Take the time to #SpeakUp2SaveLives.Speaking up can make the difference
Sudden Unexpected Death in Epilepsy (SUDEP) is one of the most devastating possible outcomes of epilepsy and occurs when a person with epilepsy passes away suddenly, for no known reason. Research indicates that 1 in 1,000 adults and children with epilepsy die from SUDEP each year1. This is why it’s critical that everyone speak up about SUDEP and epilepsy.
Today is SUDEP Action Day. Please join CURE Epilepsy in supporting this critically important day learning about SUDEP and by speaking up to save lives.
Below, please find information to watch, read, and share with everyone you can. Together, we can make a difference.
Participate in today’s webinar on Breathing & SUDEP, airing live at 1pm CT.
Listen to or watch this week’s episode of Seizing Life® with Libby Boyce and Jessica Brandes, two mothers who lost their sons much too soon to SUDEP.
Watch this message from Dr. Ann Poduri, a former CURE Epilepsy Grant recipient, and learn about the potential connection between Sudden Infant Death Syndrome (SIDS) and SUDEP.
To understand how epilepsy surgery can affect the risk of SUDEP, CURE Epilepsy-grantee Dr. Lisa Bateman and her collaborator, Dr. Catherine Schevon, analyzed rates and causes of mortality in people who had epilepsy surgery versus those who hadn’t.
Their analysis suggests that for those who have had epilepsy surgery, there was a reduction in the occurrence of death, and significantly fewer deaths from SUDEP.
The reduction in the occurrence of SUDEP for those who have had surgery appeared to be most significant in the first 10 years post-surgery.
Frequent, uncontrolled seizures, particularly generalized tonic-clonic seizures (GTCS), are a risk factor for Sudden Unexpected Death in Epilepsy or SUDEP. Epilepsy surgery can be an option to control or eliminate seizures in people with drug-resistant seizures. In addition to helping achieve seizure control, epilepsy surgery is also thought to reduce the risk of SUDEP, however, the evidence for this is limited. A strong understanding of how epilepsy surgery can affect SUDEP occurrence is important as it can help guide treatment decisions.
CURE Epilepsy-grantee, Dr. Lisa Bateman and her collaborator, Dr. Catherine Schevon recently published results from their study comparing the number and causes of death, including SUDEP, in people who had epilepsy surgery versus those who did not undergo surgery.
For their study, which was generously funded by the Henry Lapham Memorial Award, the team analyzed mortality in 590 patients who had undergone epilepsy surgery between 1977 and 2014. Deaths in this surgical group were compared to those in a group of 122 people with drug-resistant epilepsy who did not have epilepsy surgery because they were either not considered suitable candidates or refused surgery.
The team found that number of deaths was significantly reduced in the surgical group versus the non-surgical group, and SUDEP was the main cause of death in both groups. Additional causes included tumors, suicide, accidental death, status epilepticus and other conditions.
Upon further analysis, the researchers discovered that the surgical group had a statistically significant lower rate of SUDEP (1.9 per 1000 patient-years* in the surgical group versus 4.6 per 1000 patient-years in the non-surgical group), as well as a delay in the occurrence of SUDEP relative to the non-surgical group. In the surgical group, on average, SUDEP occurred 10.1 years after surgery, but in the non-surgical group it occurred an average of 5.9 years after the surgery was discussed, but not performed.
The team also found that there was a reduction in SUDEP occurrence in the first 10 years after surgery, however, this benefit appeared to lessen after this time- period. While a larger study is needed to confirm it, this finding suggests that long-term follow up of epilepsy surgery patients is important even if they are seizure-free after surgery.
This CURE Epilepsy-funded study provides evidence for the beneficial effects of epilepsy surgery in reducing overall mortality including SUDEP. A larger study will be helpful in determining how long the benefit can last and whether there are any factors that can predict who might be at greater risk for SUDEP post-surgery.
*Patient-years is a statistical term used to account for the total time all subjects spend in a study and is a more accurate measure of the rate at which an event, in this case SUDEP, occurs in the study population.
Dr. Lisa Bateman is the Director of Surgical Epilepsy Programs at Cedar Sinai Medical Center. Dr. Catherine Schevon is an Associate Professor of Neurology at Columbia University.
 Harden C., Tomson T., et. al. Practice guideline summary: Sudden unexpected death in epilepsy incidence rates and risk factors: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology 2017 Apr 25;88(17):1674-1680.
 Casadei C.H., Carson K.W., et. al. All-cause mortality and SUDEP in a surgical epilepsy population. Epilepsy & Behavior 2020 Jul;108:107093
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This month’s research news features two studies advancing Dravet syndrome research, both utilizing mouse models mimicking the disorder. One study, featuring the work of former CURE Grantee Dr. Lori Isom, tested a new type of drug and found that it decreased the frequency of Sudden Unexplained Death in Epilepsy (SUDEP) in these mice.
