Hallazgos de autopsias en la muerte súbita en epilepsia. Revisión bibliográfica y reporte de casos / Autopsy findings in Sudden Death in Epilepsy. Bibliographic review and case repo

Introducción: La muerte súbita en epilepsia, es la complicación más temida de la epilepsia y se reconoce como una de las principales causas de muerte prematura en adultos jóvenes con epilepsia; sin embargo, aún es poco reconocida, informada y escasamente investigada por los profesionales de la salud, lo que ha condicionado al escaso progreso en su detección y prevención. Metodología: Se realizó una revisión narrativa mediante la búsqueda de artículos, en Pubmed, Google académico y Lilacs; utilizando las palabras clave en inglés y español: SUDEP, epilepsia, autopsia, autopsia molecular y sus combinaciones. Además, se analizaron cinco expedientes de la base de datos de la Dirección de Medicina Forense de Tegucigalpa, consignados como SUDEP. Resultados: De los cinco casos analizados, cuatro (80%) eran mujeres y uno hombre; cuatro (80%) no fueron presenciados y ocurrieron durante el sueño. El 80 % (4) de los casos eran menores de 40 años, los hallazgos de autopsia tanto macroscópicos, como microscópicos son similares a los reportado por otros autores. En el análisis de 28 artículos se encontró que el diagnostico de la SUDEP, aun representa un desafío. Conclusión: Pese a que hay avances significativos en la comprensión de los mecanismos y factores de riesgo que contribuyen a la SUDEP, aún es necesario realizar más investigación...(AU)

Prevención de la muerte súbita en la epilepsia fármaco resistente: investigaciones de enfermería / Prevention of sudden death in drug-resistant epilepsy: nursing research

Introducción: Una muerte súbita en epilepsia (SUDEP) ocurre cada diez minutos en todo el mundo. El 30 por ciento de los pacientes epilépticos padecen de epilepsia fármaco resistente (EFR), no logran el control de sus crisis y esto aumenta el riesgo de SUDEP. Muchos aún desconocen los factores de riesgo SUDEP y cómo prevenirlos. Los tratamientos para evitarla aún son insuficientes. Las investigaciones enfermeras aportan resultados positivos en el manejo de la enfermedad. Objetivo: Revisar el estado sobre mortalidad, autogestión de epilepsia e investigaciones enfermeras para prevenir la SUDEP en adolescentes y adultos con EFR. Material y Métodos: Revisión sistemática y búsqueda bibliográfica en las bases de datos PubMed SciELO, SCOPUS, ElSEVIER, MEDES, Organización Panamericana de la Salud (OPS), INFOMED y Google académico. Los criterios de selección: trabajos de la última década en adolescentes y adultos publicados en Cuba y el mundo, enfatizando en las investigaciones enfermeras. Búsqueda de palabras claves en español e inglés, sin restricciones de idioma. Desarrollo: Entre los tratamientos para evitar la SUDEP, destacan los dispositivos de detección de crisis y programas de ayuda online, pero aún son necesarias nuevas opciones. En los factores de riesgo, destacan aquellos relacionados con el sueño. Otros factores de riesgo SUDEP pudieran ser modificables con programas educativos. A pesar de que aún se debaten las formas más adecuadas de ofrecer información sobre SUDEP, los programas educativos enfermeros han evidenciado mayor autogestión, conocimiento de la enfermedad y apego al tratamiento. Conclusiones: El enfermero es el potencial humano ideal, para implementar acciones de autocuidado en las personas con EFR, y empoderarlas con habilidades para el manejo de su enfermedad y prevención de SUDEP(AU)

