Global disparities in the recognition, diagnosis, and management of seizures or epilepsy of autoimmune etiology.

S179. The incidence of first seizures, new diagnosis of epilepsy and seizure mimics in county cork, Ireland; an Epidemiologic protocol and interim report

BackgroundEpilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition [1]. Studies...

ILAE President's midterm report

Studies adherent to international guidelines and epilepsy classification are needed to accurately record the incidence of isolated seizures, epilepsy and seizure-mimics within a population. Because the diagnosis of epilepsy is largely made through clinical assessment by experienced physicians, seizures and epilepsy are susceptible to misdiagnosis. Previous epidemiological studies in epilepsy have not captured “seizure mimics”. We therefore sought to quantify the incidence of isolated seizures, epilepsy and seizure-mimics using the International League Against Epilepsy (ILAE) classification system. In this study multiple overlapping methods of case ascertainment were applied to a defined geographic region from January 1 to March 31, 2017 to identify all patients presenting with first seizures (provoked and unprovoked), new diagnoses of epilepsy and seizure mimics. Over a 3 month period, from a population of 542,869 adults and children, 442 potential presentations were identified, and 283 met the inclusion criteria. Radiology databases were the source of the largest number of individual cases (n = 153, 54%), while electroencephalogram (EEG) databases were the source of the highest number of unique-to-source cases (those not identified elsewhere, n = 60, 21%). No single case was picked up in every method of ascertainment. Among the 283 included presentations, 38 (13%) were classed as first provoked seizures, 27 (10%) as first unprovoked seizures, 95 (34%) as new diagnosis of epilepsy and 113 (40%) as seizure mimics. Ten (3%) presentations were indeterminate. We present and apply a rigorous study protocol for investigation of the incidence of first seizures, new diagnosis of epilepsy and seizure mimics in a geographically defined region which is adherent to recently published international guidelines for epidemiologic studies and epilepsy classification. We highlight the challenges in making a diagnosis of new-onset epilepsy in patients presenting with a first seizure using the current ILAE definition of epilepsy, when epilepsy can be diagnosed in situations where the treating physician anticipates the risk of further seizures exceeds 60%.

There’s mixed news for the diagnosis and management of epilepsy in nursing homes, where the prevalence of the disorder is estimated to be more than seven times higher than among seniors in the community. Generally speaking, for instance, the main evidence-based guideline on management of an unprovoked first seizure in adults — published by the American Academy of Neurology and American Epilepsy Society (Neurology 2015;84:1705–1713) — is applicable to the nursing home population. That’s good news, as is the development of a broader and “practical” definition of epilepsy by the International League Against Epilepsy (ILAE), two epilepsy specialists said at the Annual Conference of AMDA – The Society for Post-Acute and Long-Term Care Medicine. What is vexing — and what represents a “huge hole” for clinicians caring for nursing home residents with epilepsy — is the lack of evidence to guide the appropriate use of antiseizure medications in this population, the speakers said. “I don’t necessarily have evidence that any of [newer] drugs are actually really better than others,” said Ilo Leppik, MD, during a question-and-answer session that focused largely on drug choice and questions about therapeutic monitoring. “Levetiracetam and lamotrigine appear to be better for the elderly, especially in nursing homes, but we just don’t have the data.” The 2015 guideline on management of an unprovoked first seizure does not discuss drug choice but rather decision-making about whether to initiate therapy immediately. Level A evidence supports that recurrence risk is greatest early within the first two years (21% to 45%), the guideline states. Clinical variables associated with increased risk include a previous brain insult, including stroke (level A); an electroencephalogram with epileptiform abnormalities (level A); a significant brain-imaging abnormality (level B); and a nocturnal seizure (level B). “Immediate antiseizure therapy compared with a delay pending a second seizure is likely to reduce the recurrence risk for the first two years but may not improve the quality of life due to medication side effects,” said Dr. Leppik, professor of neurology and pharmacy at the University of Minnesota, in reviewing the guideline. He said that, interestingly enough, immediate treatment is unlikely to improve the longer-term (>3 years) prognosis for seizure remission. The guideline is not as applicable to the nursing home population when it comes to adverse event risks. The guideline reports a risk of drug-related adverse events of 7% to 31% and advises that the effects are predominantly mild; however, Dr. Leppik said, “in the nursing home the side effects may be more than mild because of preexisting issues with cognition.” Levetiracetam is probably the “most widely used antiseizure medication for the elderly, including in my practice,” Dr. Leppik said during the question-and-answer session. The drug has “definite advantages ... but it causes the most issues with behavior. For that reason alone, I’m not convinced it’s the best drug to use in the nursing home setting.” Rebecca O’Dwyer, MD, assistant professor in the Department of Neurological Sciences at Rush Medical College in Chicago, agreed that levetiracetam’s side effect profile is concerning — particularly, based on her experience, in patients with frontal lobe epilepsy. “It’s helpful to know where the seizures are coming from,” she said, noting that she also often uses lamotrigine. “It’s anecdotal, but I sometimes feel that people with temporal lobe epilepsy are less susceptible to those negative side effects [of levetiracetam].” When asked about newer agents such as eslicarbazepine acetate (Aptiom) and lacosamide (Vimpat), Dr. Leppik cautioned that “industry is really pushing Aptiom for the elderly, but it’s in the family of carbamazepine and oxcarbazepine, and all three of these drugs have a propensity for lowering sodium levels.” Therapeutic drug monitoring in the elderly requires a high level of individualization, Drs. Leppik and O’Dwyer emphasized. Asked about the importance of titrating antiseizure agents based on blood levels versus on seizure control, Dr. Leppik cautioned that “therapeutic range is a lab range value and can be very misleading” in the elderly population. “It needs to be individualized,” he said. “We know from experience that the elderly generally need lower blood levels because, for one, their seizures seem to better controlled [with lower levels], and secondly they seem to get side effects at lower levels than other adults.” Dr. Leppik said he was securing funding for a national survey of medical directors to learn more about how new-onset epilepsy is managed in the nursing home. His prior research has helped define the epidemiology of epilepsy in the nursing home population. In 2017, Dr. Leppik and his coauthors reported in an analysis of all residents in all Medicare/Medicaid-certified nursing homes that the point prevalence of epilepsy/seizures was 7.7%, and that prevalence is 7 to 30 times higher in individuals with certain comorbid neurologic conditions. (Neurology 2017;88:750–757). Christine Kilgore is a freelance writer based in Falls Church, VA.

