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The syndrome known as nocturnal frontal lobe epilepsy is recognized worldwide and has been studied in a wide range of clinical and scientific settings (epilepsy, sleep medicine, neurosurgery, pediatric neurology, epidemiology, genetics). Though uncommon, it is of considerable interest to practicing neurologists because of complexity in differential diagnosis from more common, benign sleep disorders such as parasomnias, or other disorders like psychogenic nonepileptic seizures. Moreover, misdiagnosis can have substantial adverse consequences on patients' lives. At present, there is no consensus definition of this disorder and disagreement persists about its core electroclinical features and the spectrum of etiologies involved. To improve the definition of the disorder and establish diagnostic criteria with levels of certainty, a consensus conference using formal recommended methodology was held in Bologna in September 2014. It was recommended that the name be changed to sleep-related hypermotor epilepsy (SHE), reflecting evidence that the attacks are associated with sleep rather than time of day, the seizures may arise from extrafrontal sites, and the motor aspects of the seizures are characteristic. The etiology may be genetic or due to structural pathology, but in most cases remains unknown. Diagnostic criteria were developed with 3 levels of certainty: witnessed (possible) SHE, video-documented (clinical) SHE, and video-EEG-documented (confirmed) SHE. The main research gaps involve epidemiology, pathophysiology, treatment, and prognosis., (© 2016 American Academy of Neurology.)

Epilepsy is one of the most common disabling neurological disorders, but significant gaps exist in our knowledge about childhood epilepsy in rural populations. The present study assessed the prevalence of pediatric epilepsy in nine low-income rural counties in the Midwestern United States overall and by gender, age, etiology, seizure type, and syndrome. Multiple sources of case identification were used, including medical records, schools, community agencies, and family interviews. The prevalence of active epilepsy was 5.0/1000. Prevalence was 5.1/1000 in males and 5.0/1000 in females. Differences by age group and gender were not statistically significant. Future research should focus on methods of increasing study participation in rural communities, particularly those in which research studies are rare., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The Commission on Classification and Terminology and the Commission on Epidemiology of the International League Against Epilepsy (ILAE) have charged a Task Force to revise concepts, definition, and classification of status epilepticus (SE). The proposed new definition of SE is as follows: Status epilepticus is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally, prolonged seizures (after time point t1 ). It is a condition, which can have long-term consequences (after time point t2 ), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures. This definition is conceptual, with two operational dimensions: the first is the length of the seizure and the time point (t1 ) beyond which the seizure should be regarded as "continuous seizure activity." The second time point (t2 ) is the time of ongoing seizure activity after which there is a risk of long-term consequences. In the case of convulsive (tonic-clonic) SE, both time points (t1 at 5 min and t2 at 30 min) are based on animal experiments and clinical research. This evidence is incomplete, and there is furthermore considerable variation, so these time points should be considered as the best estimates currently available. Data are not yet available for other forms of SE, but as knowledge and understanding increase, time points can be defined for specific forms of SE based on scientific evidence and incorporated into the definition, without changing the underlying concepts. A new diagnostic classification system of SE is proposed, which will provide a framework for clinical diagnosis, investigation, and therapeutic approaches for each patient. There are four axes: (1) semiology; (2) etiology; (3) electroencephalography (EEG) correlates; and (4) age. Axis 1 (semiology) lists different forms of SE divided into those with prominent motor systems, those without prominent motor systems, and currently indeterminate conditions (such as acute confusional states with epileptiform EEG patterns). Axis 2 (etiology) is divided into subcategories of known and unknown causes. Axis 3 (EEG correlates) adopts the latest recommendations by consensus panels to use the following descriptors for the EEG: name of pattern, morphology, location, time-related features, modulation, and effect of intervention. Finally, axis 4 divides age groups into neonatal, infancy, childhood, adolescent and adulthood, and elderly., (Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.)