Introduction: A sudden death in epilepsy (SUDEP) occurs every ten minutes worldwide. Also, 30 percent of epileptic patients who suffer from drug-resistant epilepsy (DRE) fail to control their seizures, so the risk of SUDEP increases. Many epileptic patients are unaware of the risk factors for SUDEP and the ways to prevent it. Treatments to avoid SUDEP are still insufficient. Nursing research provide positive results in the management of the disease. Objective: To review the mortality status, self-management of epilepsy and nursing research to prevent SUDEP in adolescents and adults with DRE. Material and Methods: A systematic review and bibliographic search was carried out in the PubMed SciELO, SCOPUS, ELSEVIER, MEDES, Pan American Health Organization (PAHO), INFOMED and Google Scholar databases. Selection criteria included studies on nursing research conducted in adolescents and adults during the last decade that have been published in Cuba and other countries of the world. Keywords in Spanish and English without language restrictions were used to carry out the search. Development: Crisis detection devices and online help programs stand out among the treatments to avoid SUDEP, but new options are still necessary. Among the risk factors for SUDEP, those related to sleep are highlighted. Other risk factors for SUDEP could be modified with educational programs. Despite the most appropriate ways of offering information about SUDEP are still debated, nursing educational programs have shown greater self-management, knowledge of the disease, and adherence to treatment. Conclusions: The Nurse is the ideal human potential to implement self-care actions in people with EFR. These actions aim to learn new skills for managing their disease and preventing SUDEP(AU)

Muerte súbita en epilepsia / Sudden death in epilepsy

The risk of sudden unexpected death in patients with epilepsy (SUDEP), is 20 to 25 times greater than in the general population. This increased risk is seen specially in refractory epilepsy, with an incidence of 9:1,000 patients-years. Risk factors have been established based on retrospective studies, finding that the frequency of seizures, specially generalized tonic clonic seizures, is the most important one. The physiopathological mechanism of SUDEP is not yet fully understood. Autonomic system abnormalities, as well as cardiac and respiratory changes have been found. The finding of new molecular biomarkers to identify patients with increased risk should be a priority. Treatment is based in the management of risk factors, although clear recommendations are hard to establish given the low level of evidence.

Seizure-related death in children with epilepsy

Children with epilepsy have a significantly higher rate of death than the general population. The cause of premature death among epileptics is contributed by the underlying cause, coexisting neurologic compromise and the epilepsy itself. Mortality directly related to seizures in paediatric epilepsy is the subject of this review. Seizure-related deaths in epileptics arise from status epilepticus, aspiration pneumonia, as well as sudden and unexpected death in epilepsy (SUDEP). Epilepsy per se enhances the risks of accidents and suicide. Children with epilepsy who are otherwise neurologically intact and with normal neuroimaging findings have an exceedingly low risk of seizure-related death. Poor compliance to antiepileptic drugs (AED) , poor ly co nt rol led (intractable and refractory) seizures, impaired cognition, structural/ metabolic aetiology and antiepileptic drug polypharmacy tend to carry poor prognosis. Therefore, parents need to be appropriately advised about the risk of seizurerelated premature death. Early identification, compliance with AED prescription, and treatment of comorbid conditions can reduce mortality risk and improve health outcomes in children with epilepsy. Children with intractable types of epilepsy may benefit from medical marijuana and neurosurgery

Mortality predictors of epilepsy and epileptic seizures among hospitalized elderly / Preditores de mortalidade de epilepsia e crises epilépticas entre idosos hospitalizados

Epilepsy and epileptic seizures are common brain disorders in the elderly and are associated with increased mortality that may be ascribed to the underlying disease or epilepsy-related causes. Objective To describe mortality predictors of epilepsy and epileptic seizures in elderly inpatients.Method Retrospective analysis was performed on hospitalized elderly who had epilepsy or epileptic seizures, from January 2009 to December 2010. One hundred and twenty patients were enrolled.Results The most common etiology was ischemic stroke (37%), followed by neoplasias (13%), hemorrhagic stroke (12%), dementias (11.4%) and metabolic disturbances (5.5%). In a univariate analysis, disease duration (p = 0.04), status epilepticus (p < 0.001) and metabolic etiology (p = 0.005) were associated with mortality. However after adjustment by logistic regression, only status epilepticus remained an independent predictor of death (odds ratio = 13; 95%CI = 2.3 to 72; p = 0.004).Conclusion In this study status epilepticus was an independent risk factor for death during hospitalization.