Aetiologies of first-ever convulsive seizures may be diverse, not all leading to recurrence or epilepsy diagnosis. We aimed to describe the epidemiology of first-ever convulsive seizures in children, investigating risk factors for recurrence and epilepsy diagnosis. This was a retrospective study of children presenting with a first-ever convulsive seizure to a tertiary-care paediatric emergency department (PED) in Italy, in a 12-month period (2011-2012). One hundred and eight children (57 males, 51 females) presented to the PED for a first-ever convulsive seizure; 90.7% were 6months to 6years old (median age 1y 10mo, mean 2y 7mo, range 0mo-14y 4mo). Seizure duration was less than 5minutes in 76.8%. Seizures were 'unprovoked' in 19.4% and 'provoked' in 80.6%. At 4-year follow-up, 37.9% of patients experienced recurrence and 13.6% received a diagnosis of epilepsy. Factors significantly associated with recurrence were the 'unprovoked' nature of the first seizure, multiple seizures in the first 24hours, positive family history of febrile seizures or epilepsy, and pre-existing neurological conditions/problems. Factors significantly associated with a diagnosis of epilepsy were the 'unprovoked' nature of the first seizure, age older than 6years, pre-existing neurological conditions/problems, and focal onset of first seizure. Children presenting to the PED with first-ever convulsive seizures represent a heterogeneous group. The identification of prognostic factors for recurrence and epilepsy diagnosis may help provide tailored counselling and follow-up. Seizures were 'unprovoked' in 19.4% and 'provoked' in 80.6% of children presenting to the emergency department. At 4-year follow-up, 37.9% relapsed, and 13.6% received a diagnosis of epilepsy. 'Unprovoked' first seizure, family history of febrile seizures, and pre-existing neurological conditions were associated with recurrence. 'Unprovoked' first seizure, age younger than 6years, and pre-existing neurological conditions were associated with epilepsy diagnosis.

In July 2009, the International League against Epilepsy (ILAE) developed its four-year strategic plan in collaboration with past, current, and future leaders (www.ilae-epilepsy.org). This is the first yearly progress report, prepared by the management committee and the chair of the Strategic Task Force, to highlight progress toward achieving the plan’s goals.

