Summary: Purpose : We wished to evaluate the prognostic usefulness of various EEG parameters with respect to remission rates after discontinuation of antiepileptic drug (AED) therapy in children treated for epileptic seizures. Methods : Two hundred forty‐four children with uncomplicated epileptic seizures were randomized to either 1 or 3 years of treatment with AEDs. The treatment was then discontinued in patients who had been seizure‐free during the last 6 months of their allotted time of treatment (n = 154). After treatment discontinuation, the children were followed for at least 2 years. EEG recordings were performed before treatment was initiated and at regular intervals during treatment. Results : The overall relapse rate was 37%. In many children, the amount of epileptiform activity varied considerably between subsequent recordings made during the treatment. The remission rate was slightly higher for children whose last recordings before AED discontinuation were free of epileptiform activity as compared with children in whom such activity was present. However, children who had irregular generalized spike–wave (SW) activity in the recordings made before discontinuation of treatment had a clearly higher relapse rate (67%) both as compared with children without epileptiform activity (33%) and as compared with children with other types of epileptiform activity (33%) in their last EEG recordings before discontinuation. All children treated for only 1 year whose final EEGs displayed generalized irregular SW activity relapsed. Conclusions : We conglude that the presence of epileptiform activity does not in itself necessarily influence prognosis after discontinuation of treatment but that certain types of such activity signal a high risk of relapse.

Nineteen children with epilepsy were tested on two occasions, first during treatment with carbamazepine (CBZ) and then 6 months later without treatment. Plasma drug concentrations were within the therapeutic limits in all children. The children were examined with a standardized test of gross- and fine- motor functions, the Bruininks-Oseretsky test of motor proficiency. Significant improvements were found in response speed(p < 0.05), in composite fine-motor tests (p < 0.01) and in the total test battery (p < 0.05) after the treatment had been withdrawn. A tendency to improvement was found in the fine-motor subtest of upper limb coordination (p = 0.08). Another group of 12 children was tested twice during treatment with CBZ with an interval of 6 months. No difference was found in this group except for an impairment of the results in the subtest of visual-motor control on the second test occasion (p = 0.05).

Purpose: The main purpose of this prospective study was to analyze whether 1 year of treatment was as effective as 3 years with respect to remission rate in children with idiopathic epilepsy.Methods: Treatment for epileptic seizures was started in 207 children aged 2-16 years, They were randomized to treatment for 1 or 3 years. At the end of the predetermined treatment period, 161 children had been seizure-free for 6 months and the treatment could be gradually withdrawn.Results: The overall remission rate in our group of patients was significantly higher (71%) in the group treated for 3 years than in the group treated for 1 year (53%). However, comparison of remission rates between patients with different seizure types showed statistically significant differences in outcome depending on duration of treatment only in children with complex partial seizures (CPS).Conclusions: Our results show that 1 year of treatment can be recommended in children with benign partial epilepsy with rolandic spikes (BECT) and in children with simple partial seizures (SPS) but is clearly insufficient in children with CPS, A proper seizure classification is one important tool, although not sufficient, in offering recommendations concerning the duration of treatment in children with idiopathic epilepsy.

Summary: Purpose: The efficacy and tolerability of vigabatrin (VGB) as an add‐on therapy in the treatment of infantile spasm (IS) prompted physicians to explore its use as the first drug in this seizure type. Methods: Our retrospective study included 250 infants diagnosed with IS; the data obtained were subjected to peer‐group review. Of this infant population, 192 infants were considered to have classic IS and had received VGB as their first treatment for the spasms. There was a slight preponderance of boys (57%) in this population. Mean age of IS onset was 5.8 months; 60% had typical hypsarrhythmia. Results: Initial suppression of spasms was obtained in 68% of infants with a median time to response of 4 days at an average VGB dose of 99 mg/kg/day. The best response was seen in those infants with tuberous sclerosis (96% response) and in those younger than 3 months at onset of spasms (90% response). Of these infants, 43 (22%) of 192 subsequently had other types of seizures, and a recurrence of infantile spasms occurred in 28 (21%) of 131 responders. At the end of this study, 96 of 192 infants who could be evaluated were seizure free with VGB monotherapy. Treatment appeared to be well tolerated, with only 33 (13%) infants with adverse events, of which the most common were somnolence (15 patients) and hyper‐kinesia (eight patients). In only two cases did adverse events require VGB withdrawal. Conclusion: This study supports the opinion that VGB may be considered an initial treatment for IS regardless of cause.