Epilepsia e crises epilépticas são transtornos cerebrais comuns em idosos e estão associadas com uma taxa de mortalidade elevada que pode ser atribuída à etiologia ou a causas relacionadas à epilepsia. Objetivo Descrever preditores de mortalidade em pacientes idosos internados com epilepsia e crise epiléptica.Método Estudo retrospectivo, envolvendo idosos hospitalizados, de 60 anos ou mais, que foram admitidos de janeiro de 2009 a dezembro de 2010 por terem apresentado epilepsia e crises epilépticas durante a hospitalização. Cento e vinte pacientes foram incluídos no estudo.Resultados A etiologia mais comum foi o acidente vascular cerebral isquêmico (37%), seguido por neoplasias (13%), acidente vascular cerebral hemorrágico (12%), demências (11,4%) e distúrbios metabólicos (5,5%). Na análise univariada, duração da doença (p = 0,04), estado de mal epiléptico (p < 0,001) e etiologia metabólica (p = 0,005) estiveram associados com mortalidade. Entretanto, após ajuste por regressão logística, apenas estado de mal epiléptico permaneceu como preditor independente de morte (odds ratio = 13; IC95% = 2,3 a 72; p = 0,004).Conclusão Neste estudo, estado de mal epiléptico foi um fator independente de risco para morte durante hospitalização.

Indicadores de salud mental asociados a riesgo psicosocial laboral en un hospital público / Psychosocial stress among health care workers

Background: Health care workers are exposed to high stress levels and psychosocial risks. The imbalance between the invested efforts and received rewards acquires special importance in this setting. Aim: To assess the psychosocial risk level and its relationship with depression, distress and psychotropic drug use among health care workers. Material and Methods: Seven hundred eighty two workers (602 females; 180 males) answered self-administered questionnaires to measure psychosocial risk and mental health. Results: Twenty five percent of respondents used psychotropic drugs, 34% had a high level of distress and 23% had depressive symptoms. They also reported a low level of decisional latitude (48%), high emotional demands (47%), low social support (41%) and a significant effort-reward imbalance (67%). Those exposed to job strain (high demands and low decisional latitude), iso-strain (job strain plus low social support), and effort-reward imbalance were twice as likely to suffer symptoms of depression and elevated distress compared to non-exposed subjects. Conclusions: There are high levels of psychosocial stress among health care workers.

Seizure Frequencies and Number of Anti-epileptic Drugs as Risk Factors for Sudden Unexpected Death in Epilepsy

The purpose of this study was to investigate the association between clinical variables and sudden unexpected death in epilepsy (SUDEP) and identify risk factors for SUDEP. SUDEP is one of the most frequent causes of death in patients with epilepsy. Previous studies have reported possible risk factors associated with SUDEP, but there need to be elucidated yet. The cases were 26 patients with SUDEP and three control patients were included for each case, matched for age, sex, and date of initial clinical visit. All demographic and clinical characteristics, including age, sex, disease duration, classification of epilepsy, age at seizure onset, kind and number of antiepileptic drugs, were compared between cases and controls. Seizure frequency was higher in SUDEP cases than in controls (P=0.035). Univariate analysis using conditional logistic regression showed that higher seizure frequency (odds ratio [OR]=3.1, P=0.021) and the number of antiepileptic drugs (AEDs) (OR=2.0, P=0.009) were significantly associated with SUDEP. Only the number of AEDs remained significant in multivariate analysis (OR=1.8, P=0.026). Frequent seizures and multi-drug therapy were associated with SUDEP. This may suggest that the severity of epilepsy is associated with SUDEP, regardless of the type of AED used.

Environmental air pollution is an aggravating event for sudden unexpected death in epilepsy / Exposicao ambiental a poluicao do ar e um evento agravante para a ocorrencia de morte subita nas epilepsias

It is extremely difficult to estimate the occurrence of sudden unexpected death in epilepsy (SUDEP). On the other hand, discovering and carefully evaluating new risk factors that may contribute to the onset of cardiovascular abnormalities in people with refractory epilepsy may prevent fatal events in these individuals. In this context, we should not ignore that urban air pollution is a leading problem for environmental health and is able to cause serious cardiovascular dysfunctions that culminate in sudden death. In this regard, we aimed to determine whether environmental exposure to air pollution is an aggravating event for SUDEP.

