Sudden unexpected death in epilepsy sudep is the major cause of epilepsy related. Sudden unexplained death in epilepsy sudep accounts for 717% of deaths among people with epilepsy. The substantial lifetime risk of sudep and the lack of a clear pathophysiological connection between epilepsy itself and sudden death have fuelled increased attention to this phenomenon. Reliable biomarkers and predictors are still lacking, but the most important risk factors are nocturnal and frequent tonicclonic seizures. Sudden death is approximately 25fold higher among people with epilepsy compared with those in the general population, with the greatest risk due to sudden unexpected death in epilepsy. The investigation and certification of these deaths has been criticized.
European journal of epilepsy on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Sudden unexpected death in epilepsy sudep is a common cause of epilepsy related mortality, with an incidence of 1. Generalised tonicclonic seizures are the greatest risk factor for sudep. This observation is particularly significant as lamotrigine is increasingly recommended for the treatment of generalized epilepsy in women.
Conclusion presumed cardiac arrhythmia is the most common cause of sudden, natural death in the young. People with epilepsy are at heightened risk of sudden death compared to the general population. In a uk cohort, sudep accounted for 17% of deaths in those with chronic epilepsy. Sudden unexpected death in epilepsy sudep is the sudden, unexpected death of someone with epilepsy, who was otherwise healthy. A 57yearold man with severe mental retardation and epilepsy was found unresponsive. Sudden death in epilepsy current diagnostic pathology. Sudden unexpected death in epilepsy neurology clinical practice. Both pulmonary and cardiac pathophysiologies have been proposed. The goal of treatment should therefore be seizure freedom, using the lowest effective number and dosage of aeds.
Aug 11, 2009 sudden unexpected death in epilepsy most frequently occurs in people with chronic epilepsy, and seems to be a seizurerelated event. Sudden death in epilepsy, surgery, and seizure outcomes. Most, but not all, cases of sudep occur during or immediately after a seizure. Dravet syndrome ds is an infantileonset intractable epilepsy caused by heterozygous lossoffunction mutations in the scn1a gene, which encodes brain typei voltagegated sodium. For example, it is not due to injury or to drowning following or during a seizure and it is not due to a.
In the fourth paragraph page 1801, beginning the magnitude of the problem, the final sentence should. The leading cause of epilepsy related premature mortality is sudden unexpected death in epilepsy sudep. The frequency of sudep varies depending on the severity of the epilepsy, but overall the risk of sudden death is more than 20 times higher than that in the general population. For some people living with epilepsy, the risk of sudden unexpected death in epilepsy sudep is an important concern.
Sudden death is a recognized consequence of epilepsy. Risk factors for sudden unexpected death in epilepsy. Abstracta significant body of literature suggests that sudden unexpected death in epilepsy sudep has a cardiac mechanism. Apr 01, 20 dravet syndrome ds, or severe myoclonic epilepsy of infancy, is a rare genetic disorder usually due to mutations in the gene encoding a voltagegated sodium channel, scn1a. For those with severe epilepsy or other neurologic impairments, the risk appears to be 1. Henry was 4 years old when he died only a few weeks after his epilepsy diagnosis. The majority of persons with epilepsy develop lasting remission from seizures, although mortality is significantly greater than that of the agematched general population. Carbamazepine was added to the treatment due to his having had one or two seizures in a month. A subject discussed less frequently is sudden unexpected death in epilepsy sudep. Sudden unexpected death in epilepsy sudep is the most common cause of death in patients with intractable epilepsy. Preventing sudden unexpected death in epilepsy epilepsy. The cause of death in sudep is cardiorespiratory arrest, but there is.
