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Knowledge of epilepsy and familiarity with this disorder in the U.S. population: results from the 2002 HealthStyles Survey.

PURPOSE: To assess perceptions of knowledge and experience with epilepsy and seizures in the U.S. population to develop communication campaigns to improve the public's understanding of epilepsy. In a national survey, focal points included the public's knowledge of the disorder, whether people know someone who has it, exposure to epilepsy-related information, and knowledge about how to respond to a person having a seizure. METHODS: The Epilepsy Program of the Centers for Disease Control and Prevention included nine items on an annual mail survey that targeted a representative sample of the U.S. population. Data were weighted to be representative of the U.S. population. chi2 analyses were performed, and standardized residuals were used to examine the associations between responses and demographic variables. RESULTS: Responses were obtained from 4,397 persons. Despite the low prevalence of epilepsy, results indicate that about half of all persons have witnessed an epileptic seizure either in person or on television; about one third of all persons know someone with epilepsy, but relatively few are familiar with epilepsy, how to respond to a seizure, or with the Epilepsy Foundation. CONCLUSIONS: In general, the public has relatively little knowledge about epilepsy. Educational campaigns that inform the public about this disorder and about seizures should work through community settings to improve the general public's understanding of epilepsy.

Adolescent↗

Obstructive sleep apnea in a clinical series of adult epilepsy patients: frequency and features of the comorbidity.

PURPOSE: The aim of this study was to evaluate the rate and features of obstructive sleep apnea (OSA) in adult epilepsy patients. METHODS: Two hundred eighty-three adult epilepsy patients (137 men; mean age, 33 years; range, 18-70 years) were prospectively screened for OSA by means of a structured interview. Those in whom OSA was clinically suspected were monitored for a full night by using a portable device (Polymesam), and OSA was diagnosed when they had an Apnea/Hypopnea Index greater than five. RESULTS: Coexistence of OSA with epilepsy was found in 10.2% (15.4% of the male and 5.4% of the female) epilepsy patients investigated. The OSA was mild in 66.6%, moderate in 22.2%, and severe in 11.1% of the cases. The "epilepsy + OSA" patients were older, heavier, more frequently male, and sleepier (p < 0.05) than those with "epilepsy only." Furthermore, they experienced their first seizure at an older age (p < 0.05). CONCLUSIONS: Systematic investigation reveals that OSA is frequent in epilepsy patients. The major risk factors for OSA in our epilepsy patients were the same as those typically found in the general population. Of the epilepsy-related factors, older age at onset of seizures appears to be significantly related to comorbidity with OSA (p < 0.05). The presence in epilepsy patients of these features should alert the clinician to the possibility of an underlying OSA.

Adolescent↗

ILAE Commission of European Affairs Subcommission on European Guidelines 1998-2001: The provision of epilepsy care across Europe.

PURPOSE: To assess the needs and resources available in the provision of basic epilepsy care across Europe. METHODS: A mailed questionnaire was used, the European Epilepsy Services inventory (EESI). The EESI was distributed to all 36 European chapters of the International League Against Epilepsy (ILAE), and answers were obtained from 32, a response rate of 89%. For the purpose of studying trends across Europe, the chapters were divided into a Western, an Eastern, a Central, and a Southern group. RESULTS: The survey results showed that there was a wide range in the number of physicians and specialists involved in epilepsy care across Europe, with a trend toward higher numbers of neurologists, pediatricians, and pediatric neurologists in Eastern Europe. Many different specialties were involved in epilepsy care, and many chapters reported differences in the provision of care across their countries, with less possibility for patients to see a specialist in the least provided areas, where most epilepsy patients were cared for by general practitioners and internists. Problems with high costs of the newer antiepileptic drugs were most pronounced in Eastern Europe. Problems with lack of comprehensive care and of epilepsy specialists, with stigma and social problems, and with insufficient professional education and knowledge about epilepsy were reported all across Europe. CONCLUSIONS: Knowledge about differences in the pattern of provision of epilepsy care and about the main problems encountered by the European ILAE chapters is of importance in the continuing efforts to improve management of epilepsy all over Europe.