In a general hospital population of 74,000 children under the age of 16 years in southern Stockholm, 79 children were started on antiepileptic drugs due to epilepsy during the 2 year period 1990-92. The mean annual incidence of childhood epilepsy in this area was 53 per 100,000 children younger than 16 years. Neurological impairments were identified in 35% of the children. The epilepsy diagnoses were set according to the International Classification System proposed by the International League Against Epilepsy. Partial seizures were seen in 52% of the children. The syndrome BECT was identified in 11%. The epilepsy was symptomatic in 30%, and therapy-resistant in 23%.

Seven children with absence epilepsy were treated with valproate (VPA). All but one child became free of absence seizures during VPA monotherapy. EEG was recorded for 24 h before start of VPA treatment and repeatedly during treatment. Correlation between plasma VPA concentration and reduction of the number of epileptic discharges was significant. Plasma concentration of 440-660 microM VPA was needed to achieve at least 50% reduction of seizure activity.

The case of a patient with concomitant regional odontodysplasia, hydrocephalus, and mental retardation is presented. Tooth eruption was retarded, probably because of the presence of a cementum-like substance on the enamel surface as well as the presence of discontinuous odontogenic epithelium that surrounded the affected teeth. Neural damage during intrauterine life is suggested as a likely cause of the dental abnormalities. This hypothesis is supported by the fact that both primary and permanent teenth in the area were affected. It is further supported by the finding of dysplastic dentin.

The periodontal condition and caries experience was studied in non-institutionalized epileptic children (n = 55) who had not been subjected to any additional preventive measures. The children were distributed in a phenytoin (PHT) group with a mean age of 13.2 yr and a control group (mean age 11.8 yr) consisting of children treated with other anticonvulsants. The PHT group had a DF-s mean value of 5.4 in comparison to 7.2 in the control group. Determinations of gingival overgrowth based on the presence of gingival units with increased probing depth (greater than 4 mm) as well as the thickness of the marginal gingiva in buccolingual dimension measured on stone casts were performed. Although the plaque level and degree of gingival inflammation were similar in the two groups, 43% of the children in the PHT group showed one or more gingival units with increased probing depth (greater than 4 mm), but none in the control group. The thickness of the marginal gingiva was significantly (P less than 0.001) higher in the phenytoin-treated children compared to children who had never had phenytoin medication. In the PHT-group gingival overgrowth based on gingival units with increased probing depth was statistically significantly and positively associated with the variables gingivitis (P less than 0.05), visible plaque index (P less than 0.01), age (P less than 0.01) and years on PHT therapy (P less than 0.05).

Chondroectodermal dysplasia (Ellis‐van Creveld syndrome) has previously been diagnosed prenatally only once, using fetoscopy. We report on two consecutive pregnancies in a woman at risk of having a child with the syndrome during which fetoscopic visualization was performed. Ellis‐van Creveld syndrome was diagnosed prenatally in one instance, while it could be excluded in the other one. Non‐invasive prenatal diagnosis of the syndrome is discussed.