We wished to establish the incidence of sudden unexpected death sud in a young cohort with severe epilepsy and learning difficulties. Sudden death in epilepsy seizure european journal of epilepsy. A 20yearold man with intellectual disability and intractable multifocal epilepsy presented to a neurologist for further evaluation and management. Sudden unexpected death in epilepsy most frequently occurs in people with chronic epilepsy, and seems to be a seizurerelated event. Seizures and sudep are probabilistic events that cannot be accurately predicted. Feb 24, 2020 sudep is defined as sudden, unexpected, nontraumatic, nondrowning death in an individual with epilepsy, witnessed or unwitnessed, in which postmortem examination does not reveal an anatomic or toxicologic cause for the death. It had recently been recognized that sudden unexpected deaths in. Although a correlation has been established between freedom from seizures and reduced sudep risk, the exact relationship between seizure outcomes following epilepsy surgery and sudep is still being studied.
Sudep is a rare cause of death in the epilepsy population but exceeds the expected rate of sudden death in the general population by nearly 24 times. Each year, sudden unexpected death in epilepsy sudep occurs in about 1 out of every 1,000 people diagnosed with epilepsy. Since at least 1868, the literature has recorded an excess of deaths associated with epilepsy which are thought to be seizurerelated but in which the exact mechanism of death is not yet established. Objectivessudden unexpected death in epilepsy sudep represents a significant category of mortality in the population with chronic epilepsy. Sudep excludes other forms of seizurerelated sudden death.
Furthermore, the risk of sudden death in the epilepsy population has not been compared with that of the general population. She did not witness either generalized tonicclonic seizures or trauma. Sudden unexpected death in epilepsy sudep was defined in 1996 and is the recommended terminology to certify these deaths. Misplaced brain sodium channels in heart kindle sudden. Epilepsy is a disease that can have significant effects upon a persons life. The national sentinel audit of epilepsy related deaths in 2002 recorded 1200 epilepsy related deaths annually in the uk, of which 42% were felt to be avoidable. The mortality associated with epilepsy, with particular. Sudden unexpected death in epilepsy among patients with.
Ds patients present in the first year of life with severe treatmentresistant epilepsy, intellectual disability, and gait disorder, and have a greater risk of premature death. Aug 23, 2016 it was not until an advocacy group, citizens united for research in epilepsy, or cure, contacted dr. The sudden unexpected death rate in those with epilepsy sudep depends on the population examined. Dravet syndrome ds, or severe myoclonic epilepsy of infancy, is a rare genetic disorder usually due to mutations in the gene encoding a voltagegated sodium channel, scn1a. Recognizing and preventing epilepsyrelated mortality. Evidence of terminal seizure activity may not be present. Populationbased study of the incidence of sudden unexplained. During followup, it was learned that the patient did not use. Article pdf available in the indian journal of medical research. Update on the genetic basis of sudden unexpected death in. Sudden unexpected death is substantially more common in people with epilepsy than in the general population. Little is known about the practice of confirming the cause of sudden death from most nations.
There is considerable evidence that the majority of sudden deaths in epilepsy are seizure related and circumstantial evidence of a generalised tonicclonic seizure having occurred is often found in the form of superficial injuries, a bitten tongue or characteristic postures. Rugggunn, lina nashef, in blue books of neurology, 2009. Sudden unexpected death in epilepsy sudep in new zealand. Sudden unexplained death in epilepsy sage journals. A populationbased study of the incidence of sudep has never been performed.
