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Objective To determine the frequency and yield of neuroimaging in patients with known seizure disorders presenting to the emergency department (ED) with recurrent (nonindex) seizures. Methods We reviewed 822 consecutive ED visits for nonindex seizures at the Oregon Health & Science University and the VA Portland Health Care System. For each visit, we abstracted details of the clinical presentation, whether neuroimaging was obtained, the results of neuroimaging, and the results of previous neuroimaging studies, when available. We determined whether ED neuroimaging led to an acute change in patient management (yield). Clinical factors associated with obtaining ED neuroimaging, and with the yield of neuroimaging, were evaluated by multivariate logistic regression. Results A majority (78%) of ED seizure visits were for nonindex seizures. Neuroimaging was obtained in 381 of 822 nonindex seizure visits (46%). Of these, 11 imaging studies (3%) led to an acute change in patient management, 8 (2%) after excluding false-positive scans. Acute head trauma, prolonged alteration of consciousness, and a focal neurologic examination at presentation were associated with an increased yield of ED neuroimaging. Absent any of these 3 clinical factors the true positive yield of neuroimaging was zero. Significance ED neuroimaging was performed in nearly half of all patients presenting with nonindex seizures. A more conservative use of ED neuroimaging for nonindex seizures, based on clinical factors at presentation, could decrease imaging frequency with minimal loss of yield.

OBJECTIVE: Growing evidence suggests that psychological treatments are effective for improving outcomes in both epilepsy and psychogenic nonepileptic seizures (PNES). However, the risk of in-session seizures may cause concerns about safety and about seizures disrupting treatment. This study explores the risk of in-session seizures in patients with epilepsy and those with PNES, the timings of seizures during psychological therapy, and the responses of therapists to seizures.METHODS: Consecutive patients with epilepsy or PNES attending 2 neurology centers in the United Kingdom for psychological treatment to help with their seizure disorders were studied. Information about seizures during outpatient psychological therapy sessions was gathered using a 12-item pro forma.RESULTS: Ninety-seven patients with epilepsy and 195 with PNES were evaluated. One in 32 patients with epilepsy and 1 in 8 with PNES had at least 1 in-session seizure. A seizure occurred in 1 in 136 treatment sessions provided to patients with epilepsy, and 1 in 36 sessions provided to those with PNES. The risk of in-session seizures was significantly greater in patients with PNES than in patients with epilepsy (odds ratio [OR] 4.4, 95% confidence interval [CI] 1.3-15.2). Seizures tended to occur in the first half of treatment programs and individual sessions and only disrupted sessions briefly. Only one patient with PNES required in-patient observation not involving overnight admission.SIGNIFICANCE: In-session seizures do occur and are much more common in patients with PNES than in those with epilepsy. Seizures rarely caused major disruption to psychological treatment, and could almost invariably be managed by the treating therapist without help from additional medical staff. Nonetheless, this research suggests that psychological therapy providers should anticipate the occurrence of in-session seizures and have safe management plans in place. The greater frequency of in-session seizures in PNES may add to our understanding of the mechanisms triggering these seizures.

The revolution in high-throughput omics technologies has dramatically expanded our understanding of the epilepsies as complex diseases. It is now clear that further progress in treating the full spectrum of seizure disorders requires a systems-level framework for analyzing and integrating data from multiple omics technologies that moves beyond the search for single molecular alterations to an understanding of dysregulated pathways in epilepsy. Taking such a pathway-centered view requires further integrating the tools of systems biology into epilepsy research. In this appraisal, we highlight and summarize systems biology approaches in basic epilepsy studies as they were discussed during the 2017 Workshop on the Neurobiology of Epilepsy (WONOEP). During the 3-day event, participants exchanged emerging results and thoughts on developing the systems biology of epilepsy, and the promise and limitations of these approaches for the near term.

Summary Objective Infantile spasms (IS) have long been suspected to be a risk factor for impairment in intellectual development, but there are no controlled, prospective longitudinal data in well-characterized conditions to confirm this suspicion. We tested the hypothesis in a longitudinal study of children with tuberous sclerosis (TS), who have a high risk of developing IS. Methods Eleven infants with TS were recruited and studied longitudinally using the Mullen Scales of Early Learning. Seizure histories were assessed using a structured parent interview and by review of medical notes. Intellectual development was examined in relation to the onset and length of exposure to IS and other types of seizures. Results Six children developed IS and five children developed other types of seizure disorders. Among those that developed IS, estimated mean IQ dropped significantly (nonparametric test for trend p = 0.002) from 92 (prior to onset of spasms) to 73 (after exposure to IS for a month or less) and 62 (after exposure to IS for more than a month). By contrast, there was no significant drop in estimated IQ among the five infants exposed to other types of seizure disorders (nonparametric test for trend p = 0.9). All six children exposed to infantile spasms developed clinically significant intellectual impairment. Significance These data provide the first clear evidence of clinically significant, dose dependent, impairment in intellectual development following exposure to infantile spasms. The mechanisms underlying this developmental impairment and methods for preventing it require in depth study.

The blood-brain barrier (BBB) is located within a unique anatomic interface and has functional ramifications to most of the brain and blood cells. In the past, the BBB was considered a pharmacokinetic impediment to antiepileptic drug penetration into the brain; nowadays it is becoming increasingly evident that targeting of the damaged or dysfunctional BBB may represent a therapeutic approach to reduce seizure burden. Several studies have investigated the mechanisms linking the onset and sustainment of seizures to BBB dysfunction. These studies have shown that the BBB is at the crossroad of a multifactorial pathophysiologic process that involves changes in brain milieu, altered neuroglial physiology, development of brain inflammation, leukocyte-endothelial interactions, faulty angiogenesis, and hemodynamic changes leading to energy mismatch. A number of knowledge gaps, conflicting points of view, and discordance between clinical and experimental data currently characterize this field of neuroscience. As more pieces are added to this puzzle, it is apparent that each mechanism needs to be validated in an appropriate clinical context. We now offer a BBB-centric view of seizure disorders, linking several aspects of seizures and epilepsy physiopathology to BBB dysfunction. We have reviewed the therapeutic, antiseizure effect of drugs that promote BBB repair. We also present BBB neuroimaging as a tool to correlate BBB restoration to seizure mitigation. Add-on cerebrovascular drug could be of efficacy in reducing seizure burden when used in association with neuronal antiepileptic drugs.

A significant number of epileptic patients fail to respond to currently available anti-epileptic drugs. This suggests a need for alternative approaches to reduce the occurrence of seizures in these patients. Recent data have shown that in addition to well-known neuronal mechanism, seizures may be a consequence of misguided inflammatory response and blood-brain barrier disruption. Both peripheral and brain pro-inflammatory events have been demonstrated to govern the onset of status epilepticus. Evidence deriving from the experimental and clinical realms supports the notion that a role for pro-inflammatory and cerebrovascular events in seizure disorders is broader than previously suspected. As a result, methods to pharmacologically reduce blood-brain barrier permeability and reduce inflammation have thus emerged as means to reduce seizure burden. For instance, corticosteroids have been shown to be beneficial and the same agents may be able to further reduce seizure burden in conjunction with currently prescribed anti-epileptic drugs.

Summary: Purpose: The correct diagnosis of epilepsy leads to an appropriate treatment. Methods: The first step is to distinguish epileptic seizures from nonepileptic attacks, and to make a precise seizure diagnosis and classification. The next step is to identify the etiology or basic disorders underlying the epilepsy by physical and neurologic examinations, laboratory tests, including EEGs and neuroradiologic examinations. Although the EEG is the most important laboratory examination for the diagnosis of epilepsy, limitations of EEG interpretations must be recognized. Results: A syndromic classification of the patients, to determine whether they fit known syndromes, should be attempted. If patients do not match a described syndrome, a neurobiologic approach, utilizing genetic, neurophysiological, and neuropharmacologic knowledge, alternatively provides useful information to understand the neurobiologic background of epilepsy. Conclusions: Both approaches have advantages and disadvantages for diagnosing and treating epilepsy. Both approaches can be used interchangeably with patients with seizure disorders, depending upon their condition. The epilepsy diagnosis, etiology, and seizure-type diagnosis should be reevaluated when seizure control is insufficient with first- and second-line antiepileptic drugs.

Summary: Purpose: To compare the localizing yield of sphenoidal electrodes placed under fluoroscopic guidance (SEs) and anterior temporal electrodes (ATEs) in ictal recordings from a group of patients with seizure disorders of anterior temporal origin. Methods: We compared ictal recordings of 156 seizures obtained with SEs and ATEs from 40 consecutive patients with seizures of anterior temporal origin. Four electroencephalographers reviewed ictal recordings independently and blind to the patients' identity, presurgical data, and inclusion of ATEs or SEs. Outcome variables included (a) number of correctly localized seizures with SE and ATE recordings by at least three raters; (b) number of ictal foci in which all seizures were localized only with SEs; and (c) number of seizures in which SEs identified the ictal onset ≥5 s earlier than ATEs. Results: Interrater agreement among the four raters was significantly greater with SE than with ATE recordings (p < 0.0001). The number of seizures correctly localized was significantly greater with SEs (n = 144) than with ATEs (n = 99; p < 0.0001). All the seizures [n = 36 (23%)] originating from 14 ictal foci (29%) in 11 patients (27.5%) were localized only with SEs. Finally, the ictal onset was detected at SEs ≥5 s earlier than at ATEs in 67 (43%) seizures originating from 33 (69%) foci in 30 (75%) patients. Conclusions: SEs improve interrater agreement in the localization of seizures of anterior temporal origin, and in about one fourth of patients, SEs add ictal data not identified by ATEs.

This chapter covers the syndromes of benign epilepsy with centrotemporal spikes (BECTS), nonlesional focal epilepsy in otherwise normal children (NLFN), and the genetic generalized epilepsies. BECTS is an epilepsy syndrome that always enters terminal remission before the general age of a planned transition of adolescents. This is also the case for the majority (65%) of those with childhood absence epilepsy (CAE). Approximately 15% of patients with CAE who initially remit during their childhood years later develop juvenile myoclonic epilepsy (JME) as teenagers. They will have many issues for continuing medical care and transition, because their seizure disorder generally persists into adulthood. A significant minority of NLFN (~35%) and most patients with JME continue to have active epilepsy into adulthood. In addition, CAE, JME, and NLFN patients are at risk of a number of significant adverse social outcomes that require ongoing advice and counseling.

Benzodiazepines (BZDs) were first synthesized in 1933 but were not introduced for the treatment of epilepsy until 1965, when Henry Gastaut treated status epilepticus with intravenous diazepam. Since then, BZDs have been widely used in the management of epilepsy in both children and adults. At present, seven different BZDs are used in various countries as antiepileptic drugs. Because of their high lipophilicity, rapid brain penetration, and brain receptor binding, BZDs administered either i.v. or rectally are often first-line agents for acute seizure management. Although they have also been used for epilepsy prophylaxis, chronic treatment with BZDs is sometimes hampered by their propensity to produce sedation and other side effects, including development of tolerance to their anticonvulsant effects. Nevertheless, all patients with a devastating seizure disorder should have the opportunity for prophylaxis with a BZD. Whether or not treatment continues will depend on careful evaluation of the therapeutic response, toxicity, and the development of tolerance.

Summary: Hypothalamic hamartomas are rare developmental malformations of the inferior hypothalamus. Typically they result in symptomatic, refractory mixed seizure disorder with trademark gelastic or laughing seizures. We present a 30-month-old boy with a hypothalamic hamartoma and a nearly 2-month history of near-continuous gelastic seizures, which we have labeled “status gelasticus.” The seizures were refractory to multiple antiepileptic drugs (AEDs), and emergency transcallosal resection of the hypothalamic hamartoma was performed with resultant near-immediate cessation of the seizures. At 12-month follow-up, the patient was averaging one brief gelastic seizure every 2 weeks, with a longest seizure-free period of 2 months.

The prognoses for seizure disorders have been examined since the beginnings of epileptology, and only recently has the realization emerged that, ultimately, prognosis depends on causation, which, in turn, determines whether a condition is self-limited or progressive. This factor is more important than either mode or alacrity of therapeutic intervention. The epilepsies are a series of conditions that have the final common path of either increasing cerebral irritability or synchronizing normally occurring electrical activity in such a manner that seizures result. In turn, some seizure disorders are characterized by secondary changes in neuronal synaptogenesis, leading to the development of circuits of predilection, which then render the process autonomous. Epileptogenesis has then become epilepsy, which is the norm in acquired rather than genetic epileptogenesis. An understanding of the basic differences between the primary (idiopathic) epilepsies and the secondary (acquired or symptomatic) epilepsies is basic to a discussion concerning prognosis and to the development of a definitive individualized treatment plan. An elucidation of the genetic factors in idiopathic epilepsy and their neurochemical consequences represents a major frontier in epileptology.