Summary: The aim of this study was to see if the immediate EEG and clinical response to an intravenous dose of clonazepam was predictive for the effect of oral clonazepam maintenance therapy. Four children with petit mal epilepsy were given clonazepam intravenously during continuous EEG recording. Clonazepam plasma concentrations were determined repeatedly with a high performance liquid chromatographic method using a reversed phase system. The day after the intravenous dose the patients were given oral therapy with clonazepam. Repeated long‐term EEG recordings were made and plasma concentrations of clonazepam were determined. There was no clinically satisfactory effect of clonazepam during oral maintenance treatment in three of the children who responded well to the intravenous dose of clonazepam. Thus, the immediate response to intravenous clonazepam was not a good predictor of the long‐term effects in our patients.RESUMENEl propósito de esta comunicación ha sido la verifi‐cación de si la respuesta inmediata, clfnica y electro‐encefalografica, a una inyección intravenosa de clonazepan podría predecir el efecto de una terapia de mantenimiento del clonazepan oral. Clonazepan intravenoso ha sido administrado a cuatro niños con epilepsia petit‐mal durante un registro de EEG, contínuo. Las concentrations plasmáticas de clonazepan se calcularon repetidamente con un método de cro‐matografía de uquidos de alta resolución usando un sistema de fase revertida. Al día siguiente de la dosis intravenosa los pacientes recibieron clonazepan oral y se registraron EEGs de larga duratión. Se deter‐minaron los niveles plasmäticos de clonazepan. En tres de los niños con buena respuesta al clonazepan intravenoso, no se observaron efectos beneficiosos durante el tratamiento oral prolongado. Asf pues, la respuesta inmediata al clonazepan intravenoso no es un factor de predictión de los efectos de un tratamiento prolongado en nuestros enfermos.ZUSAMMENFASSUNGDas Ziel der Untersuchung war es festzustellen, ob die Sofortwirkung auf das EEG und die Klinik nach intravenoser Applikation von Clonazepam erlaubt, die Wirkung einer oralen Clonazepam‐Erhaltungstherapie vorherzusgen. Kinder (4) mit Petit Mai Epilepsien erhielten Clonazepam i.v, wahrend einer EEG‐Ableitung. Die Clonazepam‐Plasmakonzentration wurde wiederholt gemessen (Flüssigkeits‐chroma‐tographisch). Am Tage nach der intravenösen Applikation erhielten die Patienten eine orale Therapie mit Clonazepam. Wiederholte Langszeit‐EEG wurden abgeleitet und die Plasmakonzentrationen das Clonazepam bestimmt. Bei 3 Kindern, die gut auf das intravenöse Clonazepam reagierten, fehlte eine klinisch befriedigende Wirkung des Clonazepam während der oralen Erhaltungstherapie. Damit scheint die Sofortantwort auf intravenöses Clonazepam keine gute Vorhersage der Langzeiteffekte unserer Patienten zu erlauben.

ABSTRACT. Haglund, M., Bergvall, U., Theorell, K. and Troell, S. (Departments of Paediatrics and Diagnostic Radiology, Huddinge Hospital, Huddinge, Sweden). Infantile spasm and tuberous sclerosis. Acta Paediatr Scand, 70:751,.–During the years 1976–78, infantile spasm was diagnosed in a total of 12 children. In 4 of these patients the diagnosis tuberous sclerosis was established with the aid of computed tomography (CT). The advantages of CT examination in children with infantile spasm is discussed, especially the possibility to detect tuberous sclerosis at an early stage.

Plasma concentrations of two phenytoin products (a conventional phenytoin acid preparation and a microcrystalline form of phenytoin acid) were studied after single dose administration and during steady-state conditions in four healthy male volunteers.

THEORELL, K.: Clinical value of prolonged polygraphk recordings in high-risk newborn infants. Neuropadiatrie5: 383—401 (1974). Twelve full-term newborn infants suffering from high-risk conditions such as asphyxia and convulsions, were examined with polygraphic methods. They were examined between the fourth and 11th day of life. The physiological variables recorded were respiration, ECG, surface EMG, EOG and EEG, and the recordings lasted six hours. The paper write-outs were analysed visually with regard to behavioural state, convulsions, EEG, abnormalities of respiratory and heart rate patterns and eye movements. Two infants had clinical convulsions during state 2 and one during state 3. The relation between behavioural state and convulsions is discussed. One infant with massive necrosis of the hemispheres and who showed sleep-wakefulness cycle and alternation between sleep states is described. The advantages of prolonged polygraphic examinations are discussed.

Twenty-three low-risk full-term newborn infants were examined with polygraphic methods on their first and fifth day of life. Twelve of them were subjected to early clamping of the umbilical cord and eleven to late clamping. The physiological variables recorded were respiration, EKG, surface EMG, EOG and EEG. The recordings lasted six hours. Computer processing techniques were used in the working through of the analogue data.