This phenomenon, known as sudden unexpected death in epilepsy sudep, is believed to be a seizurerelated occurrence, but the exact underlying mechanisms are uncertain. The primary caretaker said that he was on the toilet, rolled his eyes, and stopped breathing. There was no reported history of sudden death among the relatives of most decedents. Sudden unexpected death in epilepsy refers to sudden death of an individual with a clinical history of epilepsy, in whom a postmortem examination fails to uncover a gross anatomic, toxicologic, or environmental cause of death. The magnitude of the problem of sudden unexplained death in epilepsy is unrecognized in both the medical and lay communities. Sudden death is a real problem in the management of epilepsy and is now perhaps the commonest category of seizurerelated death in patients with chronic epilepsy, often affecting young adults in this group. Sudden death in people with epilepsy is an entity of great concern. Sudden unexpected death in epilepsy sudep is the leading cause of mortality in patients with. The journal of the royal college of general practioners 2011 4. People with epilepsy face a significantly increased risk of death from direct effects of seizures as well as indirect effects of epilepsy and its treatment. Generalised tonicclonic seizures are the greatest risk. Sudden unexpected death in epilepsy was defined as. Jul 05, 2011 epilepsy patients can reduce their risk of sudden death by taking precautionary steps such as reducing the number of seizures and taking medicine properly, a study suggests. Emerging evidence suggests that seizure outcomes following.
Both cardiac and pulmonary derangement have been postulated as proximate causes. Sudep causes 1517% of all epilepsy related deaths and carries a lifetime risk of 48% 1, 2. Update on the genetic basis of sudden unexpected death in epilepsy. Sudden unexpected death in epilepsy sudep is the most important. Objectives sudden unexpected death in epilepsy sudep represents a significant category of mortality in the population with chronic epilepsy. Ive posted this american journal of forensic medicine and pathology article in pdf file format. Lack of properstable antiepileptic drug treatment and polytherapy have also been associated with increased sudep risk 12,20. Sudden death, often seizure related, may occur in patients with epilepsy. Some groups of patients are at greater risk of sudep than others, although the reasons for this are only partly understood. Sudep refers to deaths in people with epilepsy that are not caused by injury, drowning, or other known causes. His seizures began at 4 months, the night after his first dpt vaccine, and he continued to have frequent tonicclonic seizures throughout his life.
Sudden unexpected death in epilepsy sudep represents a significant category of mortality in the population with epilepsy with an estimated minimum of 500 deaths occurring yearly in the united kingdom. When an autopsy is done, no other of cause of death can be found. When the death is sudden and unexpected, it can seem especially incomprehensible. If you dont have an adobe acrobat pdf file program, you can download a free version here. A risk for sudden death in epilepsy that often goes. Sudden unexpected death in epilepsy sudep is most often associated with the occurrence of generalized tonicclonic seizures gtcs, a seizure type that can now be detected with high. Increasing awareness of sudden death in pediatric epilepsy. Epilepsy is a common neurological disorder associated with increased morbidity and mortality. Preventing sudden unexpected death in epilepsy epilepsy and. A consistent feature is that most of these deaths are unwitnessed. Sudden unexpected death in epilepsy, also known as sudep, is the main cause of death in patients with epilepsy. Sudden unexpected death in epilepsy sudep is the most common cause of death in intractable epilepsies, but physiological mechanisms that lead to sudep are unknown.
It is defined as the sudden and unexpected, nontraumatic and nondrowning death of a person with epilepsy, without a toxicological or anatomical cause of death detected during the postmortem examination. The incidence is much higher in selected groups, however. Sudden unexpected death in epilepsy sudep is defined as the sudden, unexpected, witnessed or unwitnessed, non. Sudden death in benign childhood epilepsy with centrotemporal. Sudden unexpected death in epilepsy sudep is the leading cause of mortality in patients with refractory epilepsy, and as such has been a major research focus over the last 25 years. Research during the past two to three decades has shown that incidence varies substantially depending on the epilepsy population studied, ranging from 009 per. Sudden unexpected death in epilepsy sudep is an important cause of death in people with epilepsy, especially those with chronic epilepsy. Pdf people with epilepsy have increased risk of premature death, and their life. We sought to determine how often autopsy is undertaken, clinician confidence in cause of death and identify. Sudden unexpected death in epilepsy refers to sudden death of an individual with a clinical history of epilepsy, in whom a postmortem examination fails to uncover a gross anatomic, toxicologic, or. Koroshetz and asked what is being done about sudden death in epilepsy, that the federal. Cardiopulmonary resuscitation was initiated by family members.