The World Health Organization (WHO) International Classification of Diseases (ICD) has been used to classify causes of morbidity and mortality such as epilepsy for more than 50 years. The aims of this critical commentary are to do the following: (1) Introduce the ICD classification, summarize the ICD-9 and ICD-10 codes for epilepsy and seizures, and discuss the challenges of mapping epilepsy codes between these two versions; (2) discuss how the ICD-9 and ICD-10 relate to the revised International League Against Epilepsy (ILAE) terminology and concepts for classification of seizures and epilepsies; (3) discuss how ICD-coded data have been used for epilepsy care and research and briefly examine the potential impact of the international ICD-10 clinical modifications on research; (4) discuss the upcoming ICD-11 codes and the role of the epilepsy community in their development; and (5) discuss how the ICD-11 will conform more closely to the current ILAE terminology and classification of the epilepsies and seizures and its potential impact on clinical care, surveillance, and public health and research., (Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.)

The incidence, prevalence, and mortality of epilepsy vary across countries with different economies. Differences can be explained by methodological problems, premature mortality, seizure remission, socioeconomic factors, and stigma. Diagnostic misclassification-one possible explanation-may result from inclusion of patients with acute symptomatic or isolated unprovoked seizures. Other sources of bias include age and ethnic origin of the target population, definitions of epilepsy, retrospective versus prospective ascertainment, sources of cases, and experienced and perceived stigma. Premature mortality is an issue in low-income countries (LICs), where treatment gap, brain infections, and traumatic brain injuries are more common than in high-income countries (HICs). Death rates may reflect untreated continued seizures or inclusion of acute symptomatic seizures. Lack of compliance with antiepileptic drugs has been associated with increased risk for death, increased hospital admissions, motor vehicle accidents, and fractures in poor communities. Epilepsy is a self-remitting clinical condition in up to 50% of cases. Studies in untreated individuals from LICs have shown that the proportion of remissions overlaps that of countries where patients receive treatment. When the identification of patients is based on spontaneous reports (e.g., door-to-door surveys), patients in remission may be less likely to disclose the disease for fear of stigmatization with no concurrent benefits. This might lead to underascertainment of cases when assessing the lifetime prevalence of epilepsy. In LICs, the proportion of people living in poverty is greater than in HICs. Poverty is associated with risk factors for epilepsy, risk for developing epilepsy, and increased mortality. The high incidence and prevalence of epilepsy found in LICs is also observed in low income individuals from HICs. Epileptogenic conditions are associated with an increased mortality. This may partly explain the difference between incidence and lifetime prevalence of epilepsy in LICs. Poverty within LICs and HICs could be a preventable cause of mortality in epilepsy., (Wiley Periodicals, Inc. © 2014 International League Against Epilepsy.)