É extremamente difícil estimar a ocorrência de morte súbita em epilepsia (SUDEP). Por outro lado, detectar e avaliar cuidadosamente novos factores de risco que podem contribuir para o aparecimento de alterações cardiovasculares em pessoas com epilepsia refratária poderá ser capaz de impedir a ocorrência de eventos fatais nestes indivíduos. Neste contexto, não devemos negligenciar hoje que a poluição do ar nas grandes cidades é um problema para a saúde ambiental, podendo causar graves disfunções cardiovasculares, que culminam em morte súbita. Neste sentido, propusemos nesse trabalho que a exposição ambiental a poluição do ar é um evento agravante para a ocorrência de SUDEP.

Epilepsy kills / Epilepsia mata

People with epilepsy are more likely to die prematurely, and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Thus, the purpose of this article was to alert the scientific community about SUDEP.

Pessoas com epilepsia têm maior chance de morrer prematuramente e a principal causa de morte relacionada à epilepsia é a morte súbita em epilepsia (SUDEP). Assim, o objetivo deste artigo foi alertar a comunidade científica sobre a SUDEP.

Mortality in children with severe epilepsy: 10 years of follow-up / Mortalidade em crianças com epilepsia severa: 10 anos de seguimento

Epilepsy is the main neurological condition in children and adolescents. Unfortunately patients with medical refractory epilepsy are more susceptible for clinical complications and death. We report a prospectively evaluated cohort of children followed for approximately 10 years. Fifty-three of 1012 patients died. Forty-two patients died due to epilepsy or its clinical complications and the main causes of death were pneumonia (in 16 cases), sepses (in 9 patients), status epilepticus (in 8 patients). In 11 patients cause of death was sudden unexpected death in epilepsy (SUDEP). Mental retardation was significantly more frequent in patients who did not die from SUDEP. SUDEP may be a significant condition associated with mortality in children and adolescents with epilepsy.

Epilepsia é uma das condições neurológicas mais comuns em crianças e adolescentes. Infelizmente, pacientes com epilepsias refratárias ao tratamento medicamentoso estão mais susceptíveis a complicações clínicas e óbito. Neste trabalho reportamos, em uma análise prospectiva, um cohort de crianças acompanhadas por aproximadamente dez anos. Cinquenta e três de 1012 pacientes foram a óbito. Quarenta e dois pacientes morreram em decorrência da epilepsia ou de suas complicações clínicas; as principais causas foram pneumonia (16 casos), sepse (9 casos) e estado de mal epiléptico (8 casos). Em 11 pacientes a causa da morte foi SUDEP. A presença de retardo mental foi significantemente associada a mortalidade em crianças e adolescentes com epilepsia.

Sudden death in a child with epilepsy: potential cerebellar mechanisms? / Morte súbita em uma criança com epilepsia: potenciais mecanismos cerebelares?

Epilepsy is the most common neurological disorder in humans. People with epilepsy are more likely to die prematurely than those without epilepsy, with the most common epilepsy-related category of death being sudden unexpected death in epilepsy (SUDEP). The central mechanisms underlying the fatal process remain unclear, but cardiac and respiratory mechanisms appear to be involved. Recently, cerebellar, thalamic, basal ganglia and limbic brain structures have been shown to be implicated in respiratory and cardiac rate regulation. We discuss here the potential mechanisms underlying the fatal process, with a description of cerebellar actions likely failing in that SUDEP process.

Epilepsia é uma das doenças neurológicas mais comuns em seres humanos. Pessoas com epilepsia têm maior chance de morrer prematuramente do que pessoas sem epilepsia, sendo a principal causa de óbito a morte súbita em epilepsia (SUDEP). Os mecanismos centrais envolvidos neste processo fatal não são claros, mas mecanismos cardíacos e respiratórios parecem estar envolvidos. Recentemente, regiões cerebrais como o cerebelo, núcleos talâmicos, gânglios basais e estruturas límbicas foram relacionadas com a variação das frequências cardíaca e respiratória. Aqui, discutiremos potenciais mecanismos envolvidos na SUDEP, com uma descrição do possível papel do cerebelo na sua ocorrência.

Sudden unexpected death in epilepsy: an important concern

Epilepsy is one of the most common neurologic problems worldwide. Unfortunately, individuals with epilepsy are at higher risk of death than the general population, and sudden unexpected death in epilepsy is the most important direct epilepsy-related cause of death. In this review article, our research group focused on the risk factors, mechanisms and preventative measures obtained from clinical and experimental studies on sudden unexpected death in epilepsy.