The International League Against Epilepsy (ILAE) created the ILAE Neonatal Task Force that classified neonatal seizures, defined neonatal epilepsy syndromes, and specified treatment guidelines. These frameworks, in addition to improved access to genetic testing and other recent advances, have revolutionized the diagnosis and management of neonatal seizures. PLAIN LANGUAGE SUMMARY: Neonatal seizures, occurring within the first 4 weeks of life, present unique challenges due to the immature neonatal brain. The International League Against Epilepsy (ILAE) Neonatal Task Force revolutionized diagnosis and management through classification, syndrome definition, and treatment guidelines. Despite advancements, challenges persist, including accurate diagnosis and limited evidence-based treatment protocols, emphasizing the need for global efforts to standardize care.

Sunday, December 3, 2006 Poster Session II 7:30 a.m.–4:30 p.m.

In July 2009, the International League Against Epilepsy (ILAE) developed its 4-year strategic plan. This is the second-year progress report, prepared by the management committee and the chair of the Strategic Task Force, with input from several key leaders, to highlight progress toward achieving the plan’s goals. Additional information regarding our activities can be found at our renovated website (http://www.ilae-epilepsy.org). As the major international epilepsy organization, ILAE is taking the lead in developing practice guidelines for clinical questions faced by clinicians who are managing people with epilepsy. A new Task Force on Guidelines, chaired by Tim Pedley, has been appointed. The aims are the following: to compile and review existing epilepsy guidelines; to identify those guidelines that can be adopted worldwide or in specific parts of the world; to assess the needs of the League’s chapters around the world; to identify the most important areas for new guideline development; and to determine whether a topic or question is best served by a formal clinical practice guideline (which may be hindered by the paucity of methodologically rigorous evidence) or by a position paper that represents the consensus of experts supported by the available evidence. Because the development of guidelines is time-consuming and expensive, the League may partner in this project with organizations such as the World Federation of Neurology (WFN), the American Academy of Neurology (AAN), and the European Federation of Neurological Societies (EFNS). To succeed, the League will seek the help of many volunteers and provide necessary staff support. The Epidemiology Commission and Marco Medina continue their work with the World Health Organization (WHO) to develop the epilepsy-related codes for the ICD-11. The League’s website has been redesigned. Jean Gotman, our Web and Media Director, and Ed Bertram, our Information Officer, are hiring a Web manager. Each Commission has the responsibility to oversee the content of its section and to create links to ILAE activities, including Long Distance Education and Global Outreach. A Web Editorial Board tasked to provide advice on development and use of the website has been appointed. The new web-based social networking Community Suite (Higher Logic) for ILAE groups is now fully operational. Plans are progressing to provide our Chapters access to ILAE Social Media. The League has made arrangements with Wiley-Blackwell to make Epilepsia available online at no charge to our constituencies in resource-poor countries that belong to the Hinari program. In addition, the League is discussing with Wiley-Blackwell the creation of a sister online journal to accommodate the needs of our constituents, while maintaining a high impact factor for Epilepsia. Plans are underway for appointment of the next Editors-in-Chief. By 2013, Phil Schwartzkroin and Simon Shorvon will have completed 8 years of service in this capacity, which is the maximum allowed by the constitution. We anticipate that the new Editor(s) will be appointed by January 2013 and will begin their tenure with the new Executive in 2013. The 29th International Epilepsy Congress (IEC) was held in Rome August 28th to September 1, 2011. Nearly 4,000 delegates attended this highly successful Congress. Its program aimed at integrating all aspects of epilepsy (including medical, research, social, and novel therapeutic approaches) in each session. A prime example was the Presidential symposium entitled “Avoiding Deaths in Epilepsy,” During the 29th Congress, the International Epilepsy Bureau (IBE) celebrated its 50th anniversary with two exceptional events: A get-together of the Ambassadors of Epilepsy, a highly prestigious honor initially conceived by IBE and now conferred by joint deliberations of the IBE and ILAE Executive Committee every 2 years; and a magnificent concert. Work is ongoing for the preparation of four Regional Congresses scheduled for 2012, including the 9th Asian-Oceanian Epilepsy Congress in Manila (21–25 March 2012), the 10th European Congress of Epileptology in London (30 September–4 October 2012), the Latin American Epilepsy Congress in Quito (14–17 November 2012), and the African Epilepsy Congress in Nairobi (21–23 June 2012). The latter will be the first African Congress organized jointly by the ILAE and the IBE and represents a major component of the educational activities targeted to the African continent by our newly formed Commission on African Affairs. The 30th IEC will be held in Montreal, Canada on June 23–27, 2013. During the 29th International Congress held in Rome, Dr. Jerome Engel Jr. received the Life Achievement Award and Dr. Pravina Shah received the Social Accomplishment Award. Dr. Eleonora Aronica was the Michael Prize winner and Dr. Laura Jansen was the recipient of the Morris-Coole Epilepsia Award. Twelve new Ambassadors for Epilepsy were elected (Table 1). The regional commissions are establishing bi-annual awards that recognize regional accomplishments. The Commission on European Affairs (CEA) established the European Epileptology Award in 2002. The latest awards were bestowed on Drs. Yehezkiel Ben-Ari and Martin Brodie during the 9th European Congress in Rhodes in 2010. The CEA plans to expand the number of awards starting in 2012. During the 2012 London Congress there will be up to two European Research (clinical and basic) Awards for Young investigators: one for Education and one for Service to Europe. The Asian-Oceanian Commission granted their first awards during the 8th Asian Oceanian Epilepsy Congress (AOEC) in 2010. The recipients of the Asian and Oceanian Outstanding Achievement Epilepsy Awards included Drs Hasan Aziz, Tatsuya Tanaka, Kazuichi Yagi, P. Satish Chandra, and Xun Wu. The League solidified the organizational and financial stability of the long-distance education program by acquiring direct supervision of this initiative, and by investing in the expansion of the Virtual Epilepsy Academy (VIREPA) to promote teaching courses under the coordination of regional academies. To this end, the Education Commission through its Task Force on Distance Education, led by Walter van Emde Boas, has instituted approaches to increase the attractiveness and accessibility of ongoing courses, and has added new courses on pediatric epilepsies and electroencephalography (EEG), psychiatric aspects, and sleep and epilepsy. Moreover, plans are underway to organize a course for primary caregivers in Sub-Saharan Africa in collaboration with the Task Force on Seizures and Epilepsy in the Tropics, the African Commission, and the WHO. The Education Commission led by CT Tan is developing ways to make educational material generated during our congresses freely available through the League’s website. This was started with a trial run of voice/power point slides captured during the Melbourne AOEC, and the “How to” education session during Rome