Genetic basis of sudden unexpected death in epilepsy. Sudden unexplained death in epilepsy sudep is responsible for. Several different mechanisms probably exist, and most research has focused on seizurerelated respiratory depression, cardiac arrhythmia, cerebral depression, and autonomic. Attention to the condition in the last decade has resulted in refinements in the definition a nontraumatic, nondrowning death that occurs in benign circumstances in an individual with epilepsy see e. The investigation and certification of these deaths has been criticized and there are no specific published guidelines.
Appropriate death certification and mandatory postmortem examination are essential to provide a truer picture of this neglected phenomenon. The earliest sudep research papers were published in seizure, as have scores of sudep papers since. Toxicological analysis was performed in 1140 28% and did not contribute to the cause of death in any. Epilepsy is a common neurological disorder associated with increased morbidity and mortality, including premature death from different causes. Importance children with benign epilepsy with centrotemporal spikes bects have traditionally been considered to have a uniformly good prognosis. Pdf sudden unexpected death in epilepsy researchgate. Investigative practice into sudden death in epilepsy. Patients with epilepsy have an increased mortality rate compared to the general population, to which sudden unexpected death in epilepsy sudep is a major contributor. Sudden unexpected death in epilepsy the journal supports. In one set of instances, epilepsy is the ultimate cause aspiration pneumonia and ignored. A study of incidence in a young cohort with epilepsy and learning difficulty. Sudden death is approximately 25fold higher among people with epilepsy compared with those in the general population, with the greatest risk due to sudden unexpected death in epilepsy sudep. Sudden unexplained death in epilepsy sudep is responsible for 2% to 17% of all deaths in patients with epilepsy, depending on the cohort studied.
Sudden, unexpected death in epilepsy orrin devinsky, m. The death is not known to be related to an accident or seizure emergency such as status epilepticus. Sudden unexplained death and injury in epilepsy ficker. Most postmortem examinations in possible sudep deaths will be at the request of the medicolegal. In the general population of persons with epilepsy, the risk is between 1. Although largely neglected in earlier literature, sudden unexpected death in epilepsy sudep is the most important epilepsy related mode of death, and is the leading cause of death in people with chronic uncontrolled epilepsy. Postmortem investigation of people with sudep, including histological and toxicological analysis, does not reveal a cause of death, and the mechanisms of sudep remain largely unresolved. Sudden unexpected death in epilepsy sudep refers to the sudden death of a seemingly healthy individual with epilepsy, usually occurring during, or immediately after, a tonicclonic seizure. The term sudden unexpected death in epilepsy, or sudep, encompasses sudden unexpected, witnessed or unwitnessed, nontraumatic and nondrowning deaths in epileptic patients where postmortem examination does not reveal a toxicologic or anatomic cause of death, with or without evidence of a seizure and excluding documented status. Abstractpatients with refractory epilepsy face an elevated risk of sudden death, with rates as high as 1% per year. However, benign may be a misnomer because bects is linked to cognitive deficits, a more severe phenotype with intractable seizures, and the potential for sudden unexpected death in epilepsy sudep. Of 83 cases with epilepsy seizures and no macroscopically obvious cause of death, 40 had no history of drugalcohol abuse.
Sudep has been defined as the sudden, unexpected, witnessed or unwitnessed, nontraumatic, and nondrowning death in patients with epilepsy, with or without evidence for a seizure, with exclusion of documented status epilepticus, and when postmortem examination does not reveal a structural or toxicological cause for death. Of the deaths that are thought to be directly related to seizures, sudden unexpected death in epilepsy is probably the commonest category. A seizure may cause a person to have pauses in breathing apnea. Our objective was to investigate the association between some clinical variables and sudden unexpected death in epilepsy sudep to identify risk factors. Sudden unexpected death in epilepsy sudep can affect individuals of any age, but is most common in younger adults aged 2045 years.
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