Incidence of SUDEP in a cohort of patients with refractory epilepsy: the role of surgery and lesion localization / Incidência de SUDEP numa coorte de pacientes com epilepsia refratária: papel da cirurgia e da localização da lesão

OBJECTIVE: The aim of this study was to verify incidence and characteristics of sudden unexpected death in patients (SUDEP) with refractory epilepsy and its relation to previous surgery and lesion localization. METHOD: A cross sectional study was carried out in a cohort of 550 patients with refractory epilepsy followed up by the Epilepsy Surgery Program of the University Hospital of PUCRS, Porto Alegre, Brazil, between January, 1992 and July, 2002. Patients were allocated in two groups (operated and non operated). Seizure type, distribution of interictal spikes and MRI findings were correlated with the SUDEP outcome. RESULTS: The estimated incidence of probable SUDEP amounted to 29:1000 individuals. Probable SUDEP occurred in 1.2 percent of the 166 patients of the non operated group and in 3.7 percent of the 384 patients who were operated (OR=3.02, 95 percent CI 0.69-13.16) (p=0.11). Comparing patients who died to patients alive in the operated group a significant difference was observed concerning the following variables: SUDEP patients had a predominance of generalized seizures (p=0.002), extratemporal lesion on MRI (p<0.001) and epileptiform activity over extratemporal regions (p=0.001). CONCLUSION: In surgically treated patients with refractory epilepsy, an extratemporal location of the lesion and of the epileptiform discharges significantly correlated to SUDEP. Thus it is possible that in those patients; the underlying disease may play a role in the pathogenesis of SUDEP.

OBJETIVO: O objetivo deste estudo é avaliar a incidência e características de morte súbita em pacientes com epilepsia refratária (SUDEP) e sua relação com cirurgia prévia e localização da lesão. MÉTODO: Estudo de Coorte realizado com 550 pacientes com epilepsia refratária seguidos no Programa de Epilepsia do Hospital Universitário da PUCRS, Porto Alegre, Brasil, entre Janeiro, 1992 e Julho, 2002. Os pacientes foram distribuídos em dois grupos (operados e não operados). Tipo de epilepsia, distribuição das descargas interictais e achados em RNM foram correlacionados com SUDEP. RESULTADOS: A incidência estimada de SUDEP foi 29:1000 indivíduos. A incidência de provável SUDEP nos 166 pacientes do grupo de não operados foi 1,2 por cento e nos 384 pacientes no grupo operado 3,7 por cento (OR=3,02, 95 por cento IC 0,69-13,16) (p=0,11). Comparando os pacientes que morreram com os sobreviventes do grupo operado houve diferença significativa nas seguintes variáveis: pacientes com SUDEP apresentam uma predominância de crises epilépticas generalizadas (p=0,002), lesões extratemporais na RNM (p<0,001) e atividade epileptiforme na região extratemporal (p=0,001). CONCLUSÃO: A localização da lesão e as descargas extratemporais em pacientes tratados cirurgicamente com epilepsia refratária correlacionaram-se significativamente com SUDEP. Então, é possível que nestes pacientes uma doença sobrejacente possa estar envolvida na patogênese da SUDEP.

Knowledge, attitude and practice about [Sudden unexpected death in epilepsy patients] among neurologists and paediatricians

Neurologists and pediatricians in Pakistan are generally not aware of sudden unexpected death in epilepsy patients and do they discuss this issue with the patients. To assess the Knowledge Attitude and Practices about [Sudden Unexpected Death in Epilepsy Patients] among neurologists and pediatricians. Department of Neurology, Jinnah Postgraduate Medical Centre, June 2005. Neurologists and pediatricians of Jinnah Postgraduate Medical Centre, Karachi were given a consent form to participate in the study. It was an interview-based study where a questionnaire was applied to the participants. There were 6 questions pertaining to the awareness about sudden unexpected death in epilepsy patients; about discussing the death with the patients and raising issue of sudden death in the discussion of epilepsy management. A total of 41 doctors [26 neurologists and 15 pediatricians] participated in the study. Amongst the neurologists, 13 [50%] were aware of and 6 [23.1%] had seen a case of unexpected death in epilepsy patients. Amongst the pediatricians, only 2 [13.3%] knew about it and had seen a case of sudden death. Overall 15/41[36.6%] doctors were aware of and 8/41 [19.5%] had seen a case of unexpected death in epilepsy patients. The patients of the above doctors never asked about it, except for one relative of a patient, who had lived abroad. Most of the doctors had never discussed this issue with the patients and none of them raised this issue in discussion about epilepsy management. The terminology of unexpected death in epilepsy patients was not known to many doctors and there is a need to increase awareness about this in doctors and general public