In other news, a recent study has increased our understanding of the unique way epilepsy can affect women, showing that women who have seizures that increase in frequency during their menstrual cycle are also more likely to have drug-resistant epilepsy.
Finally, research has helped pinpoint individuals with epilepsy who may be at risk for obstructive sleep apnea, a finding that may help physicians identify who is most at risk and who would likely benefit from treatment.
Summaries of these research discoveries and more are below.
Dravet Syndrome (Featuring the work of former CURE Grantee, Dr. Lori Isom) A new treatment curbs deadly seizures in a mouse model of Dravet syndrome, a severe form of epilepsy, according to a new study. This new drug counteracts the effects of mutations in a gene known as SCN1A, which cause Dravet syndrome. In this study, the drug significantly decreased the overall frequency of SUDEP, lowering the likelihood of a fatal seizure. A clinical trial is evaluating the drug’s safety in children with the syndrome.
Dravet Syndrome A study has utilized a gene therapy technique to reduce seizures and improve behaviors in a mouse model of Dravet syndrome. Researchers used the technique to activate the SCN1A gene, a gene with decreased activity in individuals with Dravet syndrome. The authors note that although more work must be done before the technique can be tested in people, the study supports a potential new approach to treating this cause of epilepsy.
Epilepsy Genetics Researchers have identified a critical new step in how brain cells function in people with one of the most common forms of epilepsy. Using mice, researchers found certain changes in gene activity and regulation in an area of the brain important in temporal lobe epilepsy. The researchers note that the study could eventually lead to targeted treatments that prevent a person from developing epilepsy.
Epilepsy and Fetal Alcohol Spectrum Disorder A study has found a much higher prevalence of epilepsy or history of seizures in individuals with fetal alcohol spectrum disorder (FASD), a disorder that refers to a range of developmental problems that result from maternal drinking during pregnancy. Although more research is needed to establish a direct cause-effect relationship between FASD and epilepsy, the study, which examined the medical histories of individuals from two FASD clinics, supports the link between maternal drinking during pregnancy and a wide array of health impacts to the child.
Drug Resistant Epilepsy and Women More frequent seizures during the menstrual cycle in women with genetic generalized epilepsy have been linked for the first time to drug-resistant epilepsy. Women with catamenial epilepsy, a generalized epilepsy characterized by increased seizure frequency during the menstrual cycle, were nearly four times more likely to have drug-resistant epilepsy than women who experience no changes in frequency.
Epilepsy and Sleep Apnea People with generalized epilepsy who have seizures arising from both sides of the brain simultaneously have a higher risk of obstructive sleep apnea than those who have focal epilepsy where seizures emanate from one area of the brain, according to a new study. These findings may help physicians better understand who is most at risk for obstructive sleep apnea and, therefore, who will benefit most from treatment.
Polytherapy, especially the use of three or more antiepileptic drugs, correlated with a substantially decreased risk for sudden unexpected death in epilepsy, according to findings from a nationwide case-control study conducted in Sweden.
The findings, which were published in Neurology, also demonstrated a link between statin use and a decreased risk for sudden unexpected death in epilepsy.
“There is an urgent need to reduce the risk of sudden unexpected death in epilepsy (SUDEP),” the researchers wrote. “Risk factors related to drug treatment may represent opportunities for prevention.”
Olafur Sveinsson MD, PhD, of the departments of neurology and clinical neuroscience at the Karolinska Institute in Sweden, and colleagues performed the population-based, case-control study to determine the relationship between AEDs, selective serotonin reuptake inhibitors (SSRIs) and “other potentially relevant drugs” and SUDEP risk.
“These results provide support for the importance of medication adherence and intensified AED treatment for patients with poorly controlled GTCS in the efforts to reduce SUDEP risks and suggest that comedication with statins may reduce risks,” Sveinsson and colleagues wrote.
Article, published on SpectrumNews.com (featuring the work of former CURE Grantee, Dr. Lori Isom)
A new treatment curbs deadly seizures in a mouse model of Dravet syndrome, a severe form of epilepsy, according to a new study. A clinical trial is evaluating the drug’s safety in children with the syndrome.
The new drug, which consists of short pieces of RNA called ‘antisense’ molecules, counteracts the effects of SCN1A mutations. The molecules boost the expression of the intact copy of SCN1A, allowing cells to produce normal levels of the sodium channel the gene encodes. They do so by silencing a ‘poison exon’ in SCN1A, a sequence of DNA that ordinarily limits the gene’s expression.