Age has a profound influence on our approach to the convulsive disorders. Age is a variable which is an important determinant for risk factors for epilepsy. Age, as a surrogate of brain maturation, is a determinant of the specific characteristics of the seizure disorder in those with epilepsy, and age-related changes in these manifestations can be identified. Age is a determinant for the occurrence of acute symptomatic seizures in several types of metabolic or central nervous system insults. Age is a determinant for prognosis, whether one considers remission, medication withdrawal in those entering remission, relapse following prolonged remission, or mortality. Last, age per se seems to be a risk factor for epilepsy independent of other factors. This seems particularly true for partial seizures.

Summary: Purpose: To examine the prevalence of self-reported epilepsy or seizure disorder and its association with self-reported recent depression and anxiety in a large sample of the U.S. adult population. Methods: We analyzed data from adults aged 18 years or older (n = 4,345) who participated in the 2004 HealthStyles Survey, a large mail panel survey designed to be representative of the U.S. population. Results: Among U.S. adults aged 18 years or older, we estimated that 2.9% have been told by a doctor that they had epilepsy or seizure disorder, and an estimated 1.6% and 0.9% had active and inactive epilepsy, respectively. After controlling for demographic characteristics, we estimated that adults with self-reported epilepsy were twice as likely to self-report depression or anxiety in the previous year as were adults without epilepsy, and adults with active epilepsy were 3 times as likely to self-report depression and twice as likely to have anxiety in the previous year as were adults without epilepsy. Conclusions: Our findings highlight the burden of self-reported depression and anxiety among adults with self-reported epilepsy or seizure disorder, and suggest that healthcare providers should attempt to determine whether adult patients with epilepsy have any psychiatric comorbidity potentially to improve health outcomes. Questions about epilepsy and related factors should be routinely included on population-based surveys so that we can better understand the epilepsy distribution in the U.S. population and identify the unmet health and psychosocial needs of people with epilepsy.

The routine EEG study may prove inadequate in selected patients with recurrent and unprovoked seizures. Video-EEG monitoring is an important diagnostic innovation that may be used to confirm the diagnosis of a seizure disorder and classify seizure type. The methodologies and clinical applications of video-EEG monitoring are reviewed. Long-term EEG recordings may be performed as an outpatient or in an epilepsy monitoring unit. Potential techniques for video-EEG monitoring include prolonged conventional routine EEG, ambulatory EEG, and computer-assisted EEG recordings. Periictal SPECT studies performed during long-term EEG recordings in the epilepsy monitoring unit may increase the diagnostic yield of a presurgical evaluation. Video-EEG monitoring is essential for surgical localization in patients with intractable partial epilepsy being considered for a focal cortical resection. The studies may also be necessary to confirm the presence of nonepileptical clinical episodes.

A 5-year-old mentally retarded child developed laboratory evidence of pancreatitis during accidental acute carbamazepine (CBZ) intoxication. He had been seizure-free with CBZ for 4 years for a seizure disorder with no obvious toxicity. CBZ had been discontinued 5 months before he was admitted to the hospital. After he accidentally ingested a CBZ overdose, he was found vomiting and lethargic. Serum amylase and lipase levels were increased for several days. With supportive treatment and no CBZ, he recovered and serum amylase and lipase levels returned to normal. No other causes of pancreatitis were identified. Therefore, most likely the chemical pancreatitis was associated with the acute CBZ intoxication.

Summary: The cause of learning impairment in children with seizure disorders is obscure, in part because of the lack of adequate animal models of learning deficiencies that can be used for study. The discriminating passive avoidance test has been used extensively to study learning behavior in chicks. In the present study, we applied the passive avoidance test to epileptic chicks to determine if learning deficiencies could be demonstrated in this epileptic model. Epileptic fowl have a hereditary form of primary generalized epilepsy characterized by tonic-clonic seizures. The seizures occur spontaneously and can also be induced by photic stimulation. The epileptic phenotype is the result of an autosomal recessive mutation. Heterozygotes do not have seizures and were used as age-matched controls. The discriminating passive avoidance test used in the present study was based on the observation that chicks readily peck at bright shiny beads. Once chicks taste a colored bead coated with a bitter chemical (methylanthranilate), they refuse to peck the bead on subsequent presentations. In addition, chicks can discriminate between colored beads and will continue to peck at a bead of different color than the methylanthranilate (MeA)-coated bead. Compared to carriers, epileptic chicks demonstrated significantly less ability to discriminate between colored beads. Furthermore, this learning impairment was observed in epileptic chicks treated with phenobarbital (PB), indicating that the learning impairment in epileptic chicks is an inherent neurological problem and not a consequence of seizure activity.

Accurate family histories of seizure disorders are important for both clinical practice and genetic research. This study evaluated validity of seizure histories of parents and siblings, obtained by interviewing 1,957 adults with epilepsy (probands). Probands were asked two questions to screen for occurrence of seizures in each relative; the first asked about epilepsy specifically, the second asked about "other seizures." For each relative who screened positive for seizures, a detailed clinical description was obtained. The final diagnosis was based on a review of all assembled information. Whenever possible, the proband's mother was interviewed independently about the family history, as were eligible relatives reported to have had seizures. Sensitivity, or the proportion of affected relatives who screened positive for seizures, was higher for epilepsy than for other seizures (87% vs. 32% assuming the mother's report to be correct, and 93% vs. 18% assuming self-report to be correct). For epilepsy, estimates of risk in siblings based on the final diagnoses were similar to previously reported findings in Rochester, Minnesota. For both isolated unprovoked seizures and acute symptomatic seizures, however, risk estimates were lower than in Rochester. These findings suggest that adults with epilepsy can report reasonably accurately about epilepsy in their parents and siblings, but isolated unprovoked seizures and acute symptomatic seizures are underreported.

Summary: A randomized controlled trial was conducted in Santiago, Chile to test the efficacy of the Children's Epilepsy Program, a child-centered, family-focused intervention developed and pilot tested in Los Angeles, CA, U.S.A., using a counseling model for parents of children with seizure disorders to help them (a) deal with their anger, resentment, and grief related to the loss of a normal child; (b) increase their knowledge about caring for their child; (c) reduce anxieties related to having a child with a seizure disorder; and (d) improve their decisionmaking skills. All parents were pretested and then retested 5 months after the educational interventions. Parents in the experimental group (n = 185) and their children separately attended four 1 A-h sessions and then met together at the end of each session to share learning experiences. Comparison group parents (n = 180) and their children jointly attended three 2-h lecture sessions followed by question-and-answer periods. Although parents' overall knowledge of epilepsy was relatively high initially, it improved considerably in both comparison and experimental groups. With regard to anxiety, at the 5-month evaluation, experimental group parents and mothers in particular were more likely than control parents to state that they were less anxious (p < 0.001), and their anxiety, as measured by the Taylor Manifest Anxiety scale, was significantly reduced (p < 0.01). RESUME Une etude randomisee et controlee a ete menee a Santiago (Chili), afin ďevaluer ľefficaciteďun programme ďeducation pour ľepilepsie de ľenfant: il s'agit ďune intervention centree sur ľenfant, en milieu familial, qui a ete mise au point et testee a Los Angeles (U.S.A.) selon un modele de conseils aux parents ďenfants epleptiques. Ce programme avait pour but: (1) de traiterles sentiments de colere, ressentiment, et tristesse lies a la perte ďun enfant normal; (2) augmenter les connaissances surles soins a apporter aľenfant; (3) reduire ľanxiete liee aľepilepsie de leur enfant; (4) ameliorer leur formation et leur conduite. Tous les parents ont ete evalues de facon preliminaire puis re-evalues 5 mois apres les interventions educatives. Les parents du groupe experimental (n = 185) et leurs enfants ont assiste separement a 4 sessions ď1 heure 1/2, puis se rencontraient a la fin de chaque session pour partager leur experience ďapprentissage. Un groupe controle de parents (n = 180) et leurs enfants ont assiste ensemble a 3 sessions comprenant des cours de 2 heures suivis par des sessions de questions et reponses. Bien que la connaissance globale de ľepilepsie chez les parents ait ete relativement importante des le depart, elle a ete considerablement amealioree dans le groupe experimental comme dans le groupe controle. En ce qui concerne ľanxiete, ľevaluation a 5 mois a montre que les parents du groupe experimental, en particulier les meres, signalaient plus souvent que les parents du groupe controle qu'ils etaient moins anxieux (p < 0.001); leur anxiete, mesuree par ľechelle ďAnxiete Manifeste de Taylor, etait significativement moindre (p < 0.01). ZUSAMMENFASSUNG Eine randomisierte, kontrollierte Studie wurde in Santiago, Chile durchgefuhrt, um die Wirkung eines Kinder-Epilepsie-Programms zu testen: dabei handelt es sich um ein in Los Angeles, Kalifornien entwickeltes und pilotgetestetes Modell zur kind und familiengerechten Intervention, entsprechend einem Beratungsmodell fur Eltern von Kindern mit Anfallen. Ziel war es, den Eltern zu helfen: (1) im Umgang mit ihren Angsten, Resentiments und Kummer durch den Verlust eines normalen Kindes (2) ihr Wissen uber die Betreuung ihres Kindes zu vergrosern (3) ihre Angst zu verringern, ein Kind mit einem Anfallsleiden zu haben (4) ihre Entscheidungsfahigkeit zu verstarken. Samtliche Eltern wurden vorgetestet und 5 Monate spater, nach der Unterweisung nachgetestet. In der Testgruppe (n = 185) besuchten die Eltern und ihre Kinder getrennt 4 1 1/2 Stunden Seminare, um sich nach jeder Sitzung noch gemeinsam zur Vertiefung des Gelernten zu treffen. In einer Vergleichgruppe (n = 180) besuchten Eltern und Kinder 3 2-Stunden-Vorlesungen, gefolgt von einer Frage- und Antwortstunde. Obwohl bei alien Eltern das Wissen ziemlich gut war, verbesserte es sich jedoch betrachtlich in beiden Gruppen. Bezuglich der Angst waren nach 5 Monaten die Eltern, besonders die Mutter der Testgruppe in der Lage zu sagen, sie seien weniger angstlich als die der Kontrollgruppe (p = 0.001); gemessen an der Taylor-Angst-Skala war ihre Angst signifikant verringert (p = 0.01).

Summary: Information on the frequency of epilepsy/ seizure disorders and factors associated with development of epilepsy in an underprivileged urban ghetto population is urgently needed. A feasibility study was undertaken, and a questionnaire to collect information on seizure activity and behavior in the community was administered to a series of households with known seizure disorders, a series of households with chronic diseases, and a series of households chosen at random. Sensitivity and specificity of the seizure questions were estimated to be 0.93 and 0.89 respectively. The community was receptive to a survey of this type, and the overall refusal/ nonresponse rate was 7.2%. Factors that would contribute to the success of an urban minority community survey are considered. RESUME Il apparait urgent de connaitre la frequence de l'epilepsie et des crises, ainsi que les facteurs associes au developpement une epilepsie, au sein des populations defavorisees des ghettos urbain. Une etude de faisabilite a ete entreprise; un questionnaire a eteetabli, qui visait a rassembler les informations sur la survenue de crises et sur le comportement dans ce type de communaute. Ce questionnaire a ete administrea une serie de foyers ou vivait une personne presentant des crises, a une serie de foyers comprenant des personnes atteintes de maladies chroniques, et une serie de foyers choisie au hasard. La sensibilite et la specificite des questions se rapportant aux crises ont ete estimees a 0.93 et 0.89 respectivement. La communaute s'est revelee receptive a une enquete de ce type, et le taux global de refus ou de non-reponses a ete de 7.2%. Les facteurs qui peuvent contribuer au succes une etude realisee en milieu urbain defavorise sont discutes dans ce travail. RESUMEN Es urgente disponer de informacion acerca de la frecuencia de la epilepsia/trastorno convulsivo y los factores asociados al desarrollo de la epilepsia en una poblacion-ghetto de zonas urbanas subdesarrolladas. Se ha llevado a cabo un estudio piloto disenando un questionario para recojer informacion sobre la actividad de los ataques y el comportamiento. El cuestionario se cumplimento en una serie de hogares en los que existfan desordenes convulsivos conocidos, en hogares con enfermedades cronicas y en varios hogares seleccionados al azar. La sensibilidad y especificidad de las preguntas se estimo en 0.93 y 0.89 respectivamente. La comunidad acepto positivamente este estudio y la totalidad de rechazos o no-respuestas fue de 7.2%. Se analizan los factores que pueden contribuir al exito de este tipo de estudios en una comunidad urbana compuesta de minorias. ZUSAMMENFASSUNG Informationen uber die Haufigkeit von Epilepsie/ Anfallskrankheiten sowie Faktoren, die mit der Epilepsieentwicklung bei einer unterpriviligierten stadtischen Gettobevolkerung assoziiert sind, sind dringend notig. Im Rahmen einer solchen Durchfuhrbarkeitsuntersuchung wurde ein Frage-bogen entwickelt, um Informationen uber Anfallshaufigkeit und das Verhalten innerhalb der Gemeinde zugewinnen. Der Fragebogen wurde einer Reihe von Haushalten mit bekannten Anfallsleiden gegeben sowie Haushalten mit chronischen Erkrankungen und zufallig ausgewahlten Haushalten. Die Sensitivitat und Spezivitat der Fragen bezuglich Anfallen wurde mit 0.93, bzw. 0.89 geschatzt. Die Gemeinde war einer solchen Untersuchung gegenuber aufgeschlossen, die Verweigerungs-, bzw. Nichtrucklaufrate betrug 7.2% Faktoren, die zum Erfolg einer solchen Ubersichtsstudie beitragen, werden erortert.