SUMMARY Polygraphic methods were used to evaluate the percentage distribution of sleep and awake states and the mean duration of individual periods spent in these states in infants with Down's syndrome, bilirubinaemia and in a heterogeneous group of ‘high‐risk’ infants. Not unexpectedly, alterations from previously established normal values were found, as were differences in pattern between the groups. Infants with Down's syndrome spend more time awake at the expense of time in state 2 (active sleep), but prolonged sleep cycles are occasionally possible. Bilirubinaemic infants showed a drastic reduction in the amount of time spent in the awake states. Individual periods spent in state 2 were significantly longer, producing also a prolonged sleep cycle duration. The high‐risk group spent abnormally short periods in state 2 and none of the infants studied produced a long sleep cycle. The amount of time spent in awake states varied widely. Anti‐epileptic drugs induced more and longer individual periods of state 1 (quiet sleep) at the expense of state 2. The pathophysiological mechanisms behind these alterations in the state cycle are discussed. RÉSUMÉ Cycles de niveaux de vigilance chez les nouveaux‐nés anormaux Grâce à des méthodes polygraphiques, trois groupes de nourrissons de conditions anormales ont étéétudiés pour évaluer le pourcentage de temps passé dans chacun des divers états de sommeil et d'éveil ainsi que la durée des périodes individuelles passées dans ces différents états. Comme on pouvait s'y attendre des différences ont été observées non seulement par rapport aux valeurs établies antérieurement mais aussi avec les trois groupes. Les mongoliens passent plus de temps éveillés aux dépens du temps passé en stade 2 (sommeil actif), mais des cycles prolongés de sommeil ont pu êtren otés occasionnellement. Les nourrissons hyperbilirubinémiques ont montré une réduction considérable du temps passé dans les états de veille. Les périodes individuelles en stade 2 étaient significativement allongées et de ce fait, les plus longs cycles de sommeil étaient également allongés. Le groupe hétérogène des nourrissons à `hauts risques' présentait des périodes anormalement courtes en stade 2 et en aucun cas, il n'a été enregistré de longs cycles de sommeil. La quantité de temps passé en état de veille variait considérablement. Les médications anti‐épileptiques provoquaient un accroissement en nombre et en durée des périodes de stade 1 (sommeil tranquille) aux dépens des périodes du stade 2. Les mécanismes physio‐pathologiques sous jacents à ces altérations des cycles de vigilance sont discutés. ZUSAMMENFASSUNG Verhaltenszustandszyklen bei abnormen Neugeborenen Drei Gruppen von Kindern mit abnormen Bedingungen wurden mit polygraphischen Methoden untersucht, um den Prozentsatz der Zeit, die sie in jedem der verschiedenen Schlaf‐ und Wachzu‐stände verbringen, und die Dauer der einzelnen Perioden dieser Zustände zu berechnen. Erwartungsgemäß fanden sich Unterschiede nicht allein von früher festgestellten Werten, sondern auch zwischen den drei Gruppen. Die Kinder mit Down‐Syndrom verbrachten mehr Zeit im Wachzustand auf Kosten der Zeit des Stadium 2 (aktiven Schlafes), doch wurden gelegentlich auch verlängerte Schlafzyklen gefunden. Die Kinder mit Bilirubinaemie zeigten eine beträchtliche Verminderung der Zeitdauer, die im Wachzustand verbracht wurde. Einzelne Perioden im Schlafstadium 2 waren signifikant verlängert und als Ergebnis dessen war der längste Schlafzyklus auch verlängert. Die heterogene Gruppe der ‘high risk’ Kinder zeigte abnorm kurze Perioden im Schlafstadium 2, und in keinem Fall wurde ein längerer Schlafzyklus festgestellt. Die Zeitdauer des Wachzustandes variierte beträchtlich. Antiepileptische Medikamente verursachten größere Mengen und längere Einzelperioden des Stadium 1 (vuhigen Schlafes) auf Kosten des Stadium 2. Die pathophysiologischen Mechanismen dieser Veränderungen im Zustandszyklus werden diskutiert.