IEC. In Rome, through an effort coordinated by our Information Officer, many presenters addressing timely topics were also interviewed to produce videos, which are currently posted on the League’s website. This will be a regular feature of future congresses. The Education Commission is creating an open access, online Textbook of Epilepsy (Shi Hui Lim, Editor), housed at the ILAE website. We expect the first edition to be available in the first quarter of 2012. We plan translations of the textbook into Spanish and Chinese. Congresses are being reorganized to promote translational research venues that allow for better communication between basic scientists and clinicians. The Neurobiology and the Education Commissions are developing proposals for further training of professionals, including a combination of residential courses with hands-on tutoring of research proposals that are likely to impact resource-poor regions favorably. This initiative may include guidance in preparing grant applications, provision of seed grants, and monitoring research progress. The Academies of the Regional Commissions have developed year-round, 1–2 day teaching courses in various parts of the regions. For example, the Asian Epilepsy Academy (ASEPA) organizes about 10 such courses yearly. These courses reach out to diverse populations, address different needs, and are generally more cost-effective than traditional large congresses. The longer and more in depth “summer schools” continue to be organized in Europe and Latin America, and have also been launched in Asia. Other projects by the regional commissions/academies include visiting professorships between the North America and the Caribbean/Latin America; fellowships by the Asian and Latin American Commissions, EEG certification examination, and publication of congress proceedings by ASEPA. Vital partners in basic research collaborations are the International Brain Research Organization (IBRO) and the Society for Neuroscience. An important aim is to identify and publicize possible funding sources for such programs, and to increase public and government awareness of the needs of people with epilepsy. Discussion is underway with the WFN and the International Child Neurology Association (ICNA) to develop collaborative visiting professorships for Africa. The key aim is to create a long-term relationship between institutions. This initiative will involve the Education, North American, and African Commissions. Finally, three commission-related reports were published (Blümcke et al., 2011; Gaillard et al., 2011; Thurman et al., 2011). We continue our Global Campaign efforts together with IBE and WHO. Because several additional initiatives do not fall under the collaborative efforts of the Global Campaign, together with IBE we created the Global Outreach Task Force, chaired by Helen Cross and Shichuo Li with expert assistance from Hanneke de Boer. The primary aim of this task force is to raise awareness of global educational and service initiatives in epilepsy throughout the world. The Task Force has identified a new slogan, initially proposed by Mike Glynn, President of IBE: “Stand up for epilepsy.” The slogan is in the process of being registered. The second SUDEP, A Global Conversation book was launched in Rome 6 years after the first edition, with forewords by the ILAE and IBE Presidents. Much has changed since the earlier edition was launched at the Paris IEC, and almost all of it for the better in relation to epilepsy deaths. However, much remains to be done, especially in the area of prevention and with regard to people at risk of sudden unexpected death in epilepsy (SUDEP) being warned of it together with their loved ones. This book brings together contributions on the subject of SUDEP from all around the world including pieces from many developing countries. ILAE and IBE are engaged in several regional activities to highlight the public health dimensions of the epilepsies, and to prioritize allocation of resources to epilepsy health care and research. These initiatives include the production of an epilepsy report by the U.S. Institute of Medicine (IOM) (http://www.iom.edu/Activities/Disease/Epilepsy.aspx), the finalization of the Pan American Health Organization (PAHO) Strategy for the Americas, (http://new.paho.org/hq/index.php?option=com_content&task=view&id=5272&Itemid=3841&lang=en), and the support for the European Written Declaration on Epilepsy endorsed by the European Parliament (http://www.ilae-epilepsy.org/visitors/initiatives/EuropeanDeclaration.cfm). The IOM report will specifically address the following questions: How can the public health burden of epilepsy for patients and families be more accurately assessed? What priorities for future population health studies could inform treatment and prevention? How can the access to health and human services and the quality of care for people with epilepsy be improved? How can the education and training of professionals who work with people with epilepsy be improved? How can the understanding of epilepsy in patients and the general public be improved to create supportive communities? The report will be released in 2012. The 51st Directing Counseling of the Pan American Health Organization (PAHO; which includes representatives from countries of North, Central, and South America) approved and endorsed the Action Plan on Epilepsy for the Americas on September 29, 2011. The Action Plan was prepared by Dr. Jorge Rodriquez from PAHO and Drs. Carlos Acevedo and Marco Medina as part of the Global Task Force of the ILAE and IBE. The Action Plan highlights the problems associated with epilepsy care throughout the developed and developing countries of the Americas and lists specific goals and anticipated deliverables that would positively impact epilepsy care in the region, with a 10-year focus on epilepsy. In Europe, efforts are ongoing to advocate for political actions in the fight against epilepsy. The executive body of the European Union (EU) is its Commission. The two key Commissioners who can influence health care and research in epilepsy are European Commissioner John Dalli, Commissioner for Health and Consumer Policy, and European Commissioner Máire Geoghegan-Quinn, Commissioner for Research, Innovation and Science. During European Epilepsy Day, both Commissioners held meetings with delegations from IBE and ILAE led by the two Presidents at the European Parliament in Strasbourg. A Joint Task Force of ILAE and IBE, cochaired by Emilio Perucca and Mike Glynn and including members of the ILAE and IBE regional governing bodies (CAE and EREC respectively), has also been established under the name Epilepsy Advocacy Europe (EAE). EAE aims at making epilepsy care and research a priority in the agenda of the European Union (EU) and national governments in the European region. Its first action was to obtain the endorsement of the Written Declaration of Epilepsy by the European Parliament. This was achieved with a majority vote of the European Parliament on September 15, 2011. The Declaration calls for the EU to support research and innovation in the prevention and early diagnosis and treatment of epilepsy; to prioritize epilepsy as a major disease that imposes a significant burden of illness across Europe; to encourage Member States to ensure equal quality of life, including education, employment, transport, and public health care, for people with epilepsy; to encourage effective epilepsy health impact assessments on all major EU and national policies; and to introduce appropriate legislation to protect the rights of all people with epilepsy. The next steps will be to capitalize on the Declaration by ensuring that its recommendations are adopted by the European Commission and by national governments. A European Conference on Epilepsy is being planned with participation of all major