Summary: This study evaluates the Wonderlic Personnel Test and its ability to duplicate the Wechsler Adult Intelligence Scale (WAIS) Full Scale IQ. The Wonderlic can be given and scored by a clerk in approximately 15 min and is suitable for both individual and group administration. With the use of original (n= 100) cross-validational (n= 50) adult epileptic samples, no biases or distortions in predictability were observed across the variables of sex, age, years of education, level of intelligence, neuropsychological impairment, emotional status, primary seizure diagnosis, etiology of seizure disorder, age at onset of seizure disorder, and seizure activity. The average error in estimation of WAIS Full Scale IQs from Wonderlic IQs ranges from 0.1 to 0.3 of a single IQ point. In individual cases, there is a 90% probability that the Wonderlic will render an IQ score within 10 points of the WAIS Full Scale IQ. The Wonderlic appears to be useful in both clinical and research contexts in evaluating people with seizure disorders. RESUME Cette etude presente ľexamen du Wonderlic Personnel Test et son aptitude a doubler ľEchelle ďlntelligence pour Adultes de Wechsler (WAIS), echelle complete donnant un QI Le Wonderlic peut etre administre et depouille par un employe en 15 minutes environ et il est adapte aussi bien a la passation individuelle que collective. ľetude de validation comportant ľechantillon ďorigine (n= 100) et ľechantillon ďadultes epileptiques (n= 50) ne permet ďobserver aucune distorsion probable en fonction des variables de sexe, ďâge, dannees de scolarite, de niveau ďintelligence, ďalteration neuropsychologique, ďetat emotionnel. de diagnostic primaire ďepilepsie, ďetiologie des crises, de ľâge ďapparition et de leur nombre. ľerreur moyenne en comparant le QI obtenu aľechelle complete de WAIS et au Wonderlic est de 0.1 a 0.3 points de QI. Dans les cas individuels, il y a 90% de probabilite pour que le QI au Wonderlic ne s'ecarte pas plus de 10 points du QI de ľechelle complete du WAIS Le Wonderlic peut etre utilise dans les deux domaines de la clinique et de la recherche pour ľexamen de sujets epileptiques. RESUMEN El estudio presenta la evaluacion de la Prueba Personal Wonderlic y su capacidad de reproducir la Escala de Inteligencia en Adultos Wechsler (WAIS) con CI de Escala Completa. El Wonderlic puede ser administrado y medido por una secretaria en unos 15 minutos y es adecuado tanto para grupos como para individuos. Comparando muestras de epilepticos adultos (n= 100 y n= 50) analizadas por uno y otro metodo, no se encontraron desviaciones o distorsiones en lo que respecta a las siguientes variables: sexo, edad, anos de educacion, nivel de inteligencia, deterioro neuropsicologico, estado emocional, diagnostico primario de las crisis epilepticas, etiologia de las convulsiones, edad de comienzo de los ataques y actividad de las crisis. El error promedio, comparando la Escala WAIS de CI y la Prueba Wonderlic de CI, oscilo entre 0.1 y 0.3 en un punto aislado del CI. En casos individuales hubo una probabilidad del 90% de que la Prueba Wonderlic mostrase un CI con una variacion de menos de 10 puntos al compararlo con la Escala WAIS de CI. La Prueba Wonderlic parece util evaluando personas con crisis comiciales, tanto en un contexto clinico como investigacional. ZUSAMMENFASSUNG Die Untersuchung pruft den Wonderlic Personnel Test und vergleicht ihn mit den Wechsler Intelligenztest fur Erwachsene (WAIS). Der Wonderlic-Test kann von einem Angestellten in etwa 15 Min. durchgefuhrt und bewertet werden; er ist geeignet zur Anwendung sowohl fur Individuen sowie fur Gruppen. Bei Verwendung einer Gruppe (n= 100) kreuzvalidierter (n= 50) erwachsener Epileptiker wurden kein Bias und keine Abweichung in der Vorhersagbarkeit der Variablen Geschlecht, Alter, Jahre der Ausbildung, Intelligenzgrad, neuropsychologische Auffalligkeiten, emotionaler Status, primare Anfalls-diagnose, Atiologie der Anfallskrankheit, Alter bei Beginn des Anfallsleidens un.

Summary: A 37-year-old woman with a post-traumatic seizure disorder had four well-documented episodes of postical acute pulmonary edema and respiratory failure. Subtherapeutic blood concentrations of phenytoin were documented on each admission. Each episode followed one or more grand mal seizures and was characterized by the development of diffuse nodular-appearing alveolar infiltrates, tachypnea, and severe hypoxemia that rapidly resolved with supportive therapy. There was no evidence of gastric acid aspiration, acute lung infection, or underlying heart or lung disease. To determine the frequency of postictal pulmonary edema in our institution, we reviewed the clinical records and chest roentgenograms of 45 consecutive patients who were admitted to our emergency room following a well-documented grand mal seizure. Only one patient (described in this report) had chest roentgenographic evidence of pulmonary edema. A review of the English literature revealed only 11 reported cases of postictal pulmonary edema since 1965 and a total of 42 episodes in 27 patients since 1908. There were no clearly documented cases of postical pulmonary edema following electroconvulsive therapy in 18 published reports totaling more than 38,000 subjects. Our findings suggest that while postictal pulmonary edema may occur repeatedly in the same patient, the overall frequency of this complication is low. RESUME Une femme de 37 ans souffrant d'une epilepsie post-traumatique a presente 4 episodes bien etudies d'oedeme aigu pulmonaire avec defaillance re-spiratoire. Un taux sanguin de phenytoine inferieur aux valeurs therapeutiques a ete constatea chaque occasion. Chaque episode a suivi la survenue d'une ou plusieurs crises Grand Mal et a ete caracterise par le developpement d'infiltrats alveolairess nodulaires diffus, d'une tachypnee et d'une hypoxemic severe qui ont cede rapidement au traitement. II n'y avait aucun signe d'aspiration d'acide gastrique, d'infection pulmonaire aigue ou de maladie cardiaque ou pulmonaire sous-jacente. Une revue des dossiers cliniques et des radiographias pulmonaires de 45 malades consecu-tivement admis au service des urgences de notre institution a la suite de crises de Grand Mai n'a montre qu'un seul cas (celui rapporte ci-dessus) comportant des signes radiologiques d'oedeme pulmonaire. Une revue de la litterature de langue anglaise fait apparaitre 11 cas d'oedeme aigu du poumon post-critique depuis 1965 et 42 episodes chez 27 patients depuis 1908. II n'y a pas un seul cas d'oedeme pulmonaire dans les suites de traitement par electrochoc rapportes dans 18 publications concernant plus de 38 000 patients. Ces observations suggerent que, bien que l'oedeme pulmonaire post-critique puisse survenir de facon repetee chez un meme malade, la frequence de cette complication est faible. RESUMEN Una mujer de 37 anos con epilepsia post-traumatica tuvo 4 episodios bien documentados de edema pulmo-nar post-ictal e insuficiencia respiratoria. En cada una de las admisiones se documentaron niveles sub-terapeuticos de fenitoina. Cada episodio tuvo lugar de uno o mas ataques de gran mal y se caracterizaron por la aparicion de infiltrados alveolares de aspecto nodular, taquipnea e hipoxemia severa que se resolvio con rapidez usando tratamiento de sosten. No hubo evidencia de aspiracion gastrica, infection pulmonar aguda o enfermedad pulmonar y cardiaca subyacente. Con objeto de determinar la frecuencia de edema pulmonar post-ictal en nuestro centro, revisamos las his-torias y las radiograffas de torax de 45 pacientes con-secutivos que fueron ingresados en Urgencias despues de un ataque de gran mal bien documentado. Solo un paciente (el que se describe aqui) mostro en la radio-grafia edema pulmonar. Una revision de la literatura inglesa mostro 11 casos de edema pulmonar post-ictal desde 1965 y un total de 42 episodios en 27 pacientes desde 1908. No hubo ningiin caso bien documentado de edema pulmonar post-ictal despues de tratamiento con electroshock (18 trabajos totalizando mas de 38.000 pacientes). Nuestros hallazgos sugieren que, aunque edema pulmonar post-ictal puede ocurrir de manera repetida en un mismo paciente, la frecuencia general de esta complication es baja. ZUSAMMENFASSUNG Eine 37 Jahre alte Frau mit posttraumatischem Anfallsleiden erlebte 4 gut dokumentierte Episoden eines postiktalen akuten Lungenodems mit Ateminsuffizienz. Subtherapeutische Blutkonzentration von Phenytoin wurden bei jeder Aufnahme dokumentiert. Jede Episode ereignete sich im Anschlus an einen oder mehrere Grand Mal Anfalle. Sie war charakterisiert durch die Entwicklung von diffusen, knotig erscheinenden alveolaren Infiltraten, einen Tachypnoe und schweren Hypoxamie, die sich bei unterstutzen-der Therapie rasch besserten. Es bestand kein Hinweis einer Aspiration von saurem Mageninhalt, einer akuten Lungeninfektion oder einer unterlagernden Herzbzw. Lungenerkrankung. Um die Haufigkeit eins postiktalen Lungenodems in unserer Institution zu bestimmen, schauten wir die Krankenblatter und Thorax-Rontgenbilder von 45 aufeinanderfolgenden Patienten durch, die als Notfalle mit wohl dokumen-tiertem Grand Mal Anfall aufgenommen worden waren. Nur ein Patient (der hier beschrieben wird), hatte im Rontgenbild des Thorax Hinweise auf ein Lungenodem. Ein Uberblick der englischen Literatur zeigte nur 4 Mitteilungen eines postiktalen Lungenodems seit 1965 und insgesamt 42 Episoden bei 27 Patienten seit 1908. Es gibt keine eindeutig dokumen-tierten Falle eines postiktalen Lungenodems als Folge einer Elektroschockbehandlung bei 18 publizierten Arbeiten uber mehr als 38 000 Patienten. Unsere Be-funde legen die Vermutung nahe, dafS ein postiktales Lungenodem sich zwar wiederholt beim gleichen Patienten entwickeln kann, die allgemeine Haufigkeit dieser Komplikationen aber niedrig ist.

