The standardized incidence rate of SE in the French-speaking part of Switzerland was lower than that reported in Rochester, MN (18.3/100,000) and in the white population of Richmond, VA (20/100,000). The discrepancy may stem from the lack of a homogeneous, rigorous, and pragmatic definition of SE and the efficient management of acute repetitive seizures in this area.

Summary: Idiopathic generalized epilepsies (IGEs) are a relatively new category of disorders defined by strict clinical and electroencephalogram (EEG) features proposed by the International League Against Epilepsy (ILAE) classification of epileptic syndromes. IGEs are usually easy to diagnose when clinical and EEG data are collected, but epilepsy is not synonymous with epileptic syndrome. So far, IGEs are studied in the large group of epilepsies of undetermined or unknown etiology although the genetic origin is now largely accepted. ILAE-proposed criteria are helpful in the clinical and therapeutic management of IGEs, but many epidemiologic studies still confuse the cryptogenic and idiopathic groups. Some syndromes in childhood, which are completely described by strict electroclinical criteria such as the absence epilepsies, juvenile myoclonic epilepsies, are usually included and analyzed in epidemiologic studies; however, other epileptic syndromes observed in infancy, such as benign familial neonatal seizures and benign myoclonic epilepsy in infancy, are quite rare and are usually excluded from epidemiologic surveys because they are difficult to describe completely in electro-clinical terms. Another strong limitation in the study of epidemiology of IGEs is the lack of EEG data, either because EEG is not available or the routine EEG is normal. This is particularly relevant in the inclusion of patients with only tonic-clonic seizures. IGEs encompass several different syndromes, and a few patients shift from one phenotype to another. The overlapping of some syndromes during infancy and adolescence increased the difficulty to individualize strictly the correct syndrome. Many discrepancies can be observed in the distribution of the different syndromes included in the group of IGEs, because the strict criteria for classifying these syndromes proposed by the ILAE are often not respected. With this understanding, the general frequency of IGEs can be assessed at 15-20% of all epilepsies. The frequency and the distribution of incidence and prevalence of the different syndromes are tentatively reported and discussed. When the term idiopathic is used following the restrictive ILAE criteria, the mortality data concerning patients with idiopathic epilepsies do not show an increased standardized mortality ratio.

Bodyweight gain is a common and frequent undesirable effect associated with the use of anticonvulsant drugs. This has been observed for many years with valproic acid (sodium valproate) and carbamazepine, and also, more recently, with some of the newer anticonvulsants such as vigabatrin and gabapentin. Very often bodyweight gain in children, adolescents and adults with epilepsy taking such anticonvulsants results in cosmetic adverse effects. On the other hand, bodyweight gain is disturbing to general health, with a possible increase in the risk of diabetes mellitus or heart disease. Other potential adverse effects, such as the association of obesity with polycystic ovaries, have been reported with the use of valproic acid. Potential mechanisms of anticonvulsant-associated bodyweight gain are not yet clear and differ between drugs used. The involvement of lowered blood glucose level, which may stimulate eating through an effect on the hypothalamus, constitutes one of the possible mechanisms. Lowered blood glucose levels may result from a competition between the binding of the drug and long chain fatty acids. An increased availability of the latter stimulates insulin production and lowers the serum glucose levels. Another possible explanation for lowered blood glucose may be a deficiency in carnitine directly caused by the drug, that would result in a reduction of fatty acid metabolism and an increase in glucose consumption. An enhancing effect of gamma-aminobutyric acid-mediated neurotransmission may increase appetite for carbohydrates and reduce energy expenditure. An antidiuretic hormone-like effect or effects on norepinephrine (noradrenaline) or serotonin-mediated neurotransmission are more rarely considered. Many studies on anticonvulsant-associated bodyweight gain illustrate how we could better define the risk factors for the development of anticonvulsant-induced bodyweight gain and uncover the mechanisms behind it.