Adult↗

A population-based study of the prevalence, clinical characteristics and effect of ethnicity in epilepsy.

UNLABELLED: The aim of this study was to determine the prevalence and clinical characteristics of epilepsy and seizure disorders in Bradford. The method used was a community-based cross-sectional study. Case ascertainment was by review of medical records identified by searching practice databases using diagnostic codes and from repeat prescribing data. A specialist epilepsy nurse reviewed the records in 39 practices covering a population of 225 439. Clinical review was undertaken where there was limited information available in the records. In total, 1643 cases of epilepsy were identified (prevalence 7.3/1000). The prevalence of epilepsy increased with age. Classification of patients by internationally agreed definitions showed 1013 (62%) of cases with active epilepsy (prevalence 4. 5/1000); 421 (26%) with epilepsy in remission on treatment and 209 (13%) with epilepsy in remission off treatment. The standardized rate for all patients of South Asian origin was 3.6/1000 compared to 7.8/1000 in the rest of the population (OR 0.46; 95% CI 0.38, 0.57). An underlying aetiology was identified in 29.5% of cases. Cerebrovascular disease and head injuries were the commonest causes. CONCLUSIONS: The prevalence of epilepsy supports previous epidemiological estimates and highlights the public health importance of epilepsy. The low prevalence in the South Asian population is a surprising finding and further research into cultural attitudes to epilepsy and the impact of stigma in the South Asian population in the UK is required.

Adolescent↗

A systematic overview--a decade of research'. The information and counselling needs of people with epilepsy.

This paper explores the background to epilepsy in terms of medical impact and psychosocial effects. The argument that information and counselling may be central to the person with epilepsy is explored. The evidence from primary research published between 1990 and 2000 investigating the information and counselling needs of people with epilepsy is appraised and synthesized. This paper seeks to answer the following questions: What are the information and counselling needs of people with epilepsy? What are the preferred formats, timing and delivery of information and counselling? What are the outcomes of information giving and counselling for people with epilepsy? The review suggests that there are unmet needs for personal and general information about epilepsy which may include individual or group education and counselling. Information related to gaining control for people with epilepsy and targeted public education may contribute to improved quality of life for people with epilepsy. Information is required which is individually relevant and could be delivered in small groups or as part of an individual counselling service. Specialist epilepsy clinics and specialist nurses can improve patient knowledge and communication and provide an effective and high quality service for people with epilepsy.

Counseling↗

Epilepsy prevalence in a rural area in Istanbul.

This study is a field study with cross-section and case control conducted in the Kucukcekmece Region of Istanbul between June 1999 and February 2000. Four hundred and ninety three dwellings selected by a simple random sampling method were visited. From a population of 2187, 58 people, after filling in a questionnaire were suspected to have epilepsy. Following an interview, neurological examination and an electroencephalogram (EEG) 17 were diagnosed as having epilepsy. Lifetime epilepsy prevalence was 0.8%. 41.2% of the 17 epilepsy cases had partial epilepsy ( n= 7), 47.0% had generalized (n = 8) and in 11.8% seizures could not be classified ( n= 2). The risk factors for epilepsy in the control group ( n= 125) from the same region and those with epilepsy were investigated by means of logistic regression analyses. Educational status (odds ratio : 1.82, 95% confidence interval : 1.13-2.94; P= 0.01), profession (OR : 0.76, 95% CI : 0.60-0.97; P= 0.03), history of epilepsy in the family (OR : 0.67, 95% CI : 0.47-0.94; P= 0.02) were determined to be correlated with epilepsy. The results have drawn attention to the fact that individuals should be informed about epilepsy.

Adolescent↗

Epilepsy surgery in children with developmental disabilities.