stakeholders from the European Commission, the European Council, national governments, funding organizations, lay organizations, and the medical and research community. Our collaborative efforts are beginning to bear fruit. In November, 2010, the Colombian Congress passed a law establishing special measures of protection for people with epilepsy with principles and guidelines that call for comprehensive care of people with epilepsy. Five additional Task Forces have been established: The Seizures and Epilepsy in the Tropics Task Force (chair P. M. Preux), working in close collaboration with the Global Outreach Task Force, aims at addressing issues pertaining to seizures and epilepsy in the tropics, in particular as they relate to etiology, sociocultural aspects, and management. The Stigma Task Force (N. Jetté, chair) with support from the North American Commission, aims at determining ways to combat epilepsy-related stigma and its consequences worldwide. The Task Force on Sports and Epilepsy (G. Capovilla, chair), has two main aims: (1) to seek out and foster opportunities for interactions with major sports authorities and organizations to increase awareness about epilepsy worldwide; and (2) to increase participation of people with epilepsy in sports activities according to their capabilities. The Task Force on Preclinical Drug Discovery (M. Simonato and T. O’Brien, chairs) intends to develop standardized definitions, pathways, techniques, and endpoints for the discovery and preclinical development of new epilepsy treatments, and to enable a stronger evidence base to identify optimal candidates to take forward to clinical development. The Task Force on ILAE Reports (E. Bertram, chair) will develop guiding principles for writing reports generated by the ILAE, standardize processes for their internal and external review, and develop criteria for their designation as official position statements of the League. The Task Force on Sports and Epilepsy is launching an exciting project to create a collection of photographs of famous sports persons meeting people with epilepsy. The photographs will convey the message that people with epilepsy, like athletes themselves, can be inspired to achieve their goals and lead full and active lives. The collection is intended for publication as a book of photographs, and the images will be made available to National Chapters for advocacy initiatives in their own countries. The ILAE’s strategic plan includes a role as the global leader in epilepsy education. To this end, under the leadership of Ed Bertram, the League developed a series of short, patient-oriented videos dealing with common issues faced by patients (what is epilepsy?, symptoms, diagnosis, treatment, surgery, pregnancy, stigma, where to find treatment, causes, and prevention). Although most of the 3-min videos were designed for web use, two shorter videos with a clear concise message will be used for public news broadcasts (pregnancy and living with epilepsy). The League has undertaken a campaign to draw attention to the successes of members of our constituency and the importance of epilepsy. Letters have been written to appropriate authorities (deans and regional and national health ministers) about important contributions made by our colleagues (awards, organization of successful events, publication of important documents, elections to leadership positions, celebrations). Authorities will receive the message that epilepsy-related work provides much positive publicity for their institution or country and that epilepsy is an important health problem. We encourage further interaction with the national chapters in these initiatives. As with any such effort, the results can be slow in appearing and require regular reinforcement, but feedback from some members have suggested that these efforts have resulted in first contacts with health ministers as well as positive responses from deans. During the 29th IEC in Rome, the General Assembly ratified seven new National Chapters (Bolivia, Cameroon, El Salvador, Kosovo, Kuwait, Nigeria, and Sri Lanka), for a total of 108 active chapters. In addition, applications from 14 countries are being processed. The League’s secretariat is revising the rules for chapter incorporation to increase participation. Regional commissions are exploring ways in which isolated clinicians can easily interact with the League in countries and territories where formation of an ILAE Chapter is unfeasible. The new Constitution was ratified by the General Assembly at the 29th International Epilepsy Congress held in Rome on August 31, 2011 and is available on the website (http://www.ilae-epilepsy.org). It ensures that all regions have a voice and a seat in the Executive Committee. In 2013, the League will be electing its new officers. The dates for each step will be communicated to all chapters by the Chair of the elections committee, Past President Peter Wolf. The new Conflict of Interest policy is also available on the website. To enhance communication among commissions and with the leadership, a Commission Chair meeting is held annually. Here, the Regional and Task Oriented Commissions present their work and identify synergies for collaboration. The Commission Chairs will meet again in December 2011 to identify projects congruent with the 2009 ILAE Strategic Plan that can be completed in 2012 and 2013. In addition, the League’s leadership has asked Commissions to include plans to disseminate reports and activities to stakeholders and to implement action items. As discussed in the Year 1 report, steps have been taken to enhance the management of our financial resources and improve transparency in reporting and disclosing financial data. Our Finance Committee and Financial Advisory Subcommittee, which include members of the financial and corporate world, have worked effectively to this end. The most important product of these activities has been the finalization of a new Investment Policy intended to improve returns on the League’s investments while maintaining a conservative approach consistent with the organization’s nonprofit status and goals. One of the goals will be for the League to be able within the next 10 years to derive a sufficient return from investments to support activities at the current level, thereby compensating for a projected loss in revenue from congresses and other sources. The new Investment Policy also recognizes the international scope of our activities by making for in and world To this end, the Executive Committee endorsed the of Finance Committee and Financial Advisory to the management of the League’s to a new Financial with in at the international The of the latter will be according to Finally, the new Investment Policy recognizes of issues by direct investments into that and medical We again the many volunteers who their and toward the League’s strategic aims and to improve the of people with epilepsy throughout the world. We also our in Peter and in close collaboration with the and Glynn under the of International of We the advice and input we received from Ed Bertram, CT Walter Boas, Jean Gotman, Mike Glynn, Hanneke de Phil Simon and Tim in the preparation of this We also Phil Schwartzkroin and Simon Shorvon for has received research support from and the is on the Editorial Board of Neurobiology of Brain and and has received a from and a from received or research from and and research support from the of the for and Research, the and the European Commission of the also in the of Epilepsy Research, in in Drug in and Drug on for Epilepsy and the of Neurological and research support from the for Research and the Brain on the of of Epilepsy Neurology and Epilepsy and on the Committee of and received research support from and We that we have the position on issues in publication and that this report is consistent with those