Summary: We reviewed 3,436 EEGs and found 24 patients with periodic lateralized epileptiform discharges (PLEDS). The etiology was unknown in 7, cerebrovascular occlusion in 7, tumor in 3, intracerebral hematoma in 2, and subdural hematoma, neonatal asphyxia, electrolyte imbalance, subarachnoid hemorrhage, and hypoglycemia in each of the remaining cases. We were successful in contacting 18 patients and/or their families for follow-up. Twenty of the 24 patients with PLEDS had seizures. Seven had focal motor alone, 10 had focal motor with secondary generalization, and 3 had generalized seizures without any observed focal features. Four patients had no seizures. Twelve patients had their first seizure at the time PLEDS were found. Fifteen adults and 3 infants were reevaluated. Only 1 adult was functionally independent. The 3 infants evidenced developmental delay. Six adults had seizures prior to the observation of PLEDS, and 5 (83%) of them reported seizures after hospitalization. Nine of the 15 adults had their first seizure associated with PLEDS, 6 of whom (67%) also reported seizures after hospitalization. In 9 patients with serial EEGs during their hospitalization, PLEDS disappeared within 22 days. We concluded that most patients with PLEDS and concomitant seizures continue having seizures after hospitalization and need antiepileptic medication. RESUME Les auteurs ont trouve 24 fois des decharges epilep-tiformes periodiques lateralisees (PLEDS) sur 3.436 traces examines. L'etiologie etait sept fois inconnue et concernait les autres fois: une occlusion vasculaire cerebrale (7 cas), une tumeur (3 cas), un hematome intracerebral (2 cas), une epilepsie neo-natale, un trouble de l'equilibre electrolytique, une hemorragie sous-arachnoidienne et une hypoglycemie (1 cas respec-tivement). L'evolution de l'affection a pu etre connue pour 18 des 24 patients. Vingt patients sur vingt quatre ont presente des crises. Sept fois focales motrices isolees, dix fois focales motrices avec generalisation secondaire, trois generalisees sans symptomatologie focale apparente. Douze patients avaient leur premiere crise au moment oil les PLEDS ont ete trouves. Quinze adultes et trois enfants ont ete revus. Les trois enfants montraient un retard du developpement. Un seul adulte etait fonctionnellement independant. Six adulte avaient des crises avant l'apparition des PLEDS et cinq (83%) d'entre eux en presentaient encore apres l'hospitalisation. Neuf des quinze adultes ont eu leur premiere crises en me me temps que les PLEDS et 6 d'entre eux (67%) en presentaient encore apres l'hospitalisation. Chez neuf patients ayant eu des EEG repetes pendant leur hospitalisation les PLEDS ont dis-paru dans un delai de 22 jours. En conclusion les auteurs admettent que la plupart des patients qui presentent des PLEDS et des crises concommitantes continued a presenter des crises apres leur hospitalisation et doivent recevoir un traitement anti-epileptique. RESUMEN Revisamos 3.436 EEGs encontrando 24 pacientes con descargas epileptiformes periodicas lateralizadas (PLEDS). La etiologia fue desconocida en 7 enfermos, habia una oclusion cerebrovascular en 7, un tumor en 3, un hematoma intracerebral en 2 y un hematoma subdural, asfixia neonatal, anomalias electroliticas, hemorragia subaracnoidea e hipoglucemia en cada uno de los restantes casos, respectivamente. Tuvimos exito contactando con 18 pacientes y/o sus familias con propositos de revision. Veinte de los 24 enfermos con PLEDS tuvieron crisis. En 7 las crisis solo fueron motoras, 10 tuvieron crisis motoras focales con generalizacion secundaria y 3 tuvieron ataques generalizados sin que se observaran signos focales. Cuatro pacientes no tuvieron ataques. Doce enfermos tuvieron el primer ataque al mismo tiempo que se observaron PLEDS. Se re-evaluaron 15 adultos y 3 ninos. Solo 1 adulto era funcionalmente independiente. Los 3 ninos mostraron retraso en el desarrollo. Seis adultos mostraron crisis antes de que se observaran las PLEDS y 5 de ellos (83%) informaron que habian tenido ataques despues de la hospitalizacion. En 9 enfermos que tuvieron repetidos EEGs durante su hospitalizacion, las PLEDS desaparecieron antes de los 22 dias. Nuestra conclusion fue que la mayoria de pacientes con PLEDS y crisis comiciales concomi-tantes continuaron teniendo ataques despues de su hospitalizacion y necesitaron medicacion anticomicial. ZUSAMMENFASSUNG Wir sahen 3436 EEG durch und fanden 24 Patienten mit periodischen lateralisierten epileptiformen Ent-ladungen (PLEDS). Die Atiologie war bei 7 unklar, 7 hatten cerebrovaskulare Verschlusse, 3 einen Tumor, 2 ein intracerebrales Hamatom, je einmal wurden ein subdurales Hamatom, eine neonatale Asphyxie, eine Elektrlytstorung, eine subarachnoidale Blutung und eine Hypoglykamie gefunden. 18 Patienten konnten erreicht und sie oder ihre Familien verfolgt werden. 20 der 24 Patienten mit PLEDS hatten Anfalle: 7 nurfokal motorische, 10 fokal motorische mit sekundarer Generalisierung und 3 generalisierte Anfalle ohne fokale Symptome. 4 Patienten hatten keine Anfalle. 12 Patienten hatten ihren ersten Anfall zum Zeitpunkt, an dem PLEDS gefunden wurden. 15 Erwachsene und 3 Kinder wurden kontrolluntersucht. Nur 1 Er-wachsener war funktionell unabhangig. Die 3 Kinder zeigten eine Entwicklungsverzogerung. 6 Erwachsene hatten Anfalle vor dem Zeitpunkt, an dem PLEDS beobachtet wurden und 5 (83%) von ihnen berichteten uber Anfalle nach der Hospitalisierung. 9 der 15 Er-wachsenen hatten ihren ersten Anfall zum Zeitpunkt der PLEDS, 6 von ihnen (67%) auch Anfalle nach der Hospitalisierung. Bei 9 Patienten mit me h re re n EEG-Untersuchungen wahrend der Hospitalisierung verschwanden die PLEDS innerhalb 22 Tagen. Wir schlossen, das die meisten Patienten mit PLEDS und begleitenden Anfallen weiterhin Anfalle nach Hospitalisierung haben und eine antiepileptische Medikation benotigen.

Summary: EEG and CAT studies were performed in 187 adult chronic epileptics. Acute convulsions, known tumor epilepsies, and seizures due to a rapidly advancing neurologic disease were excluded. Partial seizures of elementary and complex symptoms were nearly equally frequent. In 48 patients the etiology of the seizure disorder remained unknown. Among the verified etiologies residual brain damages were the most significant. In 10 cases the chronic seizure disorder was due to slowly growing brain tumors. CAT findings were normal in 86 patients (46%) and abnormal in 101 (54%). Localizing signs could be demonstrated by neuroradiologic and/or radionuclide tests in 15.7%, by CAT in 28.9%, by neurologic examination in 58.3%, and by EEG in 78.1%. Localized CAT-pathology showed no correlation to the types of partial seizures, whereas a significant correlation could be established with residual brain scars and tumors and with localized neurologic or EEG findings. Epilepsy starting after the age of 15 is more likely to be linked with CAT-pathology than with seizure disorders starting in childhood. ZUSAMMENFASSUNG 187 erwachsene Patienten mit chronischen partiellen Anfallen wurden mittels EEG und CAT untersucht. Akute Konvulsionen, bekannte Tumorepilepsien und Anfalle im Rahmen rasch fortschreitender neurologischer Erkrankungen wurden ausgeschlossen. Partielle Anfalle einfacher und komplexer Symptomatologie waren ungefahr gleich haufig. Bei 48 Patienten war die Atiologie unbekannt. Unter den be-kannten Ursachen waren residuale Hirnlasionen fuhrend. In 10 Fallen wurde ein langsam wachsender Hirntumor als Ursache des chronischen Anfallsleidens gefunden. Das CAT war bei 86 Patienten (=46%) normal und bei 101 (=54%) abnorm. Lokalhinweise er-gaben sich in 15.7% in AG, PEG und/oder Isotopenuntersuchung, in 28.9% im CAT, in 58.3% im neurologischen Befund und in 78.1% im EEG. Keine Korrelation zu einer lokalen CAT–Pathologie zeigten die Art der Anfalle, eine positive hingegen Residual– und Tumorepilepsien sowie Herdbefunde im neurologischen Status und im EEG. Die Art der lokalen EEG–Veranderung (Spitzen oder langsame Wellen) zeigte diesbezuglich keine Unterschiede. Bei Patienten, deren Epilepsie nach dem 15. Lebensjahr begann, ist die Wahrscheinlichkeit eines abnormen CAT hoher als bei fruher beginnenden.

Summary: The Wechsler Adult Intelligence Scale–Revised (WAIS-R), the newest version of the Wechsler Scales, has had several factor analytic studies, but none have been done in a sample of seizure-disorder patients. The factor structure of the WAIS-R was examined in this study in a sample of 107 patients with various seizure disorders. The results of this study revealed the 3-factor solution, as has been reported in many previous studies. The most notable difference was the relatively greater prominence of the Freedom from Distractibility factor. These results are consistent with studies of the factor structure of previous editions of the Wechsler Scales in samples of epileptic patients. The relative prominence of the Distractibility factor could suggest that attentional factors are a more important influence on performance than in other patient groups. Further studies are needed to clarify the effect of various seizure-related variables on patterns of intelligence. RESUME L'echelle d'intelligence adulte de Wechsler revisee (WAIS-R) a fait l'objet de plusieurs analyses factorielles. mais jamais chez les patients epileptiques. Nous avons etudie la structure factorielle du WAIS-R chez 107 patients presentant divers types d'epilepsie. Nous avons constate, comme les autres etudes, une solution de 3 facteurs. La difference la plus notable a ete la preeminence relative du facteur “distractibilitye”. Nos resultats corroborent ceux des etudes de structure factorielle ayant porte sur les anciennes versions du WAIS chez les epileptiques. La preeminence relative du facteur distractibilitye pourrait signifier que les facteurs lies a l'attention ont plus d'influence sur les performances que dans d'autres groupes de patients. D'autres travaux devront clarifier l'influence de diverses variables liees aux crises sur les processus de l'intelligence. RESUMEN La escala revisada de Wechsler para medida de inteligencia de adultos (WAIS-R). (la nueva version de la escala de Wechsler) ha sido sometida a varios estudios analiticos de factor pero ninguno ha sido realizado en una muestra de enfermos con ataques epileplicos. La estructura factorial del WAIS-R ha sido examinada en este estudio en una muestra de 107 enfermos con varios tipos de ataques. Los resultados han revelado las 3 condiciones factorials tal como se han publicado en muchos estudios previos. La diferencia mas notable ha consislido en la prominencia relativamente mayor del factor de libertad sobre el de distractibilidad. Los resultados son consistentes con estudios de estructura factorial, realizados con ediciones previas de la escala de Wechsler, en muestras de enfermos epilepticos. La prominencia relativa del factor de distractibilidad podria sugerir que los factores de atencion conllevan una influencia mas importante sobre las actividades totales que en otro grupo de enfermos. Estudios adicionales son necesarios para clarificar el efecto de varias variables relacionadas con los ataques sobre los patrones de la inteligencia.

A boy with infantile spasms was found to have a balanced de novo translocation, 6q;14q. The karyotype was 46,XY,t(6;14)(q27;q13.3). He had mental retardation and microcephaly but no dysmorphic features. Whether or not there is a relationship between the translocation in our patient and his infantile spasms is unknown. If there is a connection, a gene or genes on chromosome 14 may be implicated in the seizure disorders that are common in patients with ring chromosome 14 syndrome or proximal partial trisomy 14 syndrome.

This study examined the clinical effects of central cortical EEG feedback training in 8 patients with poorly controlled seizures. After base-line recordings, patients were trained in the laboratory and then initiated on a double or triple crossover design using portable equipment at home, with bimonthly laboratory test sessions. Performance at home was monitored by a strip chart recorder with the portable unit. Training was based on the simultaneous detection of two central cortical (C3--T3) EEG frequency bands (6--9 Hz and either 12--15 or 18--23 Hz), with reward provided for the occurrence of one in the absence of the other. The design consisted of successive 3 month periods of training, with reward contingencies reversed after each period without the subject's knowledge. Seizure incidence records were compared statistically before, during, and after the design. Six of the 8 patients reported significant and sustained seizure reductions, which averaged 74%, following reward for either 12--15 or 18--23 Hz in the absence of 6--9 Hz. Response to positive reward for 12--15 Hz was specific, with seizure rates returning to base line when reinforcement contingencies were reversed. Reduced seizure rates following positive reward for 18--23 Hz were not altered with contingency reversals. A nonspecific interpretation of these effects is rejected in favor of an EEG normalizing hypothesis.

Summary: The use of implantable arrays of epidural electrodes has made it possible to carry out extraoperative electrocorticography (ECoG) and functional localization in the awake child. This has permitted cortical excisions that are determined by criteria similar to those obtained during surgical procedures performed under local anesthesia in adults. In addition, the method also permits simultaneous ECoG and video monitoring during the child's symptomatic seizures, providing additional important localizing information that is impractical to obtain in operations under local anesthesia. We report our experience with 75 children, ages 5 months to 15 years, whom we have managed with epidural electrode arrays. The method of extraoperative ECoG is described and illustrative cases are presented to demonstrate its feasibility and utility in children. In addition, we call attention to gliomas as a common cause of chronic focal seizures in children. Of 49 children undergoing resection and followed for from 1 to 14 years (mean of 5.8 years), 32 (65%) are either seizure free or have had a significant reduction in seizure frequency that has unambiguously improved their quality of life. The results are analyzed further by relating the surgical outcome to each of the pathologic entities that caused the seizures. This analysis reveals the variety of neurological conditions that commonly cause intractable focal seizure disorder in children and distinguishes those pathologic entities in which the seizure disorder is apt to respond to surgical intervention from those that will not.