Purpose: Epilepsy patients have a significantly higher rate of anxiety and depression than the general population, and psychiatric disease is particularly prevalent among drug resistant epilepsy patients. Symptoms of anxiety and depression might serve as a barrier to appropriate epilepsy care. The aim of this study was to determine if drug resistant epilepsy patients with symptoms of anxiety and/or depression receive different epilepsy management than controls., Method: We identified 83 patients with drug resistant focal epilepsy seen at the Penn Epilepsy Center. Upon enrollment, all patients completed 3 self-report scales and a neuropsychiatric inventory and were grouped into those with symptoms of anxiety and/or depression and controls. Each patient's medical records were retrospectively reviewed for 1-2 years, and objective measures of outpatient and inpatient epilepsy management were assessed., Results: At baseline, 53% (n=43) of patients screened positive for symptoms of anxiety and/or depression. The remaining 47% (n=38) served as controls. Patients with anxiety and/or depression symptoms had more missed outpatient visits per year compared to controls (median 0.84 vs. 0.48, p=0.02). Patients with symptoms of both anxiety and depression were more likely to undergo an inpatient admission or procedure (56% vs. 24%, p=0.02)., Conclusion: For most measures of epilepsy management, symptoms of anxiety and/or depression do not alter epilepsy care; however, drug resistant epilepsy patients with anxiety and/or depression symptoms may be more likely to miss outpatient appointments, and those with the highest burden of psychiatric symptoms may be admitted more frequently for inpatient services compared to controls., (Copyright © 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Objective: Stigma is associated with prevalent epilepsy, but its association with incident epilepsy is unknown., Methods: We identified 209 children and adults with incident seizures from the diverse impoverished community of northern Manhattan. We interviewed 94 participants, aged 16 and older, about lifetime history of depression, health status, medical history, and stigma., Results: At baseline, 18 (22.5%) participants reported experiencing stigma. Stigma was reported by 9 (50.0%) with depression and 9 (14.5%) without depression (P=0.002). At 1 year, 7 (8.1%) participants reported experiencing stigma. Stigma was reported by 5 (31.3%) with depression versus 1 (1.6%) without depression (P<0.0001). At both time points, odds of stigma increased when lifetime history of depression and fair/poor health was present., Conclusions: Previous work revealed negative effects of prevalent epilepsy on stigma. In the low-income, predominantly Hispanic community of northern Manhattan, we found incident epilepsy was associated with stigma when lifetime history of depression or fair/poor health was present., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: To determine the persistence of disparities in health care use and outcomes in socioeconomically diverse populations of epilepsy patients., Methods: We followed patients for a year at one clinic in Houston and two in New York City that serve predominantly low-income, minority, Medicaid-insured, or uninsured patients, and a fourth clinic in Houston that serves a more balanced racial/ethnic and higher socioeconomic status (SES) population. We interviewed the patients several times regarding health care use, seizures, side effects, and outcomes, and examined differences between the patients at the three low-SES clinics and the patients at the high-SES clinic., Key Findings: After controlling for patients' age, gender, race/ethnicity, marital status, seizures, and side effects we found that low SES patients had consistently higher use of the hospital emergency room and more visits to a general practitioner. Hospitalizations were also consistently higher but the differences were not significant in most periods. Neurologist visits were relatively similar. Patients at the low SES sites also had a greater likelihood of having uncontrolled seizures, drug-related side effects, to be stigmatized, and have a lower overall quality of life throughout the study period., Significance: These findings suggest the persistence of SES-related disparities in health care use and outcomes among patients with epilepsy who are receiving regular care., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Purpose: We investigated the cumulative probability of seizure remission and relapse in an adult population with drug-resistant epilepsy and frequent seizures. In addition, we determined clinical predictors of remission and relapse in this population., Methods: IN 2003, we identified 246 patients at a single center with drug-resistant epilepsy defined as at least one seizure per month and failure of at least two antiepileptic drugs. These patients were followed prospectively (cohort design). We examined the cumulative probability of seizure remission and relapse in this population using Kaplan-Meier methodology. Clinical predictors of remission and relapse were also evaluated using Cox regression analysis., Key Findings: The estimated cumulative probability of 12-month seizure remission was 34.6% at 7 years in the entire population and 33.4% when limited to those without surgery. The risk for relapse after a 12-month period of seizure remission was 71.2% at 5 years. Negative predictors of seizure remission included developmental delay, symptomatic generalized epilepsy syndrome, duration of intractability, and number of antiepileptic drugs failed. Localization-related epilepsy was the only negative predictor of relapse., Significance: Among patients with drug-resistant epilepsy, 5% per year enter seizure remission even with a follow-up of 6 years. However, a substantial proportion of these patients relapse after the first year following a remission. The large proportion of patients entering a significant remission gives these patients hope; however, caution should be advised when discussing the likelihood of future seizures., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Purpose: The purpose of this study was to identify sociodemographic disparities in health care use among epilepsy patients receiving care at different sites and the extent to which the disparities persisted after adjusting for patient characteristics and site of care., Methods: Three months of health care use data were obtained from baseline interviews of approximately 560 patients at four sites. One-half of the patients were from a Houston site and two NYC sites that serve predominantly low-income, minority, publicly insured, or uninsured patients. The other half were at the remaining site in Houston that serves a more balanced racial/ethnic and higher sociodemographic population. Differences in general and specialist visits, hospital emergency room (ER) care, and hospitalizations were associated with race/ethnicity, income, and coverage. Logistic regression was used to assess the extent to which the differences persisted when adjusting for individual patient characteristics and site of care., Results: Compared to whites, blacks and Hispanics had higher rates of generalist visits [odds ratio (OR) = 5.3 and 4.9, p < 0.05), ER care (OR = 3.1 and 2.9, p < 0.05) and hospitalizations (OR = 5.4 and 6.2, p < 0.05), and lower rates of specialist visits (OR = 0.3 and 0.4, p < 0.05). A similar pattern was found related to patient income and coverage. The magnitude and significance of the disparities persisted when adjusting for individual characteristics but decreased substantially or were eliminated when site of care was added to the model., Discussion: There are sociodemographic disparities in health care for people with epilepsy that are largely explained by differences in where patients receive care.

