SUDEP (Sudden Death with Epilepsy)

Children with epilepsy are at risk for sudden death, although the deaths are not always, or even usually, caused by epilepsy. Although the risk is higher in children with symptomatic epilepsy, epilepsy that can be attributed to an underlying condition, and children with neurodevelopmental disorders, SUDEP can also occur in otherwise typical children whose seizures were easy to stop with medication. Families and primary care clinicians should be aware of SUDEP. A 2017 study found that 75% of primary care clinicians were unaware of SUDEP and most expect pediatric neurologists to discuss such epilepsy-related risks[Berl: 2017], though that frequently does not occur. [Friedman: 2014]

The reported incidence of SUDEP in children with epilepsy has ranged from 0.16 to 0.31 per 1000 patient years; mean of 0.22/1000 patient years or 1 in 4500 children with epilepsy per year. [Harden: 2017] The incidence is higher in those children with drug-resistant epilepsy [Saxena: 2018], defined by the International League Against Epilepsy as having failed to achieve sustained seizure freedom despite adequate trials of 2 tolerated, appropriately chosen and used antiepileptic drug schedules (whether as monotherapies or in combination). [Kwan: 2010]

Although risk factors for SUDEP in children are not well understood, difficult to control epilepsy, the presence of generalized convulsive seizures, poor adherence to medication dosing, nighttime seizures and comorbid conditions are thought to be important. [Donner: 2014]

The 2017 guidelines recommend the following for children with epilepsy: [Harden: 2017]

1. Clinicians caring for children with epilepsy should inform the children's parents or guardians that:
   • There is a rare risk of SUDEP.
   • In 1 year, SUDEP typically affects 1 in 4,500 children with epilepsy; in other words, annually, 4,499 of 4,500 children will not be affected by SUDEP
2. For persons with epilepsy who continue to experience generalized tonic-clonic seizures, (GTCS), clinicians should continue to actively manage epilepsy therapies to reduce seizure occurrences and the risk of SUDEP while incorporating patient preferences and weighing the risks and benefits of any new approach.
3. For persons with frequent GTCS and nocturnal seizures, clinicians may advise selected patients and families, if permitted by their individualized epilepsy and psychosocial circumstances, to use nocturnal supervision or other nocturnal precautions, such as the use of a remote listening device, to reduce SUDEP risk. [Maguire: 2016]
4. Clinicians should inform their persons with epilepsy that seizure freedom, particularly freedom from GTCS (which is more likely to occur with medication adherence), is strongly associated with a decreased risk of SUDEP.