SUMMARY The cyclic patterns of sleep and wakefulness (behavioural ‘states’) were studied polygraphically in a group of 21 normal newborn infants on the first and the fifth days of life. The percentage of time spent awake underwent drastic reduction between the first and the fifth days. The percentage of time spent in state 2 (active sleep) increased correspondingly. The percentage of time spent in state 1 (quiet sleep) remained relatively unchanged; it exceeded state 2 on the first day and was exceeded by state 2 on the fifth day. On the fifth day, there was a tendency for the number of state transitions per hour to be less than on the first day, and for the durations of periods in state 2 to increase. On the first day, infants quite often appeared to wake directly from state 1, but this seldom happened on the fifth day, when waking usually occurred from state 2. Late‐clamped infants spent a higher percentage of time in state 1 and less in the quiet awake states than did the early‐clamped infants. Individual differences between the lateclamped infants were much greater. Respiratory rate (which is positively related to blood volume in the neonate) was significantly lower in the early‐clamped compared with the late‐clamped group, in whom the results showed considerable scatter. It is possible that late clamping of the cord represents an additional stress to the neonate and that early clamping confers an advantage on the infant who spends more time awake on the first day, thereby promoting a sound mother and baby relationship. RÉSUMÉ Un groupe de 21 nouveaux‐nés normaux ont étéétudiés au ler et 5ème jour de la naissance à l'aide de techniques polygraphiques pour mesurer le pourcentage de temps passé dans les différents états de vigilance ainsi que la durée des périodes de chacun de ces états. Le pourcentage de temps passééveillé subit une réduction considérable entre le ler et le 5ème jour. Le pourcentage de temps en stade 2 (sommeil actif) s'accroit d'autant. Le temps passé en stade 1 (sommeil tranquille) demeure inchangé, de telle façon qu'il dépasse le temps du stade 2 le premier jour et est inférieur le 5ème. Il y a une tendance au 5ème jour à ce que le nombre de changements d'états par heure soit moindre et que les périodes individuelles de stade 2 s'allongent. Au ler jour, très souvent, les enfants se réveillent directement à partir du stade 1, mais au 5ème jour, le réveil surveint généralement à partir du stade 2. Les nourrissons à‘clampage tardif’ passent un pourcentage plus élevé de temps au stade 1 et moindre à l'état tranquille éveillé que les enfants à‘clampage précoce’. Les enfants à clampage tardif présentent des variations individuelles beaucoup plus importantes. Le rythme respiratoire qui est relié plus au volume sanguin chez le nouveau‐né est significativement plus lent chez les nourrissons à clampage précoceé qu'à clampage tardif, parmi ceux‐ci, les résultats présentent une dispersion très marquée. Il est possible que le clampage tardif du cordon représente une agression supplémentaire chez le nouveau‐né et que la clampage précoce confère un avantage en permettant au nourrisson de passer plus de temps éveillé le ler jour, ce qui favorise une bonne relation mère‐enfant. ZUSAMMENFASSUNG Es wurde eine Gruppe von 21 normaler Neugeborener am 1. und 5. Lebenstag mit polygraphischen Methoden untersucht, um den Prozentsatz der Zeit, die in jedem der verschiedenen Verhaltenszustände verbracht wurde, und die Dauer der einzelnen in ihnen verbrachten Perioden zu messen. Der Zeitanteil des Wachseins erfuhr zwischen dem 1. und 5. Tage eine drastische Verminderung. Der Prozentsatz der Zeit, die im Stadium 2 (aktiven Schlaf) verbracht wurde, stieg entsprechend an. Die Gesamtdauer des Stadium 1 (ruhigen Schlafes) blieb relativ unverändert, so daß er am ersten Tage über den Anteil des Stadium 2 hinausging und am fünften Tage hinter ihm zurückblieb. Es zeigte sich die Tendenz, daß die Zahl der Stadienwechsel pro Stunde am 5. Tage geringer war und die Dauer der einzelnen Perioden im Stadium 2 anstieg. Am 1. Lebenstag erwachten die Kindern offenbar ganz häufig direkt aus dem Stadium 1, während sie am 5. Tage gewöhnlich aus Stadium 2 erwachten. Die spätabgenabelten Kinder verbrachten einen größeren Prozentsatz ihrer Zeit im Stadium 1 und weniger im ruhigen Wachzustand als die früabgenabelten Kinder. Die spätabgenabelten Kinder zeigten viel größere individuelle Unterschiede. Die Atemfrequenz, die dem Blutvolumen beim Neugeborenen proportional ist, war bei den frühabgenabelten Kindern signifikant niedriger als bei den spätabgenabelten, bei denen die Ergebnisse wiederum eine beträchtliche Schwankungsbreite zeigten. Es ist möglich. daß das Spätabnabeln einen zusätzlichen Stress für das Neugeborene bedeutet und daß das Frühabnabeln von Vorteil ist, da es das Kind am 1. Tage mehr Zeit im Wachzustand verbringen läßt, was eine intakte Mutter‐Baby‐Beziehung begünstigt.