In model mice of Dravet syndrome, the drug significantly decreased the frequency of SUDEP, the new work found, lowering their likelihood of having a fatal seizure during the first 90 days of life from 77 to 3 percent.
Summaries of these research studies are presented below.
Seizure Onset and Dementia: US military veterans over the age of 73 who developed unprovoked seizures of unknown cause were twice as likely to develop dementia compared to veterans without seizures. Veterans who developed these seizures were more likely to be younger, black, have lower income, and a higher prevalence of co-existing illnesses. Learn more
PNES Misdiagnoses and Death: Almost 25% of people who are admitted to a hospital for uncontrolled seizures actually do not have epilepsy. Instead, they have psychogenic nonepileptic seizures (PNES), which resemble epileptic seizures but have a psychological cause rather than a neurological one. Researchers found that patients with PNES are 2.5 times more likely to die compared to people of their same age, and this risk is even greater for people under 30 years of age. Learn more
SUDEP and Socioeconomic Status: Lower socioeconomic status is associated with higher rates of Sudden Unexpected Death in Epilepsy (SUDEP), according to a review of medical records from three geographically diverse areas in the US. People with epilepsy living in the poorest communities were found to be more than twice as likely to suffer from SUDEP than those living in more affluent areas. Learn more
Differing Interpretations of Genetic Data: Different genetic testing laboratories may show conflicting interpretations of genetic data. Because genomic testing has become routine in the diagnosis, management, and treatment of pediatric epilepsy, consistency in data interpretation is important. Learn more
Antiepileptic Drugs and Birth Defects: A small study found that antiepileptic drugs (AEDs) taken during pregnancy do not increase the risk of a fetus’ developing new genetic mutations, i.e., those that arise in the sperm, egg, or fertilized egg rather than being inherited from one or both of parents. Learn more
Body Cooling and Refractory Seizures: A treatment that lowers the body’s temperature called “therapeutic hypothermia” can shorten long-lasting seizures and improve outcomes in children with severe and treatment-resistant forms of epilepsy. Learn more
Epilepsy patients in the poorest communities are more than twice as likely to suffer sudden unexplained death due to epilepsy (SUDEP) than their counterparts at the highest end of the socioeconomic ladder, although the reasons for this are difficult to ascertain, according to a review of medico-legal claims and medical examiners’ office records in three geographically diverse areas across the United States.
A team of researchers led by Daniel Friedman, MD, an associate clinical professor of neurology at NYU Langone School of Medicine’s Epilepsy Center, studied all medico-legal investigations for SUDEP at medical examiner (ME) offices in New York City, Maryland, San Diego County, in 2009 and 2010, and again between 2014 and 2015.
The investigators found 159 SUDEP cases in neighborhoods with the lowest socioeconomic status compared with 43 cases in the highest income brackets. The ratio between the lowest and highest socioeconomic status quartiles was 2.6 in 2009 and 2010, and 3.3 in 2014 and 2015. And although overall cases fell by 36 percent between the two study periods, the disparity between groups remained similar over the full five-year study period, the researchers reported in the April 23 online edition of Neurology.
Epilepsy Status by Zip Codes
The researchers identified all decedents for whom epilepsy/seizure was listed on death certificates as a cause/contributor to death, or as a comorbid condition, as well as coding reports and data pertaining to overall socioeconomic status (SES) in each area by ZIP code. The team also utilized data from the Centers for Disease Control and Prevention and the U.S. Census Bureau.
“We found that community socioeconomic status has a significant impact on SUDEP rates,” Dr. Friedman told Neurology Today. “Further studies are needed to understand the causes of disparities in poorer communities in order to identify potential targets for intervention.”
Several possible factors can influence seizure control, he said, including the inability to afford or obtain seizure control drugs, poorer adherence to drug regimens, and limited access to specialty care for treatment-resistant seizures. Mental illness and substance abuse can also interfere with the ability of individuals to self-manage their epilepsy.
Yet, even when the data was adjusted for these factors, the researchers did not find age, adherence to anti-seizure medications, substance abuse history, or comorbidity between the lowest and highest income areas that might explain the SUDEP disparities, especially to the degree that the calculations indicated.
Unfortunately, the team was unable to determine disability status, seizure frequency, or seizure severity for a majority of the cases, Dr. Friedman said. ME reports and death certificates likely underestimate the true number of SUDEP cases, he added.
“Because SUDEP rates were calculated on the basis of ME investigation and coding of death certificates, we very likely did not ascertain all SUDEPs. Moreover, the observed overall SUDEP rate of 0.33 to 0.66 per 1,000 patient-years was lower than the rate by patient-years reported in earlier population-based studies that have examined medical and vital records,” Dr. Friedman noted.