Mortalidade por epilepsia no Brasil, 1980-2003 / Mortality from epilepsy in Brazil, 1980-2003

O objetivo deste estudo é contribuir para a análise do comportamento da mortalidade por epilepsia no Brasil, no período de 1980 a 2003. Os dados, obtidos do Subsistema de Informações sobre Mortalidade do Ministério da Saúde (SIM/MS) e da base demográfica do Instituto Brasileiro de Geografia e Estatística (IBGE), revelam redução de 20,35 por cento do coeficiente de mortalidade por epilepsia no Brasil (1,13/100.000 em 1980 a 0,9/100.000 em 2003). A análise da mortalidade, segundo as características epidemiológicas, evidenciou: predominância do sexo masculino; taxas de mortalidade maiores nos grupos etários mais elevados; aumento expressivo da mortalidade na região Nordeste. A redução da mortalidade por epilepsia no Brasil, evidenciada no estudo, está de acordo com padrões internacionais. Foram discutidos os motivos que levariam ao aumento da mortalidade por epilepsia em determinados grupos populacionais, particularmente nos idosos.

The objective of this study is to contribute to the analysis of the behavior of mortality from epilepsy in Brazil over the period 1980-2003. The data were obtained through the SIM/MS (Mortality Information Subsystem of the Brazilian Health Department) and from the demographic database of the IBGE (Brazilian Institute of Geography and Statistics). There was a reduction of 20,35 percent in the mortality rate from epilepsy in Brasil (1,13/100.000 in 1980 to 0,9/100.000 in 2003). The epidemiological analysis of the mortality rate showed: predominance of males; higher mortality coefficient in the advanced age groups; expressive increase of mortality from epilepsy in the Brazilian Northeast over the studied period. The reduction of mortality from epilepsy in Brazil evidenced in this study is in accordance with international standards. The reasons leading to the increase of mortality from epilepsy in certain population groups, particularly in the elderly, were discussed.

Preventing tomorrow's sudden cardiac death in epilepsy today: what should physicians know about this?: [review]

Approximately 1 percent of the population has epilepsy, the most common neurological disorder. Moreover, people with epilepsy are more likely to die prematurely than those without epilepsy, and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Information concerning risk factors for SUDEP is conflicting, but potential risk factors include: age, early onset of epilepsy, duration of epilepsy, uncontrolled seizures, seizure frequency, number of antiepileptic drugs and winter temperatures. Additionally, the cause of SUDEP is still unknown; however, the most commonly suggested mechanisms are cardiac abnormalities during and between seizures. This review discusses the epidemiology, risk factors, etiology, and preventative measures in the management of SUDEP.

Alterações cardiovasculares e morte súbita nas epilepsias / Cardiovascular alterations and sudden death in epilepsy

A epilepsia é a doença neurológica crônica grave mais comum e o fenômeno da morte súbita nas epilepsias (SUDEP) é a causa direta de morte mais importante nesta doença. A causa da SUDEP ainda é desconhecida, no entanto, alterações cardiovasculares têm sido sugeridas como os mecanismos mais comuns. Sendo assim, enfatizamos nesta revisão a relação existente entre SUDEP e alterações cardiovasculares.

Epilepsy is the most common neurological disorder and sudden unexpected death in epilepsy (SUDEP) is the most important direct epilepsy-related cause of death . The cause of SUDEP is still unknown, however, the most commonly suggested mechanisms are cardiac abnormalities. Based on this, in this review the relationship between SUDEP and cardiac abnormalities has been emphasized.