Summary: It has long been recognized that school personnel can play an important role in the treatment of seizure disorders. In order to better understand their involvement in the treatment process, two state-wide studies of special education programs were conducted. Teachers completed questionnaires for 315 pupils in their classrooms who were receiving drug therapy for a seizure disorder, and the mothers of 130 of these children were interviewed by telephone. The two investigations yielded similar results. Teachers were often not informed about the overt features of the seizures, side effects of the medication, or seizure management, even in the case of students who experienced seizures and/or side effects in school. In 70% of the cases, teachers were involved in either evaluating the response to treatment, administering medication, or managing (coping with) seizures in the classroom. They usually interacted with parents or other school personnel; direct communication with the physician was virtually nonexistent. Over one-third of the students were rated as more drowsy or sleepy than their peers, and, according to teachers, drug-induced impairments in adaptive behavior were a common problem. Collectively, these data suggest that in many cases, no one has assumed responsibility for coordinating the delivery of services for psychosocial treatment needs. RESUME Il est reconnu depuis longtemps que le personnel scolaire peut jouer un role important dans le traitement des crises. Afin de mieux comprendre son implication dans le processus therapeutique deux etudes de programmes d'education speciale ont ete menees dans l'ensemble d'un etat. Les professeurs ont rempli des questionnaires concernant 315 eleves de leur classe actuelle en traitement pour crises, et les meres de 130 de ces enfants ont ete interviewees par telephone. Les deux investigations ont abouti a des resultats similaires. Les professeurs n'etaient souvent pas informes des caracteres des crises, des effets collateraux des medicaments, de la conduite a tenir envers les crises, meme dans le cas d'eleves ayant des crises ou des effets secondaires a l'ecole. Dans 70% des cas les professeurs etaient impliques soit en appreciant la re-ponse au traitement, soit en administrant les medicaments, soit en s'occupant des crises en classe. Ils etaient habituellement en rapport avec les parents ou les autres personnels de l'ecole; la communication directe avec le medecin etait virtuellement inexistante. Plus d'un tiers des eleves etaient consideres comme plus somnolents ou endormis que leurs camarades, et, selon les professeurs, les troubles du comportement provoques par les medicaments etaient un probleme habituel. Toutes ces donnees suggerent que dans la plupart des cas personne n'a pris la responsabilite de coordonner les actions necessaires en vue d'un traitement psychosocial. RESUMEN Es sobradamente conocido que los empleados de las escuelas pueden jugar un papel importante en el tratamiento de los desordenes convulsivos. Con el fin de comprender mejor su participacion en el proceso de tratamiento, se han realizado dos estudios de pro-gramas de educacion especial a lo largo del Estado. Losprofesores cumplimentaron cuestionarios para 315 alumnos que, en sus clases actuales, recibian drogas para control de un desorden convulsivo y las madres de 130 de esos ninos participaron mediante entrevistas telefonicas. Estas dos investigaciones condujeron a re-sultados similares. Los profesores, con frecuencia, no habian sido informados acerca de los componentes mas aparentes de los ataques, de los efectos secun-darios de la medicacion o del manejo de los ataques, incluso referentes a los estudiantes que experimenta-ban ataques y/o efectos secundarios en la escuela. En un 70% de los casos, los profesores participaban en la valoracion de la respuesta terapeutica, en la administracion de la medicacion o en el manejo y en la tolerancia de los ataques en clase. Generalmente compartian sus actos con los padres u otros empleados en la escuela pero la comunicacion directa con el medico era virtualmente inexistente. Se observo que mas de un tercio de los estudiantes estaban mas somnolientos que el resto y, segun los profesores, las dificultades en el comportamiento adaptativo derivadas de la medicacion, eran un problema comun. Colectivamente, esta informacion sugiere que, en muchos cases, nadie ha asumido la responsabilidad de coordinar los servicios para llevar a cabo las necesidades psicosociales del tratamiento. ZUSAMMENFASSUNG Es ist seit langem anerkannt, das das Schulpersonal eine wichtige Rolle bei der Behandlung von Anfallser-krankungen spielen kann. Um zu einem besseren Verstandnis dieser Rolle zu gelangen, wurden 2 landes-weite Studien uber spezielle Erziehungsprogramme durchgefuhrt. Die Lehrer fullten Fragebogen fur 315 Schuler ihrer Klasee aus, die eine antikonvulsive Therapie erhielten, die Mutter von 130 dieser Kinder wurden uber Telephon interviewt. Beide Unter-suchungen erzielten ahnliche Resultate. Die Lehrer waren haufig nicht uber offenkundige Anfallsbilder, medikamentose Nebenwirkung oder die Behandlung von Anfallen informiert, selbst wenn Anfalle oder medicamentose Nebenwirkungen in der Schule auftraten. In 70% der Falle waren die Lehrer weder bei der Bewertung der Behandlung, der Gabe von Medikamenten oder an der Behandlung bei Auftreten von Anfallen wahrend des Unterrichts beteiligt. Interak-tionen fanden gewohnlich mit Eltern oder anderem Schulpersonal statt, eine direkte Kommunikation mit dem behandelnden Arzt gab es nicht. 1/3 der Schuler wurde als weniger aufmerksam oder miider im Ver-gleich zu den anderen Schtilern beurteilt, eine medikamentose Beeintrachtigung des Verhaltens war ein haufiges Problem. Zusammengenommen lafit sich aus den Daten schliefien, das sich niemand fur psycho-soziale Behandlungsbelange zustandig fuhlte.

Here we review the major inherited convulsive disorders found in mice and discuss their possible relationship to specific clinical seizure disorders in humans. These mouse disorders include audiogenic seizures, the epilepsy (El) mutation, spontaneous seizures, the tottering/learner syndrome, cerebellar abnormalities, myelin disorders, and alcohol withdrawal seizures. Some of these disorders are symptomatic and others are idiopathic. We find that for many major types of epilepsy in humans there exists a similar counterpart in mice. Because the genetic constitution of the mouse is better known and more easily manipulated that that of other mammalian species, the mouse may serve as an excellent animal model for genetic and biochemical studies of epilepsy.

Epileptic syndromes are clusters of signs and symptoms regularly occurring together. These may include type of seizure(s), time and circumstances of occurrence, and neurological and EEG findings. Some syndromes have common signs and a predictable course (e.g., benign rolandic epilepsy). Others, such as absence seizures, are less specific and may include several subgroups with different outcomes and different associated features. Still others are rather loose collections of a few common characteristics irregularly linked together. Widely accepted syndromes in childhood include the West syndrome and Lennox-Gastaut syndromes, several myoclonic syndromes, febrile seizures of infancy, absence epilepsy, benign partial epilepsy, and juvenile myoclonic epilepsy. If the concept of epileptic syndromes is to be practically useful, it should be limited to clusters that are unequivocally identifiable. Heterogeneous epileptic syndromes such as West syndrome or absence epilepsies give only a limited guide to prognosis but may be important in determining investigations and treatment. A classification based on syndromes has the advantage of dispensing with most assumptions required by other systems. However, it cannot cover all the clinical aspects of epilepsy and gives no guide to the pathophysiology, genetics, and etiology of a seizure disorder. The ultimate goal remains the delineation of disease entities.

Summary: Cortical surface electrodes and bipolar depth electrodes were implanted stereotaxically in the ventral posterolateral and paracentral thalamic nuclei, amygdala, hippocampus, and putamen in adult cats to determine the progressive involvement of these structures in the generalization of an experimentally induced seizure disorder. Prior to (45–65 days) and following (50–70 days) subpial injection of 0.04 ml of aluminum hydroxide in the sensorimotor cortex, 30 min EEG records were obtained regularly in each animal. At the time of aluminum hydroxide injection, there was no persistent abnormal EEG activity resulting from electrode implantation. In all animals, intermittent slow waves and epileptiform activity appeared in subcortical or extrafocal structures prior to the development of epileptiform activity in the primary focus. These abnormalities were frequently associated with brief clinical seizures and were clearly independent of abnormal activity in the primary focus. Only later in the development of the seizure disorder was activity in the secondary foci observed to be dependent on frequent epileptiform activity in the primary focus. These results demonstrate that multiple, independent foci develop in subcortical structures before the occurrence of a well developed, fully “mature” cortical primary focus. RESUME Des electrodes corticales de surface et des electrodes bipolaires de profondeur ont ete implantees stereotaxiquement dans les nuclei posterolateraux ventraux et paracentraux thalamiques, les amydales, l'hippocampe et le putamen de chats adultes afin de determiner la part progressive jouee par ces structures dans le cadre general d'une attaque induite par experimentation. Avant (de 45 a 65 jours) et suivant (de 50 a 70 jours) l'injection sous-piale de 0.04 ml d'hydrate d'alumine dans le cortex sensori-moteur, des resultats de EEG d'une demi-heure ont ete obtenu sur chaque animal. Au moment de Tinjection d'hydrate d'alumine, il n'y a pas eu d'activite anormale persistente de EEG provenant de l'implantation d'electrodes. Dans chaque animal, de lentes ondes intermittentes et une activite epileptiforme ont ete relevees dans les structures sous-corticales et extrafocales avant le developpement d'une activite epileptiforme dans le foyer primaire. Ces anormalities ont souvent ete accompagnees de breves crises cliniques et etaient nettement independantes de toute activite anormale dans le foyer primaire. Ce n'est que plus tard dans la progression de l'attaque que nous avons observe dans les foyers secondaires une activite dependente de l'activite epileptiforme frequente dans le foyer primaire. Ces resultats demontrent que de multiples foyers independants se developpent dans des structures sous-corticales avant la formation d'un foyer cortical primaire “mur” et bien developpe. ZUSAMMENFASSUNG Ausgewachsenen Katzen wurden kortikale Oberflachenelektroden und bipolare Tiefenelektroden stereotaktisch in den ventralen posterolateralen und parazentralen Thalamuskern, das Amygdalon, in Hippocampus und Putamen implantiert. Sie sollten das allmahliche Einbeziehen dieser Strukturen in die Generalisation eines experimentell induzierten Krampfes erkennen lassen. Eine dreissigminutige EEG-Aufzeichnung wurde bei diesen Tieren re-gelmasig vorgenommen und zwar 45 bis 65 Tage vor und 50 bis 70 Tage nach subpialer Injektion von 0,04 ml Aluminumhydroxyd in den sensomotorischenCor-tex. Zur Zeit der Injektion von Aluminum hydroxyd bestand keine abnorme EEG-Aktivitat als Folge der Elektroden-implantierung. Bei alien Tieren zeigten sich intermittierende langsame Wellen und epileptiforme Aktivitat subkortikal oder extrafokal vor der Entwicklung der epileptiformen Aktivitat im Gebiet des primaren Fokus. Diese Abnormitaten waren haufig verknupft mit kurzen klinischen Anfallen. Sie waren eindeutig unabhangig von abnormer Aktivitat im primaren Fokus. Erst spater in der Entwicklung des Anfallsleidens zeigte sich die Aktivitat in den Sekun-darfoci abhangig von haufiger epileptiformer Aktivitat im Primarfokus. Diese Ergebnisse zeigen, das sich multiple unabhangige Foci in subkortikalen Strukturen vor dem Erscheinen eines wohl entwickelten reifen kortikalen Primarfokus entwickeln.