Objective: We aimed to determine the likelihood of remission and its clinical predictors in adult patients meeting a strict definition of refractory epilepsy. We also wanted to investigate the influence of treatment regimen on remission., Methods: A total of 246 patients with treatment refractory epilepsy (having at least 1 seizure per month and having not responded positively to at least 2 antiepileptic drugs) were identified in 2000 and followed for 3 years. We used Kaplan-Meier methods to estimate the rate of achieving a 6-month terminal seizure remission and Cox regression analysis to evaluate clinical predictors for seizure remission., Results: The estimated 6-month terminal seizure remission rate was 19% (95% confidence interval, 14-26%) for all cases and 14% (95% confidence interval, 10-21%) when limited to those treated only with medication. Negative predictors for remission included a history of status epilepticus, younger age at intractability, number of failed drug therapies, and presence of mental retardation. No specific drug was significantly associated with remission, and frequently, no clear intervention led to terminal remission., Interpretation: Fifteen percent (approximately 5% per year) of a drug refractory epilepsy population obtained a 6-month terminal seizure remission. Our results signify that no matter how many antiepileptic drug therapies have failed, there is always hope of a meaningful seizure remission in this population. Furthermore, we have elucidated four clinical predictors that can aid the epileptologist in prognostication.

Objective: To determine the likelihood of intracranial pathologic conditions requiring emergency neurosurgical or medical intervention among children without meningitis who presented to the pediatric emergency department after a first complex febrile seizure., Methods: We performed a retrospective review of prospectively collected data for children in neurologically normal condition who presented to a single pediatric emergency department after a first complex febrile seizure (focal, multiple, or prolonged). The complex febrile seizure classification was determined independently by 2 epileptologists. The presence of intracranial pathologic conditions was determined through review of neuroimaging results, telephone interviews, or medical record review., Results: Data for 71 children with first complex febrile seizures were analyzed. Fifty-one (72%) had a single complex feature (20 focal, 22 multiple, and 9 prolonged), and 20 (28%) had multiple complex features. None of the 71 patients (1-sided 95% confidence interval: 4%) had intracranial pathologic conditions that required emergency neurosurgical or medical intervention., Conclusions: For children with first complex febrile seizures, the risk of intracranial pathologic conditions that require emergency neurosurgical or medical intervention is low, which suggests that routine emergency neuroimaging for this population is unnecessary.

Background: No population-based incidence studies of epilepsy have studied syndrome classification from the outset. We prospectively studied the incidence of a single unprovoked seizure and epilepsy in the population of Iceland, and applied the syndrome classification endorsed by the International League Against Epilepsy to this population., Methods: We used a nationwide surveillance system to prospectively identify all residents of Iceland who presented with a first diagnosis of a single unprovoked seizure or epilepsy between December 1995 and February 1999. All cases were classified by seizure type, cause or risk factors, and epilepsy syndrome., Results: The mean annual incidence of first unprovoked seizures was 56.8 per 100,000 person-years, 23.5 per 100,000 person-years for single unprovoked seizures, and 33.3 per 100,000 person-years for epilepsy (recurrent unprovoked seizures). Incidence was similar in males and females. Partial seizures occurred in 40% and a putative cause was identified in 33%. Age-specific incidence was highest in the first year of life (130 per 100,000 person-years) and in those 65 years and older (110.5 per 100,000 person-years). Using strict diagnostic criteria for epilepsy syndromes, 58% of cases fell into non-informative categories. Idiopathic epilepsy syndromes were identified in 14% of all cases., Interpretation: Findings are consistent with incidence studies from developed countries. Although the epilepsy syndrome classification might be useful in tertiary epilepsy centers, it has limited practicality in population studies and for use by general neurologists.