Seizures are common in neonates, but there is substantial management variability. The Neonatal Task Force of the International League Against Epilepsy (ILAE) developed evidence-based recommendations about antiseizure medication (ASM) management in neonates in accordance with ILAE standards. Six priority questions were formulated, a systematic literature review and meta-analysis were performed, and results were reported following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) 2020 standards. Bias was evaluated using the Cochrane tool and risk of Bias in non-randomised studies - of interventions (ROBINS-I), and quality of evidence was evaluated using grading of recommendations, assessment, development and evaluation (GRADE). If insufficient evidence was available, then expert opinion was sought using Delphi consensus methodology. The strength of recommendations was defined according to the ILAE Clinical Practice Guidelines development tool. There were six main recommendations. First, phenobarbital should be the first-line ASM (evidence-based recommendation) regardless of etiology (expert agreement), unless channelopathy is likely the cause for seizures (e.g., due to family history), in which case phenytoin or carbamazepine should be used. Second, among neonates with seizures not responding to first-line ASM, phenytoin, levetiracetam, midazolam, or lidocaine may be used as a second-line ASM (expert agreement). In neonates with cardiac disorders, levetiracetam may be the preferred second-line ASM (expert agreement). Third, following cessation of acute provoked seizures without evidence for neonatal-onset epilepsy, ASMs should be discontinued before discharge home, regardless of magnetic resonance imaging or electroencephalographic findings (expert agreement). Fourth, therapeutic hypothermia may reduce seizure burden in neonates with hypoxic-ischemic encephalopathy (evidence-based recommendation). Fifth, treating neonatal seizures (including electrographic-only seizures) to achieve a lower seizure burden may be associated with improved outcome (expert agreement). Sixth, a trial of pyridoxine may be attempted in neonates presenting with clinical features of vitamin B6-dependent epilepsy and seizures unresponsive to second-line ASM (expert agreement). Additional considerations include a standardized pathway for the management of neonatal seizures in each neonatal unit and informing parents/guardians about the diagnosis of seizures and initial treatment options.

