, and 37% were considered to have intractable epilepsy. Information was not available on the remaining survivors.
Social outcomes were almost universally poor for the survivors, who were an average of 23 years old at the time of assessment. Of the 46 survivors who were evaluated, almost half were unable to walk, three-quarters had mental handicap, and half had neurologic handicap. Only three were mentally and neurologically normal.
More than half of the group was living with a parent or guardian, and one-third lived in a group home or institution. Only three individuals were living independently, although another five were judged capable of independent living by their caretakers. Of the 46, 41 (89%) were completely financially dependent on their parents or the government.
Functional literacy–reading at about a fifth-grade level–had been achieved by one-quarter of the group. Another 18 (39%) had safety literacy; they could interpret an exit or stop sign and find appropriate bathrooms by looking at the picture on the door.
A good outcome (the ability to live independently) was seen in 8 (17%) of the group. A fair outcome (not capable of independent living, but not needing total care) occurred in 10 (22%). A bad outcome (requiring assistance with all activities of daily living) occurred in 28 (61%).
Almost all of the group (94%) were considered by their caregiver to have satisfactory overall health. Problematic sexual behavior occurred in 6 (11%), 13 (29%) were judged aggressive, and 10 (22%) were socially isolated. Some of the group had more than one problematic behavior.