Summary: We have investigated the comparative effects of estradiol benzoate (EB), the antiestrogenclomiphene citrate (CC), and the progesting medroxyprogesterone acetate (MPA) on seizures induced by systemic injection of kainic acid (15 mg/kg i.p.) in male and female rats. Subcutaneous administration for 10 days of EB (10 μg/kg) or high doses of CC (50 mg/kg) significantly potentiated kainate-induced seizures, with the effect being more pronounced in male animals. Doses of 2.5 mg/kg of CC potentiated kainate-induced seizures in male rats but were ineffective in female rats. Low doses of CC (0.5 mg/kg) exhibited a mild anticonvulsant effect in both sexes. repeated administration of MPA (2.5 mg/kg) partially protected female animals against kainate-induced seizures; in male animls, MPA induced a 30% increase in the seizure severity score, although the difference fro the score of control male rats was not significant. These data suggest hat sex steroids influence kainate-induced seizures in a sex-dependent manner and that the effects of the antiestrogen CC are dose dependent. This should be taken into account in view of a possible use of CC and MPA in hormonal therapy for seizure disorders. RESUME one a etudie les effets du benzoate d'oestradiol (EB), de l'antioestrogene citrate de clomifene (CC) et du progestatif acetate de medroxyprogesterone (MPA) sur les crises produites par l'injection d'acide kainique (15 mg/kg par voie intraperitoneale) a des rats mâles et femelles. L'administration sous-cutanee pendant 10 jours d'EB (10 μg/kg), ou des doses elevees de CC (50 mg/kg) potentialisent significativment les crises induites par l'acide kainique, cet effet etant plus prononce chez les mâles. Des doses de 2,5 mg/kg de CC potentialisent les crises induites par l'acide kainique chez les mâles, mais pas chez les femelles. De faibles doses de CC (0,5 mg/kg) ont un effet anticonvulsivant faible dans les 2 sexes. L'administration repetee de MPA (2,5 mg/kg) protege partiellement les femelles contre les crises induites par l'acide kainique. Chez les mâles, le MPA entraine une augmentation de 30% du score de severite des crises, quoique cependant la difference avec le score des mâles de controle ne soit pas significative (p < 0.05).Les donnees suggerent que les steroides sexuels influencent les crises induites par l'acide kainique d'une maniere dependant du sexe, et que les effets de l'antioestrogene CC sont dose-dependants. Cela doit etre pris en consideAration dans l'utilisation possible du CC et du MPA dans le traitement hormonal des epilepsies. RESUMEN En ratas hembras y machos hemos investigado los efectos comparativos del benzoato de estradiol (EB), del antiestrogeno citrato clomifeno (CC) y del progestin acetato medroxiprogesterona (MPA) sobre los ataques inducidos mediante la inyeccion sistomica de acido kainico (15 mg/kg, intraperitonealmente). La administracion subcutonea durante 10 dias de EB (10 μg/kg) o dosis altas de CC (50 mg/kg) potenciaron significativamente los ataque inducidos por el kainato, siendo este efecto mas pronunciado en los animales macho. Dosis de 2.5 mg/kg de CC potenciaron los ataques inducidos por el kainato en los machos pero no produjeron efectos en las hembras. Dosis bajas de CC (0.5 mg/kg) mostraron un efecto anticonvulsivo ligero en ambos sexos. La administracion repetida de MPA (2.5 mg/kg) protegieron parcialmente a las hembras de los ataques inducidos por el kainato; en los machos, el MPA produjo un 30% de incremento en la escala de severidad de los ataques aunque la diferencia con la escala de los machos control no fue significativa (p > 0.05). Toda esta informacion sugiere que los esteriodes, segun el sexo, influencian los ataques inducidos por el kainato de un modo sexo-dependiente y que los efectos del antiestrogeno CC son dosis-dependientes. Estos datos podrian ser tenidos en cuenta en vista de una posible utilizacion del CC y del MPA en la terapeutica hormonal de los trastornos convulsivos. Wir untersuchten die Wirkungen von Oestradiolbenzoat (EB), dem Antioestrogen Clomiphencitrat (CC) und dem Progestinmedroxyprogesteronacetat (MPA) auf Anfalle, die durch die systemische Injektion von Kainsaure (15 mg/kg intraperitoneal) bei mannlichen und weiblichen Ratten hervorgerufen wurden, 10-tagige subkutane Anwendung von EB (10 ug/kg) oder hohe Dosen von CC (50 mg/kg) potenzierten in signifikanter Weise Kain-induzierte Anfalle. Diese Wirkung trat besonders bei mannlichen Tieren auf. Dosen von 2, 5 mg/kg CC potenzierte Kainat-induzierte Anfalle bei maannlichen aber nicht bei weiblichen Ratten. Niedrige Dosen von CC (0, 5 mg/kg) zeigten eine milde antikonvulsive Wirkung bei beiden Geschlechtern. Die wiederholte Anwendung von MPS (2, 5 mg/kg) schutzte weibliche Tiere partiell gegen Kainatinduzierte Anfalle. Bei mannlichen Ratten bewirkte MPA eine 30-prozentige Zunahme der Schwere der Anfalle, obwohl der Unterschied zu dem Score der Kontrolltiere nicht signifikant war (p > 0,05). Diese Ergebnisse legen nahe, daβ Geschlechtssteroide Kainat-induzierte Anfalle in einer geschlechtsabhangigen Art und Weise beeinflussen und daβ die Wirkungen des Antioestrogens CC dosisabhangig sind. Das muβ bei einer moglichen Anwendung von CC und MPA in der Hormontherapie von Anfallsleiden [berucksichtigt werden].

Summary: Epilepsy is probably an inherited disorder. Animal studies, clinical studies on relatives of epileptics, and the study of the EEG both in patients and their relatives have provided evidence for this. Different specific modes of inheritance have been discussed. Since the HLA system offers one of the best sets of human genetic markers found so far, our aim was to investigate the occurrence of HLA antigens and haplotypes in children with different types of primary epilepsy and their families. The sample consisted of 53 mentally normal Caucasian children with epilepsy, 104 parents and 72 siblings. The types of epilepsy in the probands were divided into “benign epilepsy of childhood” (21), absences (18) in the haplotype study, comprising 51 families (two children were excluded), tonic-clonic seizures (9), and complex partial seizures (5). With regard to HLA antigens, there was no difference between probands and their parents, respectively, and a control sample. Compared with the occurrence of the most common haplotypes in controls, there was a statistically significant low incidence of the haplotype A1,B8 in probands and parents for benign epilepsy of childhood, as well as for the total material. Due to its common occurrence in the general population, the haplotype A1,B8 must offer some kind of biological advantage, for example, carrying “protecting” genes against certain noxious mechanisms. Lack of this haplotype may explain a lowered immunologic defense mechanism, resulting in increased risk for the appearance of seizure disorders. A comparison of the HLA haplotype distribution among siblings and probands in the 17 families with more than one affected child suggested dominant inheritance, most evident for benign epilepsy of childhood. However, since the frequency of no shared haplotypes is fairly high, the results must be interpreted cautiously. Investigations with HLA typing in larger populations of epileptic individuals and their families may give a more reliable and conclusive answer regarding the genetic character of epilepsy. RESUMEN La epilepsia es, probablemente, un trastorno heredi-tario. Esta afirmacion se ha evidenciado en estudios en animates y chinicos, en los familiares de los epi-lepticos y en el analisis de los EEG de los pacientes y familiares. Se han propuesto diversos modos es-peci'ficos de herencia. Puesto que el sistema HLA es uno de los mejores marcadores de la genetica humana, se ha intentado investigar la presencia de antigenos HLA y haplotipos en ninos con diversos tipos de epilepsia primaria y en hsus familias. El material consiste en 53 ninos normales con epilepsia, 104 padres y 72 hermanos. Los tipos de epilepsia en los probandos se dividieron en: “epilepsia benigna de la infancia” (21), ausencias (18 en en es-tudio de haplotipo, que consto de 51 familias con dos ninos excluidos), ataques tonico-clonicos (9) y ataques parciales complejos (5). Con respecto a los anti'genos HLA no se observaron diferencias entre los probandos y sus padres con respecto al grupo control. Se encon-tro una reducida incidencia del haplotipo Al, B8 es-tadisticamente significativa en los probandos y padres en el grupo “epilepsia benigna de la infancia,” asi como en el grupo total, con respecto a la presencia de los haplotipos mas comunes en los controles. Debido a la frecuente existencia del haplotipo Al, B8 en la pob-lacion general, debe ofrecer algun tipo de ventaja biologica como por ejemplo, poseer genes protectores contra ciertos mecanismos nocivos. La falta de este haplotipo puede explicar una reduccion de los mecanismos inmunologicos defensivos que resultan en un incremento del riesgo de la aparici6n de un tras-torno comicial. La comparacion de la distribucion del haplotipo HLA entre los hermanos y los probandos en 17 familias, con mas de un nino afecto, ha sugerido la existencia de una tendencia hereditaria mas evidente para la “epilepsia benigna de la infancia”. Sin embargo, puesto que la frecuencia de haplotipos no com-partidos es relativamente elevada, los resultados deben ser interpretados con cautela. Las inves-tigaciones sobre la tipificacion del HLA en amplias poblaciones de individuos epilepticos y sus familias pueden otorgar respuestas mas fiables y conclusivas, en lo que concierne al estado genetico de la epilepsia. ZUSAMMENFASSUNG Die Epilepsie ist wahrscheinlich eine vererbbare Storung. Dafur sprechen Tieruntersuchungen, klinische Untersuchungen von Verwandten Anfallskranker und die Untersuchungen des EEG sowohl bei Patienten wie ihren Verwandten. Un-terschiedliche Erbgange wurden diskutiert. Da das HLA-System eines der besten “sets” genetischer Marker fur den Menschen bietet, lag die Absicht nahe, das Auftreten von HLA Antigenen und Haplotypen bei Kindern mit unterschiedlichen Arten primarer Epilepsien und ihren Familien zu untersuchen. 53 kaukasische, geistig normale Kinder mit Epilepsie, 104 Eltern und 52 Geschwister wurden untersucht. Die Epilepsietypen der Probanden wurden unterteilt in be-nigne Epilepsie des Kindesalters (21), Absencen (18 in der H'aplotypuntersuchung, entsprechend 51 Familien, 2 Kinder wurden ausgeschlossen), tonisch-klonische Anfalle (9), komplexe Partialanfalle (5). Hinsichtlich HLA-Antigenen existierten keine Unterschiede zwischer Probanden und ihren Eltern und den Kontrollen. Verglichen mit dem Auftreten der haufigsten Haplotypen bei den Kontrollen, traten bei alteren Probanden mit benigner Epilepsie des Kindesalters ebenso wie im gesamten Material statistisch signifikant seltener Haplotypen Al, B8 auf. Entsprechend ihrem allgemeinen Vorkommen in der Bevolkerung miissen die Haplotypen Al, B8 irgendeine Art biologischen Vorteil bieten, z.B. Protektorgene gegen bestimmte schadliche Mechanismen tragen. Das Fehlen dieser Haplotypen konnte einen verminderten immunologis-chen Abwehrmechanismus kennzeichnen, der filr das erhohte Risiko einer Anfallskrankheit verantwortlich ist. Der Vergleich der HLA Haplotypen unter Gesch-wistern und Probanden der 17 Familien, in denen mehr als 1 Kind erkrankt ist, lassen eine dominante Vererbung ganz besonders fur die benigne Epilepsie im Kindesalter vermuten. Da jedoch die Haufigkeit verschiedener Haplotypen ziemlich hoch ist, miissen die Ergebnisse vorsichtig interpretiert werden. Untersuchungen groBerer Kollektive epileptischer Kranker und ihrer Familien mit HLA-Typisierung versprechen verlasslichere und genauere Antworten hinsichtlich der Genetik der Epilepsien.

Infantile spasms constitute a severe seizure disorder unresponsive to standard anticonvulsants. Both prednisone and adrenocorticotropic hormone (ACTH) have produced remission of seizures in some patients. The mechanisms of action of these hormones are not known. Eight infants with infantile spasms were treated with prednisone for 2 weeks. This controlled the seizures in two patients. In the remaining six patients, prednisone was continued and ACTH was added. This treatment produced cessation of spasms in four patients. Serum prednisone and cortisol were measured at a number of points during treatment. In infants receiving prednisone and then prednisone plus ACTH, serum cortisol was suppressed to about one-quarter of baseline levels with the initiation of prednisone, and remained suppressed during ACTH administration. We conclude that ACTH can exert its effect on infantile spasms in the setting of adrenal suppression, and can act without stimulating endogenous cortisol production. A CNS site of action is suggested and should be sought.

UMMARY Detailed clinical, electrographic, and neuropathologic studies are reported in the case of a 4-month old child who had showed infantile myoclonic spasms, some focal seizures and a variety of severe EEG changes including hypsarhythmia. ACTH therapy had been given. In the pathologic studies of such cases now available, the presence of prolonged terminal illness or obvious gross structural brain disease makes it difficult to decide whether observed pathologic findings are related to the seizure disorder or to other factors. A sudden untimely death early in this illness, therefore, makes this case of exceptional interest. Much of the brain appeared normally developed for age and anatomically intact. Positive findings included an unusual malformed gyrus in the right parietal lobe, changes in the cytoarchitecture of the Betz cells, generally increased microglia and astrocytes, and several foci of neuronophagia in frontal cortexwithminimaldemyelini-zation in the subjacent white matter. It is speculated that the relative paucity of findings may be related to the short duration of symptoms (3 months) or the effect of ACTH. A few tentative electrographic and clinical-pathologic correlations are discussed. RESUME Il s'agit de l'etude clinique, electrographique et neuropathologique detaillee d'un enfant de 4 mois, ayant ete traite par 1'A.C.T.H. pour des spasmes myocloniques infancies avec quelques crises focales et des modifications importantes et variees de 1'E.E.G., dont une hypsarythmie. Dans les observations pathologiques de cas analogues publicees jusqua ce jour, la presence d'une maladie terminale prolongee ou d'une affection cerebrale organique grossiere, rend difficile dedecider si les constatations anatomo-patliologiques dependent des crises ou d'autresfacteurs. C'est pour cette raison que la mort subite et prematuree de notre patient rend son observation anatomique particulierement interessante. Une grande partie du cerveau semble normalement developpee et anatomiquement intacte compte tenu de l'âge du malade. Les anomalies observees comprennent un gyrus mal forme dans le lobe parietal droit, des modifications de la cytoarchitecture des cellules de Betz, une augmentation generate de la nevroglie et des astrocytes et plusieurs foyers de neuronophagie dans le cortex frontal avec demyelinisation minimum de la substance blanche sous-jacente. L'auteur pense que la pauvrete relative de ces constatations peut etre attribuee a la courte duree des symptomes (3 mois) ou a 1'effet de 1'A.C.T.H. Suit discussion comportant quelques tentatives pour etablir des correlations anatomo-electro-clinique.