Purpose: Status epilepticus (SE) is a medical emergency associated with a high mortality. Clinical series have suggested that mortality after SE has decreased. No studies have systematically examined trends in incidence, mortality, and case fatality after SE in a well-defined population., Methods: All first episodes of SE receiving medical attention between January 1, 1935, and December 31, 1984, were ascertained through the Rochester Epidemiology Project Records-Linkage System and followed up until death or study termination (February 1, 1996). We calculated incidence rates in the 50-year period (1935-1984), while we considered mortality and case-fatality in the last 30-year period (1955-1984)., Results: Incidence of SE increased over time to 18.1/100,000 (1975 through 1984). The increase was related to an increased incidence in the elderly and to the advent of myoclonic SE after cardiac arrest, a condition not seen in the early decades. In the last decade, approximately 16% of the incidence was due to myoclonic SE. The mortality rates increased from 3.6 per year in the decade 1955-1965 to 4.0/100,000 per year between 1975 and 1984. The 30-day case-fatality (CF) was unchanged, although a trend toward improvement was shown after excluding myoclonic SE., Conclusions: Incidence and mortality rates of SE have increased in the last 30 years. Case fatality remained the same. The increased incidence and mortality are due to the occurrence in the last decade of myoclonic SE after cardiac arrest. The mortality in the elderly was twice that of the youngest age group, across all study periods. Changes in the age and cause distribution of SE over time are responsible for the stable survivorship. There is improvement in survivorship in the last decade when myoclonic SE is excluded.

Nulliparity has been linked to bone loss and fractures, but the contribution of infertility is unclear. The purpose of this study was to assess the long-term risk of fractures among infertile women. In a population-based retrospective cohort study, all 658 Olmsted County, Minnesota, women with infertility (failure to conceive after 1 year despite intercourse without contraception) first diagnosed at Mayo Clinic in 1935-1964 were followed for fractures. Risk was assessed by comparing new fractures of each type to the number expected from sex-specific and age-specific fracture rates in the general population (standardized incidence ratios [SIR]). During 18,130 person-years of follow-up, 184 women experienced at least one fracture when 291 would have been expected on the basis of fracture incidence rates in the general population (SIR 0.6, 95% CI 0.5-0.7). There was no increase in proximal femur fractures (SIR 1.0, 95% CI 0.6-1.6) and a statistically significant decrease in the risk of distal forearm fractures (SIR 0.7, 95% CI 0.5-0.97), two of the three sites traditionally associated with osteoporosis. By contrast, there was a significant increase in subsequent vertebral fractures (SIR 1.9, 95% CI 1.4-2.4) that was consistent across divergent causes of infertility and reported menstrual patterns. Although an apparent increase in the risk of vertebral fractures requires further investigation, we saw no indication of an increase in limb fractures, suggesting that infertility does not have long-term adverse skeletal effects like those reported for athletes and dieters with irregular menses.

Purpose: To determine whether an increased risk of status epilepticus (SE) and complex partial status epilepticus (CPSE) is associated with tiagabine (TGB) therapy., Methods: Thirteen cases in which an EEG, performed on patients with altered mental status taking TGB, was reported to demonstrate spike-and-wave discharges (SWDs) were reviewed by a panel of experts. In addition, all cases of suspected SE from TGB clinical trials were reviewed. The occurrence of SE in four epidemiologic cohorts from Rochester, Minnesota, Turku, Finland, Bronx, New York, and New Haven, Connecticut was analyzed as an external comparison., Results: Review of the 13 cases with reported SWDs found that the majority had had prior EEGs with similar findings, and only three were thought to have electrographic evidence of SE. There was no difference in the frequency of SE or CPSE in the placebo-controlled clinical trials between the TGB-treated (1.0% SE, 0.8% CPSE) and placebo-treated (1.5% SE, 1.5% CPSE) groups. The 5% frequency of SE and 3% frequency of CPSE in the TGB-treated patients in the long-term safety studies, which included 2,248 patients, were very similar to the rates of occurrence of SE and CPSE in the four external cohorts. The major risk factor for the occurrence of SE and CPSE in all groups was a prior episode of SE (p < 0.0001)., Conclusions: Over a 3-year period, SE will occur in 5-10% of patients with epilepsy not in remission. At highest risk are those who have had a prior episode of SE. Treatment with TGB in recommended doses does not increase the risk of SE in patients with partial seizures.