Surgery for treatment of medically uncontrolled epilepsy in children is now widely accepted with reported outcomes similar to those in adults. Epilepsy is reported in 8.8% to 32% of children with mental retardation (MR) and in up to half of children with severe retardation. There has been concern that patients with low IQ will experience unsatisfactory outcomes from epilepsy surgery and not achieve good seizure control. It is appropriate to reassess the prior bias against resective epilepsy surgery in children with MR in view of the changing criteria for potential candidacy for epilepsy surgery in infants and young children. There are three prerequisites for epilepsy surgery: (1) the epilepsy must be medically intractable; (2) the surgery must be feasible, that is, the epileptogenic zone can identified and safely resected; and (3) there is high likelihood of a satisfactory outcome as regards both the epilepsy and the patient's functional status. Patients with MR may have diffuse cerebral dysfunction and diffuse or multifocal epileptogenic regions. Appropriate patient selection is made possible through use of current technology that allows identification of lesions or areas of cerebral dysgenesis, aiding in identification of localized areas of epileptogenesis. Results from various series of patients with MR who have undergone resective surgery for epilepsy have shown that with careful presurgical evaluations, outcomes are similar between patients with normal IQ scores and those with low scores. Surgical protocols specifically for patients with MR and intractable epilepsy are required, including careful definition of desired outcomes.

Child↗

A clinical comparative study evaluating the effect of epilepsy versus ADHD on timed cognitive tasks in children.

Both children with epilepsy and children with ADHD may be characterized by slowing on reaction-time measurement. This is of particular interest, as neuropsychological assessment is often requested in the differential diagnosis between children with short non-convulsive epileptic seizures and children with ADHD. In this study we attempt to identify patterns of impairment on timed tasks that are specific for epilepsy, relative to ADHD. This study was an open, controlled parallel-group clinical investigation which included two groups of patients: 60 children with ADHD and 60 children with epilepsy. These children were compared with a control group (n=30) on two types of timed cognitive tasks: tasks with low information load (simple reaction-time measurement) and tasks with high information load (multiple decision reaction-time measurement). The simple reaction-time measurements show significant differences between ADHD and controls (all except for visual RT non-dominant hand) and between epilepsy and controls (only one test). No significant differences were found between epilepsy and ADHD. The two tests with high information load show significant slowing compared with the controls for epilepsy on the Binary Choice Reaction-Time Test and for ADHD on the Visual Searching Test. On both tests also the differences between epilepsy and ADHD are significant. The two tests in combination have a relatively satisfactory potential to classify the children with ADHD (75% correct classification) and the children with epilepsy (55% correct classification). We may conclude that complex reaction-time tests (i.e., timed tasks with high information load) have potential for assessing the differential impact of ADHD and epilepsy on attentional function. These tasks specifically reveal general slowing for children with epilepsy and slowing as an effect of failures of inhibitory self control on unstructured tasks for ADHD.

Attention↗

Predictors of sleepiness in epilepsy patients.

Sleepiness, a common complaint of epilepsy patients, is frequently attributed to antiepileptic medications. To determine predictors of subjective sleepiness in epilepsy patients, we gave self-administered, validated surveys of sleepiness [Epworth sleepiness scale (our major outcome measure)] and sleep apnea [sleep apnea scale of the sleep disorders questionnaire (SA/SDQ)] to 158 epilepsy patients and 68 neurology patients without epilepsy (controls). An elevated Epworth score (>10) was more likely in epilepsy patients compared to controls after controlling for age and gender (p < 0.05). When Epworth scores were adjusted for SA/SDQ scores and restless legs symptoms (RLS), however, epilepsy patients showed only a nonsignificant trend toward elevated Epworth scores compared to controls (p = 0.08). SA/SDQ scores (p < 0.005) and RLS (p < 0.007) were significant predictors of elevated Epworth score in both epilepsy patients and controls. Among the epilepsy patients, the number or type of antiepileptic medication, seizure frequency, epilepsy syndrome (partial vs. generalized), and the presence of sleep-related seizures were not significant predictors (p > 0.10) of elevated Epworth score. Before attributing sleepiness in epilepsy patients to antiepileptic medications or uncontrolled seizures, clinicians should consider the possibility of a coexisting sleep disorder.

Adolescent↗

Genetics of the epilepsies.