The main purposes of this study were to validate the epilepsy diagnosis in incident epilepsy cases in the Danish National Patient Registry (DNPR), which contains information on nearly 9 000 000 individuals, and to identify persons in the validated cohort who fulfilled the International League Against Epilepsy (ILAE) criteria for drug-resistant epilepsy (DRE). We reviewed a random sample of medical records from all individuals registered with a first diagnosis of epilepsy (International Classification of Diseases, 10th Revision [ICD-10]: G40) or seizures (ICD-10: G41, R56, or F445) in the Central Denmark Region from 2010 to 2019. In persons with a validated incident epilepsy diagnosis, we determined the proportion with DRE at the latest contact. We performed logistic regression analyses to identify clinical factors that correlated with risk of DRE. Of 20 723 persons with a first diagnosis of epilepsy (n = 11 812) or seizures (n = 8911), we reviewed the medical records of n = 1067 with incident epilepsy and n = 610 with incident seizures. Among those with a register diagnosis of epilepsy, the diagnosis was confirmed in 838 cases (45% females, mean age at onset = 42.4 years), providing a positive predictive value (PPV) of 79% (95% confidence interval [CI] = 76%-81%). The PPV of focal epilepsy was 86% (95% CI = 82%-89%), and the PPV of generalized epilepsy was 71% (95% CI = 61%-80%). Of 740 patients with confirmed incident epilepsy and ≥1 year of follow-up, 103 (14%) fulfilled the definition of DRE, 476 (64%) were drug responsive, and 161 (22%) had undefined responsiveness. In multivariable logistic regression analysis, early age at epilepsy onset, cognitive impairment, and a history of status epilepticus were associated with DRE. In the DNPR, we found a PPV of the epilepsy diagnosis of 79%. Among persons with confirmed epilepsy, 14% fulfilled ILAE criteria for DRE. Early age at epilepsy onset, cognitive impairment, and a history of status epilepticus were independently associated with drug resistance.