Objective: To investigate the adequacy and efficacy of antiepileptic drug (AED) treatment of nonfebrile status epilepticus (SE)., Patients and Methods: We performed a population-based retrospective cohort study to evaluate the medical management of SE. Participants included 184 residents of Rochester, Minn, who experienced a first episode of nonfebrile SE between 1965 and 1984., Results: Of the 184 patients, 133 (72.2%) received appropriate, prompt medical treatment for SE, i.e., intravenous diazepam, phenytoin, or phenobarbital. In 100 patients (75.8%), the dose of the first AED administered was less than that currently recommended. The first treatment was effective in terminating SE in 41 (31.1%) of 132 patients. The adequacy of treatment was highly predictive of drug efficacy (P = .002). The dose of the second AED treatment was inadequate in 52 (80%) of 65 patients treated., Conclusion: Based on this retrospective study, the treatment of SE is remarkable for both inadequacy and ineffectiveness. The inappropriate use of therapeutic regimens in the management of SE may be an important cause of ineffective medical treatment.

We tested the hypothesis that major depression meeting DSM-III-R criteria or medical therapies for depression increase the risk for unprovoked seizures. Major depression was associated with a sixfold increased risk for unprovoked seizures (95% CI, 1.56-22). The risk remained increased even when controlling for age, sex, length of medical follow-up, and medical therapies for depression. In the absence of known prior neurological insult, major depression is associated with an increased risk for unprovoked seizures.

We asked whether acute symptomatic status epilepticus (SE) increases the risk for subsequent unprovoked seizure compared with less prolonged acute symptomatic seizure. We also explored whether the risk of unprovoked seizure differs by cause. We ascertained all first episodes of acute symptomatic seizure among residents of Rochester, Minnesota, through the Rochester Project's records-linkage system. Information was collected on seizure duration, age, sex, cause, and subsequent unprovoked seizure. At 10 years of follow-up, the risk of unprovoked seizure was 41% for those with acute symptomatic seizure with SE and 13% for those without SE. Controlling for age, sex, and cause, SE increased the risk for subsequent unprovoked seizure 3.3-fold (95% confidence interval, 1.8-6.1) compared with brief acute symptomatic seizures. Among patients with SE, the risk of unprovoked seizure was increased 18.8-fold for patients with anoxic encephalopathy, 7.1-fold for patients with a structural cause, 3.6-fold for patients with a metabolic cause. The increased risk for unprovoked seizure after SE compared with shorter seizures may be due to SE being a marker for severity of injury, damage caused by SE, or a biological substrate associated with the tendency to experience SE.

Purpose: To analyze the nonfatal adverse events (AE) associated with a first episode of status epilepticus (SE)., Methods: We performed a population-based retrospective cohort study to determine the morbidity of SE. Participants included 184 residents of Rochester, Minnesota who experienced nonfebrile SE between 1965 and 1984., Results: The etiology of SE was acute symptomatic in 100 patients and unprovoked in 84 patients. The most common seizure-types were continuous partial (n=56, 30%), generalized convulsive (n=52, 28%), and generalized with focal features (n=32, 17%). Morbidity related to SE was noted in 5 of the 146 patients (3.4%) surviving 30 days. The AE included hemiparesis (n=3), encephalopathy (n=2), mental retardation (n=1), and aphasia (n=1). All patients with morbidity had an acute symptomatic (n=4) or remote symptomatic (n=1) etiology. Thirty-four patients (18.5%) had a second episode of SE., Conclusions: Based on this retrospective study, significant morbidity related to SE is uncommon and is associated with the underlying etiology.

Purpose: Studies evaluating short-term mortality among people who experience status epilepticus (SE) have produced conflicting results. Most studies are derived from clinical series with results affected by unspecified follow-up period and select referral of cases. This study was planned to evaluate short-term mortality after a first episode of SE., Methods: We performed a population-based retrospective cohort study to determine the short-term mortality following a first episode of SE. Between January 1, 1965 and December 31, 1984, we studied all first episodes of afebrile SE who received medical attention in Rochester, Minnesota. Cases were followed until death or end of the study (February 1996)., Results: Mortality within the first 30 days was 19% (38 deaths out of 201 incident SE). Thirty-four deaths (89%) occurred among those with nonfebrile acute symptomatic SE, while 4 deaths (11%) occurred among those with unprovoked SE. Within the acute symptomatic group, after adjusting for age, there was a decreased risk of death in women (RR = 0.4; 95% CI: 0.2-0.9). No effect of duration or seizure type was shown after adjusting for other risk factors., Conclusions: One out of 5 subjects with SE died within the first 30 days. Short-term mortality is associated with the presence of an underlying acute etiology. Among acute symptomatic cases, women had a decreased risk of dying.