Summary Objective The risk of premature death is increased in patients with intractable epilepsy. The effect of vagus nerve stimulation ( VNS ) on mortality remains unclear. In a previous study by Annegers et al., mortality was raised, comparable to similar intractable cohorts. Our aim was to calculate standardized mortality ratios ( SMR s), identify epilepsy‐related deaths, and estimate sudden unexpected death in epilepsy ( SUDEP) rates in patients treated with VNS for epilepsy. Methods All United Kingdom patients undergoing VNS between January 1, 1995 and December 31, 2010 at King's College Hospital, London were flagged through the national Medical Research Information Service. Analysis was performed in relation to all deaths occurring by December 31, 2010. Deceased patients were identified from the national death register, and additional information on cause and circumstances of death sought where appropriate to allow for classification of deaths. Results The cohort consisted of 466 patients, with 2993.83 person‐years of follow‐up and a median observation period of 5.9 years. Twenty‐nine deaths occurred, 27 with the device active. SMR was 7.1 (95% confidence interval [CI] 4.8–10.3) for the active device; 12 deaths were considered epilepsy related, including 10 definite or probable SUDEP and one fatal near SUDEP . Definite/probable and fatal near SUDEP occurred at a rate of 3.7/1,000 person‐years. SMR s decreased from 10.5 (5.6–19.5) in the first 2 years after implantation to 5.9 (3.7–9.5) thereafter, although CIs overlapped. SUDEP rates did not alter over time. Significance SMR s and SUDEP rate in this study are comparable to other cohorts with intractable epilepsy, with SUDEP an important cause of death. VNS does not appear to lower the risk of premature death overall. There was a clear trend with lower SMR after 2 years of implantation, although CIs overlapped. SUDEP rates, however, did not change.
Summary Objective This study aimed to determine the prevalence of epilepsy in four European countries (Austria, Denmark, Ireland, and Romania) employing a standard methodology. The study was conducted under the auspices of ESBACE (European Study on the Burden and Care of Epilepsy). Methods All hospitals and general practitioners serving a region of at least 50 000 persons in each country were asked to identify patients living in the region who had a diagnosis of epilepsy or experienced a single unprovoked seizure. Medical records were accessed, where available, to complete a standardized case report form. Data were sought on seizure frequency, seizure type, investigations, etiology, comorbidities, and use of antiseizure medication. Cases were validated in each country, and the degree of certainty was graded as definite, probable, or suspect cases. Results From a total population of 237 757 in the four countries, 1988 (.8%) patients were identified as potential cases of epilepsy. Due to legal and ethical issues in the individual countries, medical records were available for only 1208 patients, and among these, 113 had insufficient clinical information. The remaining 1095 cases were classified as either definite ( n = 706, 64.5%), probable ( n = 191, 17.4%), suspect ( n = 153, 14.0%), or not epilepsy ( n = 45, 4.1%). Significance Although a precise prevalence estimate could not be generated from these data, the study found a high validity of epilepsy classification among evaluated cases (95.9%). More generally, this study highlights the significant challenges facing epidemiological research methodologies that are reliant on patient consent and retrospective chart review, largely due to the introduction of data protection legislation during the study period. Documentation of the epilepsy diagnosis was, in some cases, relatively low, indicating a need for improved guidelines for assessment, follow‐up, and documentation. This study highlights the need to address the concerns and requirements of recruitment sites to engage in epidemiological research.
