In the December 23, 2010, New England Journal of Medicine, Drs. Sillanpaa and Shinnar report long term mortality rates in a group of individuals in Finland with childhood onset epilepsy followed over 40 years. The results showed that 24% of the group had died and 55% of the deaths were related to epilepsy.

Background

There are few studies on long-term mortality in prospectively followed, well-characterized cohorts of children with epilepsy. We report on long-term mortality in a Finnish cohort of subjects with a diagnosis of epilepsy in childhood.

Methods

We assessed seizure outcomes and mortality in a population-based cohort of 245 children with a diagnosis of epilepsy in 1964; this cohort was prospectively followed for 40 years. Rates of sudden, unexplained death were estimated. The very high autopsy rate in the cohort allowed for a specific diagnosis in almost all subjects.

Results

Sixty subjects died (24%); this rate is three times as high as the expected age- and sex-adjusted mortality in the general population. The subjects who died included 51 of 107 subjects (48%) who were not in 5-year terminal remission (i.e., ≥5 years seizure-free at the time of death or last follow-up). A remote symptomatic cause of epilepsy (i.e., a major neurologic impairment or insult) was also associated with an increased risk of death as compared with an idiopathic or cryptogenic cause (37% vs. 12%, P<0.001). Of the 60 deaths, 33 (55%) were related to epilepsy, including sudden, unexplained death in 18 subjects (30%), definite or probable seizure in 9 (15%), and accidental drowning in 6 (10%). The deaths that were not related to epilepsy occurred primarily in subjects with remote symptomatic epilepsy. The cumulative risk of sudden, unexplained death was 7% at 40 years overall and 12% in an analysis that was limited to subjects who were not in long-term remission and not receiving medication. Among subjects with idiopathic or cryptogenic epilepsy, there were no sudden, unexplained deaths in subjects younger than 14 years of age.

Conclusions

Childhood-onset epilepsy was associated with a substantial risk of epilepsy-related death, including sudden, unexplained death. The risk was especially high among children who were not in remission. (Funded by the Finnish Epilepsy Research Foundation.)


ETP Response

The article published in the December 23, 2010 New England Journal of Medicine (NEJM) by Drs. Sillanpaa and Shinnar is an important analysis and clearly demonstrates that epilepsy is not a benign condition. The authors note that seizure freedom can modify the risk of death, and they advocate for early referral to epilepsy centers and earlier epilepsy surgery in eligible patients. Epilepsy Therapy Project's mission is to stop seizures by supporting clinical translational research to accelerate getting new treatments to patients sooner to control seizures. ETP currently helps the cause to stop seizure deaths by:

  1. Offering a patient seizure diary – a self-management resource [developed by the Epilepsy Therapy Project and available on www.epilepsy.com] to monitor seizures and trends that can help patients, families and their physicians base decisions on real data and not free recall. Seeing trends and monitoring progress may help patients see the reality of their situation and make the appropriate therapeutic changes with their medical professionals to stop seizures. The calendar also has features to help people to remember their medications and track when they take or miss them. Our hope is that this will help people take their medications more easily and lead to better seizure control. Moreover, individuals can track other modifiable lifestyle factors that may impact their seizures.
  2. Funding promising new epilepsy treatments and epilepsy research in collaboration with other epilepsy-related organizations.
  3. Encouraging individuals with epilepsy to take this time to open a dialogue with their doctors and other health care providers to understand their own risks. It is also important to talk to others in similar situations to help them cope with difficult times. However, this dialogue should not replace taking real action with their health care team. If someone is suffering with seizures, we would like to suggest taking advantage of online resources at the American Epilepsy Society or National Association of Epilepsy Centers to help individuals find neurologists with expertise in managing epilepsy.
  4. Providing extensive education on epilepsy and its treatments on Epilepsy.com.

We acknowledge the information presented in the NEJM paper can be troubling to those currently suffering with epilepsy and their families, we want to assure you that the Epilepsy Therapy Project is here to help win this fight.

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