Summary:Purpose: We describe first unprovoked seizures and newly diagnosed epilepsies at initial presentation, with a special emphasis on epilepsy syndromes, in a large cohort recruited in the mid-1990s in France.Methods: The French Foundation for Research on Epilepsy set up a network to conduct a prospective study of patients with newly diagnosed unprovoked seizures. Information was provided by 243 child or adult neurologists. Four neurologists classified each case according to the International League Against Epilepsy (ILAE) criteria. First-seizure patients and patients with previously undiagnosed seizures were compared.Results: Between May 1, 1995, and June 30, 1996, 1,942 patients aged from 1 month to 95 years were identified: 926 (47.7%) with a single seizure and 1,016 (52.3%) with newly diagnosed epilepsy. All but 17 patients had EEGs. In the firstseizure and newly-diagnosed-epilepsy groups, neuroimaging studies were performed in 78.2 and 68.3% of patients, and medication prescribed in 54.1 and 89.6%, respectively. There were significant differences between the two groups with respect to age at onset and diagnosis, sex, etiology, several specific syndromes, as well as the type and presentation of initial seizure. In patients for whom the first seizure was convulsive, only sex, multiple seizures in a day or status epilepticus, and cryptogenic localization-related syndrome differed between the two groups.Conclusions: Approximately half of patients who first came to attention for an unprovoked seizure already met epidemiologic criteria for epilepsy. There were significant differences between the types of patients with a first seizure and those with newly diagnosed epilepsy. One or several seizures at diagnosis did not influence the diagnostic assessment of the patients but had a strong influence on the initiation of treatment.

Summary:Objective: In the last decade several studies have been published on incidence, etiology, and prognosis of status epilepticus (SE) with population-based data from the United States and Europe. The aim of this review is to summarize the available information on the epidemiology of SE and to outline the sources of the variability in reported mortality after SE.Methods: Comparison of mortality studies in SE from the United States and Europe.Results: The incidence of SE is lower in Europe (9.9-15.8/10,000) than in the United States (18.3-41/100,000). The overall mortality after SE is similar in the two U.S. studies: the case fatality is 21% in Rochester, and 22% in Richmond. All European studies excluded SE after anoxic encephalopathy following cardiac arrest. This exclusion may partly explain the lower case fatality (around 10%) found in two of the European studies. The study from Bologna showed the highest case fatality (33%) even after exclusion of anoxic encephalopathy. The mortality in acute symptomatic SE was higher than for other forms of SE across all studies.Conclusions: Short-term mortality after SE occurs mainly in the acute symptomatic group. Based on published data, it is not clear if differences in early management and medical treatment have any impact on prognosis or whether the differences can be attributed only to differences in distribution of the underlying causes in acute symptomatic SE. Future studies should address this issue. Key Words: Status epilepticus-MortalityPrognosis.Status epilepticus (SE), usually defined as a seizure lasting at least 30 min, is considered a medical emergency. It occurs most frequently in the context of an acute systemic or neurological insult and less frequently in patients with epilepsy.There have been many published studies regarding mortality after SE, most of which are based on clinical series from tertiary care centers. The reported mortality varies from 7% to 46% (1-15).Part of the variability in reported mortality after SE is dependant upon methodological issues. (1) In most studies the measure of mortality is case fatality (CF), the proportion of subjects dying in the cohort. The CF of different studies can be compared if the length of follow-up is the same. Most clinical series follow the patient through the hospital stay without specifying the number of days of follow-up, making comparison of CF across studies difAddress correspondence and reprint requests to Dr. Giancarlo Logroscino at Harvard School of Public Health, Kresge Bldg. 819, 677 Huntington Avenue, Boston, MA 02115, U.S.A. E-mail: glogrosc@hsph. harvard.eduThe commission wishes to acknowledge the support of UCB Pharma for logistical support in the organization of this workshop. ficult. (2) Multivariate techniques have been used only in the most recent studies to control for possible confounders such as age, gender, and underlying cause. (3) The consequences of SE have been compared by Sholtes et al. and differences in outcome are seen, according to the setting in which cases are identifie...