PURPOSE OF REVIEW: This article reviews the most significant advances in the field of genetics of the epilepsies during the past year, with emphasis on newly identified genes and functional studies leading to new insights into the pathophysiology of epilepsy. RECENT FINDINGS: Mutations in the chloride channel gene CLCN2 have been associated with the most common forms of idiopathic generalized epilepsies. A mutation in the ATP1A2 sodium potassium ATPase pump gene has been described in a family in which familial hemiplegic migraine and benign familial infantile convulsions partly co-segregate. The leucine-rich, glioma-inactivated 1 gene (LGI1) (also known as epitempin) was found to be responsible for autosomal-dominant lateral temporal lobe epilepsy in additional families. The serine-threonine kinase 9 gene (STK9) was identified as the second gene associated with X-linked infantile spasms. Mutations in the Aristaless-related homeobox gene (ARX) have been recognized as a cause of X-linked infantile spasms and sporadic cryptogenic infantile spasms. A second gene underlying progressive myoclonus epilepsy of Lafora, NHLRC1, was shown to code for a putative E3 ubiquitin ligase. SUMMARY: Genes associated with idiopathic generalized epilepsies remain within the ion channel family. Mutations in non-ion channel genes are responsible for autosomal-dominant lateral temporal lobe epilepsy, a form of idiopathic focal epilepsy, malformations of cortical development, and syndromes that combine X-linked mental retardation and epilepsy. Most genetic epilepsies have a complex mode of inheritance, and genes identified so far account only for a minority of families and sporadic cases. Functional studies are leading to a better understanding of the mechanisms underlying hyperexcitability and seizures.

Carrier Proteins↗

Channelopathies as a genetic cause of epilepsy.

PURPOSE OF REVIEW: This review describes the significant number of new gene associations with epilepsy syndromes that have emerged during the past year, together with additional mutations and new electrophysiological data relating to previously known gene associations. RECENT FINDINGS: Autosomal dominant juvenile myoclonic epilepsy was demonstrated to be a channelopathy associated with a GABA(A) receptor, alpha1 subunit mutation. Benign familial neonatal infantile seizures were delineated as another channelopathy of infancy, by molecular characterization of sodium channel, alpha2 subunit defects. A sodium channel, alpha2 subunit defect was previously found to be associated with generalized epilepsy with febrile seizures plus. Similarly, the clinical spectrum associated with potassium channel, KQT-like mutations was extended to include the channelopathy myokymia and neonatal epilepsy. Mutations in the non-ion channel genes, leucine-rich, glioma inactivated 1 gene and Aristaless related homeobox gene, have emerged as important causes of their specific syndromes, with mutations in the latter gene frequently underlying X-linked mental retardation with epilepsy. SUMMARY: All but one of the idiopathic epilepsies with a known molecular basis are channelopathies. Where the ion channel defects have been identified, however, they generally account for a minority of families and sporadic cases with the syndrome in question. The data suggest that ion channel mutations of large effect are a common cause of rare monogenic idiopathic epilepsies, but are rare causes of common epilepsies. Additive effects of genetic variation, perhaps within the same ion channel gene families, are likely to underlie the common idiopathic generalized epilepsies with complex inheritance. The genetics of epilepsy is progressing rapidly toward a more detailed molecular dissection and definition of syndromes.

Calcium Channels↗

Sudden unexpected death in epilepsy: from mechanisms to prevention.

PURPOSE OF REVIEW: To discuss the pathophysiology and potential prevention of sudden unexpected death in epilepsy. RECENT FINDINGS: Long-term electrocardiogram monitoring over several months has detected ictal asystole in three out of 20 (15%) patients with refractory epilepsy, suggesting that high-risk ictal arrhythmias occur in a greater proportion of patients with refractory epilepsy than previously thought. In case-control studies, sudden unexpected death in epilepsy was found to be associated with frequent generalized tonic-clonic seizures and greater ictal maximal heart rate, especially during nocturnal attacks. Conversely, supervision at night was associated with a lower risk of occurrence. The impact of epilepsy surgery on the risk of death and sudden unexpected death in epilepsy remains unclear, with comparable long-term survival in an epilepsy surgery cohort compared with a matched population of patients with refractory epilepsy who did not undergo surgery. Previous results may have been partly confounded by the association observed between preoperative decreased heart rate variability and poor postoperative seizure outcome. SUMMARY: Ictal arrhythmias may represent a more prevalent cause of sudden unexpected death in epilepsy than previously thought. No clear recommendations have emerged from the literature regarding the most appropriate therapeutic strategies to prevent the event, apart from the supervision at night of patients with refractory epilepsy.

