Sudden Unexpected Death in Epilepsy (SUDEP) is defined as the death of an otherwise healthy person with epilepsy that occurs either after a seizure or even when there is no recent seizure activity. Risk factors for SUDEP include having epilepsy for a long period of time, having drug-resistant generalized tonic-clonic seizures, and having seizures at night. In Phase 2, EpLink researchers discovered more about what leads to SUDEP and who is most at risk.
After a tonic-clonic seizure, there is reduced brain activity and a “flattening” of electroencephalography (EEG) brain wave recordings. This flattened EEG pattern is also known as Postictal-Generalized EEG Suppression or PGES. Typically, PGES passes without harm and the patient awakens shortly after the tonic-clonic seizure is over. However, Dr. Hiroshi Otsubo at the Hospital for Sick Children believes that after a very strong tonic-clonic seizure, a deep and prolonged PGES can occur, and may stop a person’s breathing and result in death. This research provides a starting point for identifying patients at risk for SUDEP.
After conducting a large pediatric population-based study of SUDEP in children, Dr. Elizabeth Donner and colleagues found that SUDEP is as common in children as in adults. This is an important consideration for neurologists when counselling pediatric patients and families. The SUDEP research program also found out that many child neurologists do not routinely inform the families of children with children about the risk of SUDEP.
During Phase 2, Dr. Miguel Cortez began screening infants at risk for West syndrome, (e.g. children with tuberous sclerosis complex). West syndrome is a severe form of epilepsy. Dr. Cortez and his team conducted regular EEGs and checked for hypsarrhythmia, an EEG pattern associate with infantile spasms. The team identified four cases of infants with hypsarrhythmia, and have been actively managing these case with drug therapy (i.e. vigabatrin), with the goal of eliminating hypsarrhythmia by the 12-month EEG follow-up. By continuing to screen and treat infants at the first sign of hypsarrhythmia, Dr. Cortez hopes that the infantile spasms – and potential intellectual disability –may never occur in these children.
Publications
- Akiyama et al., Clinical Neurophysiology. 2015
- Baba et al., Scientific Reports. 2016
- Blichowski et al., Epilepsia. 2015
- Ito et al., Brain and Development. 2015
- Jia et al., Orphanet Journal of Rare Diseases, 2018
- Joshi et al., Annals of Neurology. 2016
- Kagawa et al., Epilepsia. 2017
- Kagawa et al., Clinical Neurophysiology. 2016
- Limotai et al., Journal of Neurosurgery: Pediatrics. 2015
- Nakajima et al., Clinical Neurophysiology. 2018
- Nenadovic et al., Seizure. 2018
- Nishimura et al., Epilepsy Research. 2018
- Nita et al., Clinical Neurophysiology. 2016
- Okanari et al., Epilepsia. 2015
- Okanari et al., Pediatric Neurology. 2015
- Okanishi et al., Epilepsy Research. 2016
- Okanishi et al., Epilepsia. 2014
- Okanishi et al., Brain & Development. 2015
- Sakuma et al., Epilepsia. 2016
- Sato et al., Clinical Neurophysiology. 2015
- Song et al., Frontiers in Cellular Neuroscience. 2018
- Stover et al., IBRO Reports. 2017
- Wong et al., Clinical Neurophysiology. 2016
- Wu et al., Experimental Neurology. 2015