Press release from The European Academy of Neurology
A new study conducted in Scotland and presented at the European Academy of Neurology (EAN) Virtual Congress showed that the burden of potentially avoidable epilepsy-related deaths in young adults remains large, with those aged between 16 and 24 having a six-fold increased risk of epilepsy-related death. In addition, mortality rates for epilepsy-related deaths did not decrease between 2009 (6.8 per 100,000) and 2015 (9.1 per 100,000) despite advances in treatment during this time. Young adult patients in their early 20s and 30s were found to be at the highest risk, with 78% of epilepsy-related deaths under the age of 55 years classified as potentially avoidable. The aim of the study was to identify the burden of epilepsy-related deaths, what proportion of these are potentially avoidable, and determine the factors that may put patients at an increased risk.
The researchers collected anonymous data from healthcare settings for patients who died between 2009 and 2016, identifying 2,149 epilepsy-related deaths. 60% of these patients (1,276) had one or more seizure-related or epilepsy-related hospital admission in the years prior to death, yet less than a quarter (516) were seen in a neurology clinic. The most common causes of death within the study were sudden unexpected death in epilepsy (SUDEP), aspiration pneumonia, cardiac arrest, congenital malformation, and alcohol-related deaths. The data will be compared with data from living patients with epilepsy of the same age and gender. These comparisons will focus on the patients’ epilepsy type, socio-economic class, standards of care received, and the presence of additional disorders such as depression.
Dr Gashirai Mbizvo, completing this Scottish Epilepsy Deaths Study (SEDS) at The University of Edinburgh, comments that “Epilepsy patients are at a higher risk of early death than the general population, but reasons for this are unclear. We hope that we can use these data to learn lessons and reduce the burden of epilepsy-related deaths in the future, many of which we believe are likely to be avoidable. Highlighting such risk factors, and identifying those that could be prevented, might lead to changes in epilepsy care and, ultimately, fewer epilepsy-related deaths in the future.”
Epilepsy is a chronic noncommunicable disease of the brain that affects around 50 million people globally, making it one of the most common neurological diseases worldwide. It can cause seizures or periods of confusion in patients, which can either occur randomly or from triggers, such as a lack of sleep, stress or drinking alcohol. In many cases, people with epilepsy suffer from negative stigma and discrimination.
Sudden unexpected death in epilepsy (SUDEP) claims the lives of one in every thousand epileptic patients each year, but the precise cause(s) had not yet been identified. The researchers propose a single sequence of causes and effects that collectively focus on obstructive apnea that occurs during the seizure itself. Obstructive apnea is a condition in which the muscles supporting the soft tissues in the throat (tongue & soft palate) temporarily relax, thereby narrowing or closing off the airway to cut off breathing.
Based on detailed animal studies that are sometimes impossible in humans, and striking parallels with a growing body of clinical examples, this framework (1) accounts for the autonomic, cardiac, and respiratory data to date by showing the causal relationships between specific elements, and (2) highlights specific kinds of data that can be used to precisely classify various patient outcomes. The framework also justifies a “near miss” designation to be applied to any cases with evidence of obstructive apnea even, and perhaps especially, in individuals that do not require resuscitation. Lastly, the rationale for preventative oxygen therapy is demonstrated.
With better mechanistic understanding of SUDEP, this team suggests changes for detection and classification to increase survival rates and improve risk stratification.
Objective: Movement-based wearable sensors are used for detection of convulsive seizures. The identification of the absence of motion following a seizure, known as post-ictal immobility (PI), may represent a potential additional application of wearables. PI has been associated with potentially life-threatening complications and with sudden unexpected death in epilepsy (SUDEP). This study aimed to assess whether wearable accelerometers (ACCs) could be used as a digital marker of PI.
Method: Devices with embedded ACCs were worn by patients admitted to an epilepsy monitoring unit. Participants with convulsive seizures were included in the study. PI presence and duration were assessed by experts reviewing video recordings. An algorithm for the automatic detection of post-ictal ACC silence and its duration was also developed and the relationship between the automatically detected duration of post-ictal ACC silence and the duration of the expert-labeled PI was analyzed.
Results: Twenty-two convulsive seizures were recorded from 18 study participants. Twenty were followed by PI and two by agitation. The automated estimation of post-ictal ACC silence identified all the 20 expert-labeled PI. Analysis showed that the duration of the post-ictal ACC silence was correlated with the duration of PI, the age of study participants and the duration of post-ictal generalized electroencephalography suppression (PGES), defined as the immediate post-ictal, generalized absence of EEG activity.
Significance: Researchers highlight a novel application of wearables as a way of recording post-ictal manifestations associated with an increased risk of SUDEP. The occurrence of a fatal seizure is unpredictable and the continuous, non-invasive, long-term identification of risk factors associated with each individual seizure may assume great clinical importance.
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