This study estimated epilepsy prevalence, psychiatric co-morbidity and annual costs associated with epilepsy.We used Danish national health registers to identify persons diagnosed with epilepsy and psychiatric disorders, and persons using antiseizure medication and persons using drugs for psychiatric disorders. We calculated the prevalence of epilepsy and co-morbid psychiatric disorders in Denmark on December 31, 2016, using information on epilepsy and psychiatric disorders based on combinations of hospital contacts and use of antiseizure and psychoactive medication. Further, direct and indirect annual costs associated with epilepsy were calculated using individual-level data from a range of socioeconomic registers.There were 5,044,367 persons alive and living in Denmark on December 31, 2016, including 33,628 persons with at least one hospital contact with epilepsy in the previous five years (epilepsy prevalence 0.67% (0.69% males; 0.65% females)). Among these persons with epilepsy, we identified 12,562 (37.4%) persons with a psychiatric disorder or use of drugs used for psychiatric disorders as compared with 801,052 (15.9%) persons in the general population. The estimated total annual individual net costs associated with epilepsy was €30,683. Compared with prevalence estimates on December 31, 2006, the prevalence of epilepsy on December 31, 2016, was slightly higher in the older population and slightly lower in children CONCLUSIONS: Population estimates from national registers provide epilepsy prevalence estimates of approximately 0.6-0.7% - similar to previous reviews of epilepsy prevalence. In addition, the national sample allowed idenitfication of high prevalence of psychiatric disorders and high societal costs associated with epielspy.
Studies using relative measures, such as standardized mortality ratios, have shown that patients with epilepsy have an increased mortality. Reports on more direct and absolute measure such as life expectancy are sparse. We report potential years lost and how life expectancy has changed over 40 years in a cohort of patients with newly diagnosed epilepsy.We analyzed life expectancy in a cohort of adult patients diagnosed with definite epilepsy between 1970 and 2010. Those with brain tumor as cause of epilepsy were excluded. By retrospective probabilistic record linkage, living or death status was derived from the national death registry. We estimated life expectancy by a Weibull regression model using gender, age at diagnosis, epilepsy etiology, and year of diagnosis as covariates at time of epilepsy diagnosis, and 5, 10, 15, and 20 years after diagnosis. Results were compared to the general population, and 95% confidence intervals are given.There were 249 deaths (105 women, age at death 19.0-104.0 years) in 1,112 patients (11,978.4 person-years, 474 women, 638 men). A substantial decrease in life expectancy was observed for only a few subgroups, strongly depending on epilepsy etiology and time of diagnosis: time of life lost was highest in patients with symptomatic epilepsy diagnosed between 1970 and 1980; the impact declined with increasing time from diagnosis. Over half of the analyzed subgroups did not differ significantly from the general population. This effect was reversed in the later decades, and life expectancy was prolonged in some subgroups, reaching a maximum in those with newly diagnosed idiopathic and cryptogenic epilepsy between 2001 and 2010.Life expectancy is reduced in symptomatic epilepsies. However, in other subgroups, a prolonged life expectancy was found, which has not been reported previously. Reasons may be manifold and call for further study.
Status epilepticus (SE) is a severe neurologic condition associated with high morbidity and mortality. Population-based studies in adults have found a wide range of incidences in various regions in the world. Although the incidence of SE increases almost exponentially in the elderly, data on census-based population statistics in these studies are scarce. This study provides a critical review with an emphasis on census-based population statistics and study characteristics in adults.We performed a systematic search of population-based studies on SE in adults in PubMed using "status epilepticus" in combination with "epidemiology", "population", and "incidence" as search terms, and also screened references. For each identified study, we assessed and extracted the respective population pyramids of study and reference population, and study characteristics.We identified 22 population-based studies (eleven from Europe, six from North America, three from Asia, one from Africa, and one from Australasia). Incidence rates of patients with SE ranged from 1.29 to 73.7/100,000 adults (95% confidence interval (CI): 76.6-80.3) and of SE episodes up to 81.1/100,000 adults (95% CI: 75.8-87.0). The proportions of elderly and very old patients varied by a factor of 2.6 and 8.5, respectively, depending on study period and place. Further major reasons for heterogeneity were retrospective or prospective study design, definition of time to diagnose SE, variable detection of nonconvulsive SE (NCSE), different etiologies, inclusion of children, recurrent episodes, postanoxic patients, exclusion of patients with preexisting epilepsy or patients identified outside the emergency department, and choice of reference population for age- and gender adjustment. The most recent definition and classification of SE by the International League Against Epilepsy (ILAE) 2015 was used in two studies. Four studies (18.2%) reported incidences per ten-year age strata necessary for age adjustment to various reference populations.This critical review reveals a marked heterogeneity among population-based studies on SE in adults. It provides comprehensive details on census-based population statistics in study and reference populations and various study designs and characteristics essential for direct comparisons between studies. Reporting on these essential key features should be improved in population-based studies on SE.