UMMARY 1 A rare electrographic pattern of periodically recurring focal discharges, mainly in the parieto-occipital area, has been described. 2 The constancy of the rate of discharges under various conditions has been noted. In some patients, the discharge seems to occur at preferential time intervals after the heart beat. 3 Most of the patients were adults with histories of a seizure disorder, especially with a focal aspect, and also with neurological symptoms, mainly motor deficits, and psychiatric signs, but the seizures of the majority of patients temporarily lessened with anticonvulsant medication. 4 The most common etiological factor was cerebrovascular disease; the possibility that prolonged hypoxia or anoxia may be responsible for the discharges is discussed. 5 A phenomenon, possibly related to the recurring focal discharge in the human, has also been noted in the monkey. REASUMEA 1 Les auteurs deAcrivent un type rare de deAcharges focaliseAes qui se reApeGtent de facLon peAriodique et surviennent principalement dans la reAgion parieAto-occipitale. 2 La peAriodiciteA du pheAnomeGne est constante dans des conditions varieAes et, chez quelques patients, il semble que les deAcharges surviennent preAfeArentiellement un certain temps apreGs les pulsatiAons cardiaques. 3 Laplupart des patients eAtaient des adultes preAsentantdes crises aG seAmeAiologie focale ainsi que des symptoCmes neurologiques, principalement deAficitaires, et des manifestations psychiatriques; les crises de la plupart de ces sujets furent atteAnueAes par une theArapeutique anti-convulsive. 4 Le facteur eAtiologique le plus commun eAtait repreAsenteA par une affection vasculare ceAreAbrale; le roCle d'une hypoxie prolongeAe ou d'une anoxie peut done eCtre envisageA a l'origine de ces deAcharges. 5 Un pheAnomeGne qu'il est possible derattacher aG ces deAcharges focaliseAes reAcurrentes de l'homme a eAteA noteA chez les singes.

Summary: In this review, we assess the following six aspects of the epidemiological literature addressing the association between perinatal factors and seizure disorders (mainly of the grand mal type): (1) study design; (2) selection of cases; (3) selection of controls; (4) definition and ascertainment method of seizures; (5) definition and ascertainment method of perinatal factors; (6) sample size and power. Needed are studies of perinatal factors in a homogeneous group of seizure patients, such as those with grand mal seizures. Because of the rarity of the seizure disorder, most follow-up studies of this subject suffer from inadequate sample size. Improvements are suggested for case-control studies, which appear, by necessity, to be the more appropriate design. RESUME Cette revue etudie six aspects de la litterature epidemiologique concernant l'association entre les facteurs perinataux et les manifestations epileptiques (en particulier du type Grand Mal): (1) protocole d'etude, (2) selection des cas, (3) selection des controles, (4) definition et methode d'evaluation des crises, (5) definition et methode d'evaluation des facteurs peri-nataux, (6) taille et valeur discriminante de l'echantillion. Il est necessaire d'avoir des etudes des facteurs perinataux dans un groupe homogene de patients epileptiques, comme ceux avec crises Grand Mal. Etant donne la rarete de ce type d'epilepsie la plupart des etudes a long terme souffrent du volume inadequat de l'echantillon de patients. Les auteurs suggerent des ameliorations pour les etudes de cas-controles qui paraissent etre, par necessite, le protocole le plus adequat. RESUMEN En esta revision determinamos seis aspectos de la literatura epidemiologica que tratan de la asociacion entre factores perinatales y trastornos convulsivos (fundamentalmente del tipo gran mal): (1) diseno del estudio, (2) seleccion de casos, (3) seleccion de los controles, (4) definicion y metodo de identificacion de los ataques, (5) definicion y motodo de identificacion de los factores perinatales, (6) tamano y poder de la muestra. Son necesarios estudios de los factores perinatales en un grupo homogeneo de pacientes con ataques, tales como los que tienen ataques de gran mal. Dada la rareza del trastorno convulsivo, la mayor parte de los estudios de seguimiento sobre este tema, sufren de tamanos de muestra inadecuados. Se sugieren mejorias para los estudios de caso-control que parencen ser, pro necesidad, el diseno mas apropiado. ZUSAMMENFASSUNG In dieser Ubersicht werden 6 Gesichtspunkte aus der epidemiologischen Literatur bezuglich der Assoziation zwischen perinatalen Faktoren und Anfallsleiden (hauptsachlich Grand mal) untersucht: 1. Design der Studie, 2. Fallauswahl, 3. Auswahl der Kontrollen, 4. Definition der Anfalle und ihrer methodischer Absicherung, 5. Definition der perinatalen Einflusfaktoren und deren methodische Absicherung, 6. Grose der Untersuchungsgruppen und der Aussagekraft. Notwendig sind Untersuchungen der perinatalen Faktoren in homogenen Patientengruppen zum Beispiel mit Grand mal Anfallen. Wegen der Seltenheit von Anfallsleiden ist bei den meisten Follow-up-Studien die Patientengrose zu gering. Verbesserungen sind wunschenswert fur Fall-kontrollierte Studien, die notwendigerweise die adaquatere Untersuchungsform darstellen.

Profiles of free amino acids in cerebrospinal fluid (CSF) were determined by high-performance liquid chromatography for 20 nonneurologic control patients and 12 patients with infantile spasms. Statistical comparisons showed significantly elevated levels of lysine (p less than 0.001) and the excitatory neurotransmitter, glutamate, (p less than 0.01) for the infantile spasms group as compared to the nonneurologic control group. When the infantile spasms patients were subdivided according to the presence or absence of etiologic associations, highly elevated amino acid levels were observed only in CSF from patients of the symptomatic subgroup. The idiopathic subgroup showed levels of free amino acids that were not statistically different from those of the nonneurologic control group. These results indicate that while abnormalities of amino acid metabolism often accompany infantile spasms, no specific pattern of the major free amino acids in CSF appears to be directly related to this seizure disorder. Elevated levels of the excitatory amino acids, aspartate and glutamate, do not necessarily accompany infantile spasms, and in this study were only observed in symptomatic patients.

To test the effect of maternal seizure disorders on prenatal structural development, the mouse neurological mutant quaking (qk) was used. Administering phenytoin in the drinking water of females homozygous for the quaking allele reduced the frequency of tonic-clonic seizures typical for this mutant from a background rate of 2.06 to 0.34 seizures per mouse day. As the seizure frequency decreased, the percentage of fetuses exhibiting abnormalities associated with the mouse fetal hydantoin syndrome increased from 0 to 77. As in previous studies with the mouse syndrome, the increase in malformations was associated with increased maternal serum phenytoin levels. The results of this study indicate that the maternal serum phenytoin level, and not the maternal seizure disorder, is the etiologic agent responsible for the malformations observed in this syndrome.

A group of patients with benign epilepsy of children with centrotemporal EEG foci, consisting of 40 patients reported 10 years ago, has been reinvestigated. Thirty-seven of these patients were reinvestigated. They were 26-34 years old (mean, 29 years). The observation time from the first seizure was 13-27 years (mean, 21 years). Thirty-six patients had been seizure free for 14-23 years (mean, 18.5 years); 32 patients had stopped medication 5-22 years ago (mean, 18.5 years); and 5 patients had had problems in obtaining their drivers' licenses. No epileptic discharges were found in those 24 patients who volunteered for a new EEG study. The conclusion is drawn that this common seizure disorder in children has a good long-term prognosis.

Developing antiepileptic agents that are specifically tailored to a patient's individual biochemistry has long been a goal of neurology. Three patients who had hyperuricosuria combined with a seizure disorder that failed to respond to traditional anticonvulsants are described. The patients had the best control of their seizure disorder when a specific metabolic drug, allopurinol, was used as an anticonvulsant. All three patients had onset of the seizure disorder at 22 months of age, a finding possibly related to maturation of purine enzymes. Because elevated uric acid levels in the immediate postictal period may occur in seizure patients, the presence of an elevated uric acid clearance in seizure-free periods is needed to consider the diagnosis of an allopurinol-responsive seizure problem in any individual patient. In the two patients past the onset of puberty, lowering (one case) and cessation (other case) of the dose of allopurinol has been possible.

UMMARY If the patterns of seizure disorders in parents and offspring could be assessed accurately, some insight might be gained into the relative role of genetic and environmental factors in the development of convulsive disorders. In this study, 908 children born in a Rochester, Minnesota hospital from 1922 through 1972 whose mother or father had a verified and classified diagnosis of seizure have been followed from birth for evidence of any convulsive episode or seizure disorder. The observed numbers of various types of convulsions in the offspring are compared to the expected number based on local age-specific incidence rates. The outstanding finding was that a higher than expected number of children whose mothers had epilepsy also suffered from seizures (epilepsy or febrile convulsions), whereas no such increase was detected among the children of affected fathers. RESUME Si l'on pouvait evaluer avec precision le type d'epilepsie chez les parents et les descendants, on pourrait obtenir des renseignements sur le role par les facteurs genetiques et l'environnement dans le developpement de Pepilepsie. On a essaye de preciser l'existence de tout episode convulsif ou paroxystique chez 908 enfants suivis des la naissance, nes dans un Hopital a Rochester, Minnesota, entre 1922 et 1972, et dont le pere ou la mere ont eu une forme d'epilepsie bien caracterisee. Le nombre des differentes convulsions observees chez les enfants avec un parent epileptique, a ete comparea celui qu'on peut s'attendre a observer dans une population du meme âge. Le resultat significatif est que les enfants de mere avec epilepsie ont eu des crises (epilepsie ou convulsions febriles) en nombre plus eleve que la normale, tandis qu'il n'y avait pas une telle augmentation chez les enfants de pere epileptique. RESUMEN Si se pudieran conocer con seguridad los patrones de los desordenes convulsivos en los padres y en los hijos, se podria intentar aclarar el papel relativo que los factores geneticos y ambientales juegan en el desarrollo de los desordenes convulsivos. Se han observado, desde su nacimiento, 908 ninos nacidos en un hospital de Rochester, Minnesota, desde 1.922 a 1.972. cuyos padres o madres habian sido diagnosticados de desordenes convulsivos. Se han comparado el numero y los diversos tipos de convulsiones que ocurrieron en los ninos con el numero teorico de ataques que deberian haber tenido segun los indices de frecuencia para su edad y region geografica. El hallazgo sorprendente consistio en que un mayor numero de ninos que el calculado y cuyas madres habian tenido epilepsia, desarrollo ataques (epilepsia o convulsiones febriles) mientras que no se observe tal incremento en ninos cuyos padres padecian epilepsia. ZUSAMMENFASSUNG Die genaue Kenntnis von Anfallsmustern bei Eltern und Kindern muβte Erkenntnisse uber die relative Bedeutung genetischer und exogener Faktoren fur die Entwicklung eines Krampfleidens liefern. In dieser Untersuchung wurden 908 Kinder, die im Krankenhaus in Rochester, Minnesota von 1922 bis 1972 entbunden wurden und deren Mutter oder Vater ein bestatigtes und klassifiziertes Anfallsleiden hatten, von Geburt an verfolgt, um das Auftreten konvulsiver Episoden oder eines Anfallsleidens zu erkennen. Die Haufigkeit verschiedener Anfallsarten bei den Kindern wurde verglichen mit Erwartungswerten, entsprechend lokalen altersabhangigen Haufigkeitsraten. Der hervorstehende Befund bestand darin, daβ eine hohere als die erwartete Anzahl von Kindern, deren Mutter eine Epilepsie hatte, auch Anfalle (epileptische oder febrile) bekamen, wahrend eine solche Zunahme bei Kindern anfallskranker Vater fehlte.