Diagnosis of Epilepsy

Background: Less than one-third of people with epilepsy will develop drug-resistant epilepsy (DRE). Establishing the prognosis of each unique epilepsy case is an important part of evaluation and treatment.Most studies on DRE prognosis have been based on a pooled, heterogeneous group, including children, adults, and older adults, in the absence of clear recognition and control of important confounders, such as age group. Furthermore, previous studies were done before the 2010 definition of DRE by the International League Against Epilepsy (ILAE), so data based on the current definitions have not been entirely elucidated. This study aimed to explore the difference between 3 definitions of DRE and clinical predictors of DRE in adults and older adults.Methods: Patients with a new diagnosis of epilepsy ascertained at a Single Seizure Clinic (SSC) in Saskatchewan, Canada were included if they had at least 1 year of follow-up. The first study outcome was the diagnosis of DRE epilepsy at follow-up using the 2010 ILAE definition. This was compared with 2 alternative definitions of DRE by Kwan and Brodie and Camfield and Camfield. Finally, risk factors were analyzed using the ILAE definition.Results: In total, 95 patients with a new diagnosis of epilepsy and a median follow-up of 24 months were included. The median age of patients at the diagnosis of epilepsy was 33 years, and 51% were men. In the cohort, 32% of patients were diagnosed with DRE by the Kwan and Brodie definition, 10% by Camfield and Camfield definition, and 15% by the ILAE definition by the end of follow-up. The only statistically significant risk factor for DRE development was the failure to respond to the first anti-seizure medication (ASM).Conclusion: There were important differences in the percentage of patients diagnosed with DRE when using 3 concurrent definitions. However, the use of the ILAE definition appeared to be the most consistent through an extended follow-up. Finally, failure to respond to the first ASM was the sole significant risk factor for DRE in the cohort after considering the age group.

1. Philippe Major, MD* 2. Elizabeth A. Thiele, MD, PhD* 1. *Department of Neurology, Massachusetts General Hospital, Boston, Mass After completing this article, readers should be able to: 1. Classify the different seizure types. 2. Distinguish seizures from other paroxysmal phenomena. 3. List the possible causes of seizures according to age and mode of presentation. Seizures are among the most common pediatric neurologic disorders. The overall prevalence of epilepsy is approximately 1%, and as many as 5% of all children experience febrile seizures before the age of 6 years. Seizures are caused by an abnormal and excessive discharge of neurons, usually accompanied by behavioral or sensorimotor manifestations. Epilepsy is defined classically as the occurrence of two or more unprovoked seizures. A seizure can be viewed as a symptom of an underlying central nervous system disorder that requires thorough evaluation and specific treatment. In addition to the consequences of the seizures, 50% of those who have epilepsy experience learning difficulties, and 30% to 50% have mental health and behavioral issues. In this first of two articles on seizures in children, we review the diagnosis and classification of seizures as well as possible causes of seizures in childhood. The second article, to be published in the November 2007 issue of Pediatrics in Review , focuses on the laboratory diagnosis and management of seizure disorders. The diagnosis of epilepsy and classification of specific seizure types are essential to determining a prognosis and choosing an appropriate treatment. The investigation of a child experiencing seizures begins with a medical history and physical examination. Although significant technologic advances have been made in electrophysiology and neuroimaging, the diagnosis of seizures and epilepsy remains largely clinical. Table 1 lists key features of the medical history and physical examination of a child presenting with paroxysmal events. | Questionnaire | || | |