Cause of Death↗

Cost of epilepsy in patients attending a secondary-level hospital in India.

The study objective was out to provide an estimate of cost of epilepsy in a secondary level hospital in northern India where a once a week epilepsy clinic is run. Cost data were based on existing information on costs of the hospital and market rates for drugs and investigations. Other necessary information was extracted from patient records for the year 2001. Both direct (consultation fees, cost of investigation, drugs and facility costs) and indirect (traveling and loss of productivity) were estimated. A 25% loss of productivity was assumed based on interviews with the epilepsy patients attending the clinic. There were a total of 184 patients attending the epilepsy clinic during the year 2001. The annual drug cost of epilepsy treatment using phenobarbitone was 11 US dollars. The cost of drugs was in the ratio 1:2:3:4 for phenobarbitone, phenytoin, carbamazepine and sodium valproate. The average annual cost of outpatient treatment of epilepsy was found to be 47 US dollars per patient. The annual cost incurred in emergency and inpatient management was estimated at 810.50 US dollars and 168.30 US dollars for all the patients attending the secondary hospital during the year 2001. The total annual treatment cost for patients attending the hospital was 11,470 US dollars. The annual productivity loss for the same patients was estimated at 20,475 US dollars. Applying these to the estimated 5 million epilepsy patients in India, it comes to about 0.2% of the GNP of the country. As disease cost is much lower than productivity loss, epilepsy treatment is a worthwhile investment for the society. Treating epilepsy patients at primary level using phenobarbitone will increase the treatment coverage and reduce treatment costs. Simultaneous efforts must be made to bring the epilepsy patients on mainstream so as to reduce the productivity loss.

Anticonvulsants↗

Intellectual disability and psychotic disorders of adult epilepsy.

PURPOSE: To investigate the prevalence, psychopathology, and cognitive functions associated with psychotic disorders among adult epilepsy patients with intellectual disability (ID) based on a multicenter study in Japan. METHODS: The study was divided into three phases: a prevalence study of psychotic disorders among new referrals of epilepsy, a polydiagnostic comparative study of patients with psychotic epilepsy and those with schizophrenia, and a neuropsychological study of patients with psychotic epilepsy and education level-matched controls. RESULTS: Among 336 new referrals of epilepsy, a higher prevalence of psychotic disorders was found among patients with ID (24%) than among those with normal intelligence (6%). The psychotic symptoms and operational diagnoses of psychotic epilepsy patients with ID were similar to those of patients with normal intelligence. A wide range (7-86%) of psychotic epilepsy patients was diagnosed as having schizophrenia, depending on the operational criteria used. Patients with psychotic epilepsy had more disturbances in verbal memory and attention functions than controls. CONCLUSIONS: Epilepsy patients with ID show a predisposition to develop psychotic disorders. Distinguishing their psychotic symptoms from those of schizophrenia is difficult. Subtle cognitive disturbances predispose to psychotic disorders in epilepsy.

Adult↗

Public attitudes toward epilepsy in Denmark.

Social acceptance of persons with epilepsy very often constitutes a considerable problem for patients and their relatives. Nationwide opinion pools on the knowledge of and public attitude toward epilepsy have been taken in several countries, but never in Denmark. We report a Gallup survey of the general knowledge of and attitude toward epilepsy. A representative population of 1,500 persons aged greater than or equal to 15 years was selected in a four-state proportional sampling procedure. Ninety-seven percent of respondents had heard or read about epilepsy, 60% of these knew a person with epilepsy, and 50% had seen an epileptic seizure. The attitudes toward social acceptance and employment of persons with epilepsy were generally favorable, but 7% had objections to social contact between their children and persons with epilepsy in the playground and at school and 7% had objections to equal employment. Familiarity with persons with epilepsy was correlated to questions about attitudes and general knowledge of epilepsy. Such knowledge and public attitude in Denmark are mainly positive, but we believe that a continuous information campaign about epilepsy is essential, especially among youth.