SUMMARY Background. Early identification of potential epilepsy surgery candidates is essential to the treatment process. Aim. To evaluate the clinical applicability of the ILAE definition of drug resistant epilepsy and its potential in identifying surgical candidates earlier compared to three established “older” definitions of drug resistant epilepsy. Material and Methods. Retrospective analysis of 174 patients who underwent epilepsy surgery between 1998 and 2009. Clinical factors and course of disease were extracted from patients' charts. Drug resistant epilepsy was classified according to four definitions and the time until fulfillment of criteria compared. Results. Mean time to fulfillment of criteria of drug resistant epilepsy ranged from 11.8 (standard deviation (SD) 9.8) to 15.6 years (SD 11.3). Time to drug resistance was significantly longer applying the only definition, requiring failure of three antiepileptic drugs (AEDs) (Canada definition), whereas time to fulfillment of all other definitions did not differ. Fifty percent of all patients experienced a seizure free period of ≥1 year prior to being classified as drug resistant, 13% entered another 1-year remission after fulfilling any criteria for drug resistance. Conclusion. We conclude that the ILAE definition identifies drug resistant epilepsy, with similar latency like two of three formerly used definitions. It is an easy applicable tool to minimize the delay of referral to a specialized center. Intermittent remissions delay assessment of drug resistance for all definitions and 13% of patients enter a remission despite established drug resistance.
Epilepsy is a devastating condition with a considerable increase in mortality compared to the general population. Few studies have focused on cause-specific mortality which we analyse in detail in over 4,000 well-characterized epilepsy patients. The cohort comprised of epilepsy patients ≥ 18, treated between 1970 and 2009 at the epilepsy clinic of Innsbruck Medical University, Austria, and living in the province of Tyrol, Austria. Epilepsy diagnosis was based on ILAE guidelines (1989); patients with brain tumor were excluded. Deceased patients and causes of death (ICD-codes) were obtained via record linkage to the national death registry. We computed age-, sex-, and period-adjusted standardized mortality rates (SMR) for 36 diagnoses subgroups in four major groups. Additional analyses were performed for an incidence cohort. Overall cohort: 4,295 patients, 60,649.1 person-years, 822 deaths, overall SMR 1.7 (95 % CI 1.6-1.9), highest elevated cause-specific SMR: congenital anomalies [7.1 (95 % CI 2.3-16.6)], suicide [4.2 (95 % CI 2.0-8.1)], alcohol dependence syndrome [3.9 (95 % CI 1.8-7.4)], malignant neoplasm of esophagus [3.1 (95 % CI 1.2-6.4)], pneumonia [2.7 (95 % CI 1.6-4.2)]. Incidence cohort: 1,299 patients, 14,215.4 person-years, 267 deaths, overall SMR 1.8 (95 % CI 1.6-2.1), highest elevated cause-specific SMR congenital anomalies [10.8 (95 % CI 1.3-39.3)], suicide [6.8 (95 % CI 1.4-19.8)], alcohol dependence syndrome (6.4 [95 % CI 1.8-16.5)], pneumonia [3.9 (95 % CI 1.8-7.4)], cerebrovascular disease at 3.5 (95 % CI 2.6-4.6). Mortality due to mental health problems, such as suicide or alcohol dependence syndrome, malignant neoplasms, and cerebrovascular diseases was highly increased in our study. In addition to aim for seizure freedom, we suggest improving general health promotion, including cessation of smoking, lowering of alcohol intake, and reduction of weight as well as early identification of psychiatric comorbidity in patients with epilepsy.