SUMMARY The statistical properties of crying bouts have been studied in newborn infants during their first nine days of life. The crying was elicited by mild discomfort and by pinching the skin. Twenty‐one newborns were neurologically examined daily and, according to the diagnosis, divided into three groups: (1) normal; (2) transiently abnormal and (3) consistently abnormal. In the normals the duration of individual cries in a crying bout and the intervals between them are characteristic for the individual. There is an overall tendency for cry duration and interval to become shorter and less variable during the first nine days of life. In contrast to the normal babies, infants from groups 2 and 3 showed a large variation in the crying parameters, especially in the cry duration. The significance of these findings is discussed. RÉSUMÉ Analyse statistique des crises de pleurs chez le nouveau‐né normal et anormal Les propriétés statistiques des crises de pleurs ont étéétudiées chez des nouveaux‐nes durant les 9 premiers jours de la vie. Les pleurs étaient provoqués par un inconfort léger ou par pincements de la peau. 21 nouveaux‐nés ont été examinés neurologiquement tous les jours et selon le diagnostic, divisés en trois groupes: (1) normaux; (2) transitoirement anormaux; (3) anormaux en permanence. Chez l'enfant normal, la durée de chaque pleur individuel dans une crise de pleurs ainsi que l'intervalle entre les pleurs est spécifique pour un individu. II y a une tendance générate à ce que la durée des pleurs et les intervalles deviennent plus courts et moins variables durant les neuf premiers jours de la vie. A l'opposé du nouveau‐né normal, les enfants des groupes 2 et 3 présentaient une grande variation dans les parametres de pleurs, specialement dans la durée des pleurs. La signification de ces faits est discutée. ZUSAMMENFASSUNG Die statischen Eigenschaften des akustischen Signals des Weinens wurden bei Neugebornnen in den ersten neun Lebenstagen untersucht. Ausgelöst wurde das Weinen dadurch, dass das Kind kurz vor der Fütterung aus der Wiege genommen wurde, sovvie durch leichtes Kneifen der Haut. 21 Neugeborene wurden täglich neurologisch untersucht und je nach der Diagnose in drei Gruppen eingeteilt: (1) normale Kinder; (2) Kinder mit vorübergehend abnormen Zeichen; (3) konsistent abnormale Kinder. Bei den Normalen war die Dauer der einzelnen Schreie, sowie die Intervalle zwischen den einzelnen Schreien charakteristisch für das Individuum. Im allgemeinen werden die Schreidauer und die Intervalle während der ersten neun Lebenstage kürzer und regelmässiger. Im Gegensatz zu den normalen Kindern zeigten die Kinder der Gruppe 2 und 3 eine grössere Varianz der Schreiparameter. Dies gilt besonders für die Schreidauer. Die Bedeutung dieser Befunde wird diskutiert. RESUMEN Un análisis estadistico de tipos de llanto en recién nacidos normales y anormales Se ha estudiado la estadística de accesos de llanto en recién nacidos durante los nueve primeros días de la vida. Se incitó el llanto por condiciones de incomodidad moderada y por pellizcar la piel del lactante. Se realizaron tests neurológicos diarios en 21 recién nacidos, que se repartieron según el diagnóstico en tres grupos: (1) normales; (2) anormales transitorios; (3) anormales consistentes. En los lactantes normales, la duración de los gritos individuos y los intervalos éntre estos en un acceso de llanto son típicos para el individuo. En el grupo entero, se ve una tendencia hasta duracion e intervalos más cortos y menos variables durante los nueve primeros dias de la vida. En cambio, lactantes de los grupos 2 y 3 mostraban una gran variatión de los parámetros del llanto, especialmente de la duración de los gritos. Se discute la significancia de estos hallazgos.