Summary: We reviewed the long-term outcome of 39 adults whose electroencephalograms (EEGs) showed acute periodic lateralized epileptiform discharges (PLEDs). Parameters studied included outcome, presence or absence of a seizure disorder (both prior to and subsequent to the PLEDs), seizure type and frequency, and etiology of the PLEDs. The mortality rate was 41%, as 16 of 39 patients died during their hospitalization or within 2 months of discharge. Of the 23 survivors, 7 (30%) had a significant decline in their ability to provide self-care. A recent stroke (13 patients, 33%) was the most frequent cause of PLEDs. In this subgroup particularly, poor outcome, i.e., death or functional decline, was common (10 of 13 patients). Thirty-six patients had seizures just prior to or during the hospitalization when PLEDs were observed; focal motor seizures were the most common type (n = 22). Patients with a history of seizures prior to the occurrence of PLEDs continued to have seizures following discharge. In addition, 4 of 9 patients with new onset seizures associated with PLEDs had subsequent seizures. RESUME Nous avons etudie l'evolution a long terme chez 39 patients adultes qui avaient presente des decharges epileptiques peri-odiques lateralisees (DEPL). Les parametres suivants ont eteevalues: devenir, presence ou absence de manifestations critiques (avant et apres les DEPL), type et frequence des crises, etiologie des DEPL. Le taux de mortalite s'etablit a 41%: 16 des 39 patients moururent pendant 1'hospitalisation ou dans les 2 mois suivant la sortie. Parmi les 23 survivants, 7 (30%) ont pre-sente une diminution significative de leur autonomie. Un accident vasculaire recent represente la cause la plus frequente des DEPL (13 patients, 33%). Une evolution defavorable (deces ou perte d'autonomie) a ete particulierement frequente dans ce sous-groupe. 36 patients ont presente des crises juste avant ou pendant, l'hospitalisation pendant laquelle les DEPL ont ete constatees. Des crises partielles motrices ont eAte les plus fre-quemment constatees (n = 22). Les patients qui avaient presente des crises avant la survenue des DEPL ont continuea en avoir apres leur sortie de l'hopital. De plus, 4 patients sur les 9 qui avaient commencea preasenter des crises pendant la p6riode de DEPL ont continue a presenter des manifestations critiques par la suite. RESUMEN Se han revisado los resultados a largo plazo de adultos cuyos EEGs mostraron descagas epileptiformes lateralizadas period-icas agudas (PLEDs). Los parametros estudiados fueron: resultados, presencia o ausencia de desorden convuisivo (anterior o posterior a la presencia de PLEDs), tipo de ataques y frecuencia asi como la etiologia de las PLEDs. La mortalidad alcanzd el 41% y 16 de los 39 pacientes fallecieron durante su hospitaiza-cion o en los 2 primeros meses despues de la fecha de alta. De los 23 supervivientes, 7 (30%) mostraron un declinar significa-tivo en su capacidad de administrarse sus propios cuidados. Un ictus reciente (13 pacientes, 33%) fue la causa mas frecuente de las PLEDs. Particularmente en este subgrupo, los resultados mas pobres (muerte y deterioro funcional) fue un hallazgo comun (10 de 13 pacientes). Treinta y seis enfermos mostraron ataques inmediatamente, o durante la hospitalizacion, cuando las PLEDs fueron registradas; los ataques motores focales fueron el tipo mas comun (n = 22). Los enfermos que tenian una historia de ataques antes de la demostracion de las PLEDs con-tinuaron con los ataques despueas de su fecha de alta. Ademas 4 de 9 enfermos que iniciaron sus ataques asociados a las PLEDs, continuaron tenigndolos. ZUSAMMENFASSUNG Wir berichten uber die Spatprognose von 39 Erwachsenen mit periodisch lataralisierten epileptiformen Entladungen (PLEDs) im EEC Folgende Parameter wurden untersucht: Klinischer Verlauf, Auftreten von Anfallen (vor oder nach dem Auftreten der PLEDs), Anfallstyp und Frequenz sowie Atiologie. Die Mortalitatsrate betrug 41%, das heisst 16 der 39 Patienten starben wahrend des Krankenhausaufenthaltes oder innerhalb von 2 Monaten nach Entlassung. Von den ubrigen 23 Patienten zeigten 7 Patienten (30%) eine zunehmende Unselbstandigkeit. Wiederholte SchlagAnfalle (13 Patienten, 33%) war die haufigste Ursache der PLEDs. In dieser Untergruppe wa die Prognose am schlechtesten (Tod oder funktionelle Defizite bei 10 von 13 Patienten). 36 Patienten zeigten Anfalle vor oder wahrend des Krankenhausaufenthaltes, wenn PLEDs auftraten; fokal-motor-ische Anfalle waren der haufigste Type (n = 22). Patienten mit Anfallen vor dem Auftreten von PLEDs zeigten auch spater noch Anfalle. 4 von 9 Patienten mit neu aufgetretenen Anfallen zusammen mit den PLEDs zeigten auch weiterhin Anfalle.

Summary: In an isolated tribe in the interior of Tanzania the authors found that approximately 200 persons among an intake population of 10,000 persons presented at the bush clinic with kifafa, a convulsive seizure disorder that often leads to severe burns in those afflicted. Many of these patients showed parkinsonian symptoms and/or other neurological abnormalities and mental deterioration; there was high mortality. A frequent variant of the disease in children involved head nodding, which was often a precursor of later grand mal seizures. Transient psychotic episodes were seen in approximately 20% of all patients. The clinic patients were followed up to 10 years. The necessarily simple anticonvulsive treatment with phenobarbitone reduced the seizure frequency and intensity in the majority of cases. We shall discuss possible reasons for the high prevalence of seizure disorders in this tribe. Genetic factors appear to be of major importance and are reflected in the demonstrated high family incidence. Genetic analysis of the data does not support chromosomal, multifactorial, X-linked, or autosomal-dominant modes of inheritance. Analysis by the Li-Mantel method indicates that the segregation ratios are compatible with a hypothesis of single autosomal-recessive inheritance. Clinical and genetic data suggest that a hitherto unknown systemic disease of the central nervous system is highly prevalent in this isolated population. RESUME Les auteurs decrivent une maladie convulsivante, le “kifafa” (mot swahili designant cette affection), observee chez 200 sujets environ appartenant a une tribu isolee de la Tanzanie comportant 10.000 âmes; les porteurs de la maladie souffrent souvent de brulures graves secondaires a des chutes dans les feux domestiques. Un grand nombre de ces malades ont des symptomes parkinsoniens et/ou d'autres anomalies neurologiques, ainsi qu'une deterioration men tale; le taux de mortalite est eleve. Une variante frequemment observee chez L';enfant se manifeste sous forme de balancements de la tete qui constituent souvent un signe precurseur des crises de Grand Mai. Des episodes psychotiques transitoires sont observes dans 20% des cas. Certains malades ont ete suivis pendant 10 ans. Un traitement anticonvulsivant necessairement simple (phenobarbital seul) reduit la frequence et la gravite des crises chez la plupart des malades. Les auteurs discutent les diverses possibilityes pouvant expliquer L';importante prevalence de la maladie dans cette tribu. L';importance de facteurs genetiques est certaine et explique bien L';incidence familiale particulierement elevee. L';analyse genetique n'est pas en faveur d'un mode de transmission multifactorial, liea L';X ou dominant autosomique. Une etude par la methode de Li-Mantel montre que les rapports de segregation sont compatibles avec L';hypothese d'une heredite de type recessif autosomique. Ces donnees cliniques et genetiques demontrent L';existence d'une maladie systemique du systeme nerveux central jusqu'ici meconnue; cette affection est extremement repandue au sein de cette population isole. RESUMEN En una tribu aislada del interior de Tanzania los autores encontraron que, de una poblacion de 10.000 personas, unos 200 se presentaron en la clinica de campana con “kifafa”, un trastorno comicial que, a menudo, conducia a quemaduras graves a quienes lo padecian. Muchos de estos casos mostraron sinto-mas parkinsonianos y/u otras anomalias neurologicas, asi como deterioro mental y una mortalidad elevada. Una variante comun en ninos consistia en cabeceo que, a menudo, era un precursor de ataques de gran mal. Episodios psicoticos transitorios fueron vistos en un 20% de los casos. Los pacientes fueron seguidos hasta 10 anos y el tratamiento (forzosamente simple) con fenobarbitona redujo la frecuencia y la intensidad de las crisis en la mayoria de los casos. Los autores discuten posibles razones para esta elevada prevalencia de desordenes convulsivos en esta tribu. Factores geneticos parecen ser de la mayor importancia, como se demuestra por la alta incidencia familiar. Analisis genetico de los datos no apoya un modo de herencia cromosomico, multifactorial, ligado al cromosoma X o dominante autosomico. El analisis por el metodo de Li-Mantel indica que la relacion de segregacion es compatible con la hipotesis de un modo de herencia autosomico recesivo unico. Los datos clinicos y genetocis sugieren que una, hasta ahora desconocida, enfermedad sistemica del sistema nervioso central es muy prevalente en esta poblacion aislada. ZUSAMMENFASSUNG Bei einem isolierten Stamm im Inneren von Tanzania fanden die Autoren, das etwa 200 einer Ein-wohnerzahl von 10,000 sich in einer Buschklinik mit der Krankheit Kifafa, einem Anfallsleiden vorstellten, das haufig zu schweren Verbrennungen bei den Betroffenen fuhrt. Viele der Falle zeigten Parkinson-Symptome und/oder andere neurologische Besonderheiten, Demenz sowie eine hohe Mortalitat. Eine haufige Variante der Erkrankung bei Kindern besteht in “Kopfnickern,” die haufig spateren Grand-Mal-Anfallen vorangingen. Vorubergehende psychotische Episoden wurden bei 20% der Falle gesehen. Die Patienten wurden 10 Jahre lang verfolgt. Das notwen-digerweise einfache antikonvulsive Regime mit Phenobarbital verminderte die Anfallsfrequenz und Intensitat bei den meisten Patienten. Die Autoren dis-kutieren die moglichen Grunde fur das hohe Vorkommen des Anfallsleidens bei diesem Stamm. Genetische Faktoren scheinen eine hervorragende Bedeutung zu haben besonders unter Berucksichtigung der hohen Familiaritat. Die genetische Analyse der Daten liefert keine Hinweise auf chromosomale multifaktorielle geschlechtsgebundene oder autosomal dominante Vererbungsformen. Die Analyse mit Hilfe der Li-Mantel-Methode weist daraufhin, das das Tren-nungsverhaltnis zu vereinbaren ist mit der Hypothese einer einfachen autosomal rezessiven Vererbung. Klinische und genetische Daten lassen vermuten, das eine bisher unbekannte systemische Erkrankung des ZNS bei dieser isolierten Bevolkerung vorherrscht.

Summary: We have analyzed 66 cases of sudden unexpected death (SUD) in persons with seizure disorders, which were examined by the Office of the Medical Examiner, Cook County (Chicago), Illinois. The individuals ranged in age from 10 months to 60 years (mean age, 28 years). Autopsy findings were insufficient to explain death, and there was no evidence of major systemic pathology. Approximately 40% of victims were found dead in bed, and the remainder in some other room at home, apparently having been engaged in normal activity. Several died in an emergency room following a seizure at home. Cardiopulmonary resuscitation was attempted but was ineffective. Neuropathological examination revealed brain lesions, which probably caused the seizures, in 60% of the cases. In 68% the anticonvulsant blood level was subtherapeutic or below detectable levels. The prevalence of seizure-associated SUE) may be between 1:525 and 1:2,100 among epileptics. The mechanism of death in these cases probably involves cardiac arrhythmias mediated by sympathetic autonomic events occurring during the seizure. RESUMEN Hemos analizado 66 casos de muerte subita imprevista (SUD) en personas con trastornos convulsivos que habian sido examinadas en la Oficina del Forenso en el Condado de Cook (Chicago), Illinois. La edad oscilaba entre 10 meses y 60 anos (media 28). Los hallazgos de la autopsia eran insuficientes para explicar la muerte y no se encontro patologia sistemica importante. Aproximadamente un 40% de las victimas fueron halladas muertas en la cama y el resto en otras habitaciones de la casa habiendose ocupado de actividades normales. Varios fueron trasladados a un servicio de urgencias, tras el ataque en sudomicilio, donde se intentaron medidas de resucitacion que fueron ineficaces. Los estudios neuropatologicos revelaron que el 60% de los casos tenian lesiones cerebrales, probable causa de sus ataques. En el 68% los niveles de anticonvulsivantes eran subterapeuticos o indetectables. La prevalencia de SUD asociada a ataques puede oscilar entre 1:525 y 1:2100 en los epilepticos. Los mecanismos de muerte en estos casos estan probablemente relacionados con arritmias cardiacas mediadas por acontecimientos autonomicos simpaticos que puedan ocurrir durante los ataques. ZUSAMMENFASSUNG Analyse von 66 Fallen eines plotzlichen und unerwarteten Todes (SUD) bei Anfallskranken, die vom Buro der Gesundheitsbehorde (Cook County, Chicago, Illinois) untersucht wurden. Das Durchschnittsalter betrug 28 Jahre (10 Monate bis 60 Jahre). Die Autopsiebefunde reichten nicht aus, um den Tod zu erklaren und es gab keine Hinweise auf eine grobe System-pathologie. Etwa 40% der Betroffenen wurden tot im Bett gefunden. Die ubrigen fand man,–offenbar ihrer normalen Tatigkeit nachgehend–in anderen Zimmern ihrer Wohnung. Mehrere wurden als Notfall nach einem hauslichen Anfall aufgenommen, nachdem Wiederbelebungsversuche wirkungslos geblieben waren. Die neuropathologische Untersuchung zeigte, das 60% Hirnlasionen hatten, die wahrscheinlich fur die Anfalle verant-wortlich waren. Bei 68% waren die Blutspiegel der Antikon-vulsiva im subtherapeutischen Bereich oder unterhalb der Nachweisgrenze. Die Pravalenz von Anfallen in Beziehung zum SUD mag bei Epileptikern zwischen 1:525 bis 1:2100 liegen. Der Mechanismus des Todes erfolgt in diesen Fallen wahrscheinlich auf Grund cardialer Arrhythmien im Gefolge sympathischer au-tonomer Dysregulationen, die wahrend des Anfalls erfolgen.