Summary Objective In 2015, the International League Against Epilepsy ( ILAE ) proposed a new definition of status epilepticus ( SE ): 5 minutes of ongoing seizure activity to diagnose convulsive SE ( CSE , ie, bilateral tonic–clonic SE ) and 10 minutes for focal SE and absence SE , rather than the earlier criterion of 30 minutes. Based on semiology, several types of SE with prominent motor phenomena at any time (including CSE ) were distinguished from those without (ie, nonconvulsive SE , NCSE ). We present the first population‐based incidence study applying the new 2015 ILAE definition and classification of SE and report the impact of the evolution of semiology and level of consciousness ( LOC ) on outcome. Methods We conducted a retrospective population‐based incidence study of all adult patients with SE residing in the city of Salzburg between January 2011 and December 2015. Patients with hypoxic encephalopathy were excluded. SE was defined and classified according to the ILAE 2015. Results We identified 221 patients with a median age of 69 years (range 20‐99 years). The age‐ and sex‐adjusted incidence of a first episode of SE , NC SE , and SE with prominent motor phenomena (including CSE ) was 36.1 (95% confidence interval [CI ] 26.2‐48.5), 12.1 (95% CI 6.8‐20.0), and 24.0 (95% CI 16.0‐34.5; including CSE 15.8 [95% CI 9.4‐24.8]) per 100 000 adults per year, respectively. None of the patients whose SE ended with or consisted of only bilateral tonic–clonic activity died. In all other clinical presentations, case fatality was lower in awake patients (8.2%) compared with patients with impaired consciousness (33%). Significance This first population‐based study using the ILAE 2015 definition and classification of SE found an increase of incidence of 10% compared to previous definitions. We also provide epidemiologic evidence that different patterns of status evolution and LOCs have strong prognostic implications.
The primitive anastomosis between the carotid artery and the vertebrobasilar arteries usually regress, in rare cases they persist beyond fetal development and form vascular anomalies such as primitive persistent hypoglossal artery(PPHA), with prevalence of 0.02-0.1% in the general population.A 77-year-old female presented with aphasia, weakness of both legs and arms. Computed Tomography Angiography (CTA) revealed subacute infarct in right pones, severe stenosis of the right internal carotid artery(RICA) and ipsilateral PPHA. We performed Right carotid artery stenting (CAS) using a distal filter into the PPHA to protect the posterior circulation, with good result.The posterior circulation was utterly dependent on the RICA, therefore, despite the general notion that carotid stenosis is usually associated with anterior circulation infarcts, in cases having vascular anomalies it may cause a posterior stroke. Carotid artery stenting offer a safe and simple solution, however the use of EPD requires special considerations regarding decision on the suitable protection technique and placement.Neurological symptoms in the presence of carotid artery stenosis and PPHA can manifest as ischemia of the anterior and/or the posterior circulation. In our opinion, CAS gives a simple and safe treatment solution.
Death rates of patients with epilepsy are two to three times higher than expected. The aim of our study was to further delineate the causes and the patterns of premature death in patients with epilepsy.We included all patients who were prospectively enrolled between 1970 and 1999 in our epilepsy outpatient clinical database. Patients were followed until death or December 31, 2003. Standardized mortality ratios (SMRs) were calculated using reference rates from the same region.After 48,595 person years of follow-up, 648 of 3,334 patients had died, resulting in an overall SMR of 2.2 (95% confidence interval [CI] 2.0-2.4). The highest SMRs were for patients aged 26-45 years (6.8, 95% CI 3.8-11.2) and with symptomatic epilepsies (3.1, 95% CI 2.3-4.9); those for cryptogenic causes (2.2, 95% CI 1.6-3.1) were also elevated, whereas those for idiopathic causes were not increased (2.7, 95% CI 0.7-7.0) after 2 years of follow-up. SMRs for patients with persistent seizures (3.3, 95% CI 2.6-4.4) were higher than those for seizure-free patients (1.4, 95% CI 0.8-2.3). The highest cause-specific SMRs were for epilepsy (91.6, 95% CI 66.3-123.4), brain tumors (22.7, 95% CI 15.7-31.8), and external causes (2.4, 95% CI 1.8-3.3) at end of study period.Epilepsy patients have a higher-than-expected risk of death throughout life and especially during the first 2 years following diagnosis. Standardized mortality rates were especially high in younger patients and in patients with symptomatic epilepsies. Persistent seizures are strongly related to excess mortality.