Adolescent↗

Clinical indicators of genetic susceptibility to epilepsy.

We evaluated clinical indicators of genetic susceptibility to epilepsy in the families of 1,957 adults with epilepsy (probands) ascertained from voluntary organizations. Very few of the probands in this series had idiopathic epilepsy syndromes. Among relatives of probands with postnatal CNS insults, risks of epilepsy were no higher than in the general population. Risk was increased in relatives of probands without identified CNS insults (i.e., those with idiopathic/cryptogenic epilepsy) or with neurological deficit presumed present at birth, compared with relatives of probands with postnatal CNS insults. Among relatives of probands with idiopathic/cryptogenic epilepsy, risks were higher in parents and siblings, but not in offspring, of probands with generalized onset as compared with partial onset seizures. Risks in offspring were higher if the probands had onset of idiopathic/cryptogenic epilepsy before age 10 as compared with age > or = 10 years, but risks in parents and siblings were not associated with the probands's age at onset. These results suggest that genetic susceptibility increases risk of some forms of cryptogenic epilepsy and of epilepsy associated with neurological deficit presumed present at birth, but not of postnatal symptomatic epilepsy. The influences on risk in offspring may differ from those in parents and siblings.

Adolescent↗

Antiepileptic drug therapy: when is epilepsy truly intractable?

We define intractable in the first 5 years of epilepsy treatment as an average of at least one seizure every 2 months. For the longer term, we define intractable as at least one seizure per year. Population studies from Chicago, IL, U.S.A., Finland, and Nova Scotia, Canada indicate that with long follow-up, many children with intractable epilepsy eventually have remission of their seizure disorder. Epilepsy is no longer intractable when the seizures stop completely. How often does a new antiepileptic drug (AED) render a child seizure-free when one or more AEDs have failed? Literature on adults with epilepsy suggests that few with chronic epilepsy who have not achieved seizure control with several AEDs will achieve complete seizure control with additional AEDs. The Nova Scotia study suggests that if a child's seizure fails to be controlled with a first AED, there is an increased risk of intractable epilepsy. Nonetheless, the chance of eventual, complete remission of epilepsy (seizure-free without AED treatment) is approximately 40%. We conclude that intractability should not be considered until there has been failure of at least three first-line AEDs. Intractable epilepsy is rare. Careful definition of the characteristics of children with intractable epilepsy who do respond completely to new AEDs will likely provide the only rational approach to treatment of children with three drug failures. Collaboration by multiple epilepsy centers will be required to gain this information.

Adult↗

Prevalence, classification, and severity of epilepsy and epileptic syndromes in children.

PURPOSE: To determine the point prevalence of active childhood epilepsy in a defined area and evaluate the usefulness of ILAE classification of seizures, and epilepsies/syndromes with special interest in severe epilepsies. METHODS: By using the latest ILAE International Classification of Epileptic Seizures (ICES, 1981) and Epilepsies and Epileptic Syndromes (ICE, 1989), we determined the age- and sex-specific prevalence rates of epilepsy, type of seizures, epilepsies, and recognizable epileptic syndromes, as well as the proportion of severe cases in each seizure/epilepsy/syndrome category in all children 0-15 years of age from a geographically defined area in Finland. All medical records, neurophysiological recordings and available clinical data were reviewed retrospectively. RESULTS: Point prevalence of active epilepsy on December 12, 1992 was 3.94 per 1,000. According to ICES/ICE, we were able to classify 96% of seizures and 90% of epilepsies and syndromes. Generalized seizure and epilepsy/syndrome types were more prevalent in children 0-6 years of age and partial/localization-related in children 6-15 years of age. Epilepsy was intractable in 17% of all cases and correlated significantly with symptomatic etiology and early onset of epilepsy, as well as with additional neuroimpairments. CONCLUSIONS: A considerable number of cases fell into the nonspecific categories of ICE, which limits the value of present epilepsy/syndrome classification in terms of prognosis, prediction, and indication for special investigations in individual cases. A number of intractable cases was relatively low, indicating good prognosis in many childhood epilepsies, especially when additional neuroimpairments are absent.

Adolescent↗