Repetitive transcranial magnetic stimulation to treat benign epilepsy with centrotemporal spikes.

OBJECTIVES: To investigate the effects of low-frequency repetitive transcranial magnetic stimulation (rTMS) on patients with benign epilepsy with centrotemporal spikes (BECTS). METHODS: In this open pilot study, we enrolled four BECTS patients who had frequent seizures (at least 3 seizures during the 3-month baseline). After localizing sources of interictal epileptiform discharges (IEDs) with magnetoencephalography, IEDs-source-rTMS (1 Hz) with 500 pulses at 90% of resting motor threshold was applied for 10 weekdays in each patient. The primary outcome measure was the seizure-reduction rate after rTMS. Other outcome measures were the spike-wave index (SWI), behavioral evaluation, and adverse effects. RESULTS: All four patients received at least 3 months seizure-free after rTMS. Compared with baseline, SWI decreased significantly after rTMS in three patients (patient 1, 3 and 4) (P = .002, P = .007, and P < .001, respectively). Attention deficit identified in two patients in baseline recovered to the normal range after rTMS. No adverse effect was observed. DISCUSSION: Our preliminary observation provides a promising approach to reducing clinical seizures for BECTS with frequent seizures. Of importance, our data may provide a potentially novel method for the high prevalence of behavioral problems in BECTS patients via decreasing cortical hyperexcitability.

Cortical Excitability, Synaptic Plasticity, and Cognition in Benign Epilepsy With Centrotemporal Spikes: A Pilot TMS-EMG-EEG Study.

PURPOSE: Children with benign epilepsy with centrotemporal spikes have rare seizures emerging from the motor cortex, which they outgrow in adolescence, and additionally may have language deficits of unclear etiology. We piloted the use of transcranial magnetic stimulation paired with EMG and EEG (TMS-EMG, TMS-EEG) to test the hypotheses that net cortical excitability decreases with age and that use-dependent plasticity predicts learning. METHODS: We assessed language and motor learning in 14 right-handed children with benign epilepsy with centrotemporal spikes. We quantified two TMS metrics of left motor cortex excitability: the resting motor threshold (measure of neuronal membrane excitability) and amplitude of the N100-evoked potential (an EEG measure of GABAergic tone). To test plasticity, we applied 1 Hz repetitive TMS to the motor cortex to induce long-term depression-like changes in EMG- and EEG-evoked potentials. RESULTS: Children with benign epilepsy with centrotemporal spikes tolerate TMS; no seizures were provoked. Resting motor threshold decreases with age but is elevated above maximal stimulator output for half the group. N100 amplitude decreases with age after controlling for resting motor threshold. Motor cortex plasticity correlates significantly with language learning and at a trend level with motor learning. CONCLUSIONS: Transcranial magnetic stimulation is safe and feasible for children with benign epilepsy with centrotemporal spikes, and TMS-EEG provides more reliable outcome measures than TMS-EMG in this group because many children have unmeasurably high resting motor thresholds. Net cortical excitability decreases with age, and motor cortex plasticity predicts not only motor learning but also language learning, suggesting a mechanism by which motor cortex seizures may interact with language development.

Core Health Outcomes in Childhood Epilepsy (CHOICE): Development of a core outcome set using systematic review methods and a Delphi survey consensus.

OBJECTIVE: Establishing a core set of outcomes to be evaluated and reported in intervention trials aims to improve the usefulness of health research. There is no established core outcome set (COS) for childhood epilepsies. The aim of this study was to select a COS to be used in evaluative research of interventions for children with rolandic epilepsy (RE). METHODS: We followed guidance from the COMET (Core Outcome Measures in Effectiveness Trials) Initiative. First, we identified outcomes that had been measured in research through a systematic review. Second, young people with RE, parents, and professionals were invited to take part in a Delphi survey in which participants rated the importance of candidate outcomes. Last, a face-to-face meeting was convened to seek consensus on which outcomes were critical to include and to ratify the final COS. RESULTS: From 37 eligible papers in the review, we identified and included 48 candidate outcomes in the survey. We sent invitations to 165 people registered to take part in the survey; of these, 102 (62%) completed Round 1, and 80 (78%) completed Round 2 (three young people, 16 parents, 61 professionals). In Round 2 we included four additional outcomes suggested by participants in Round 1. The consensus meeting included two young people, four parents, and nine professionals who were eligible to vote and ratified the COS as 39 outcomes across 10 domains. SIGNIFICANCE: Our methodology was a proportionate and pragmatic approach toward producing a COS for evaluating research on interventions aiming to improve the health of children with RE.

Spike persistence and normalization in benign epilepsy with centrotemporal spikes - Implications for management.

PURPOSE: This study was performed 1) to determine the timing of spike normalization in patients with benign epilepsy with centrotemporal spikes (BECTS); 2) to identify relationships between age of seizure onset, age of spike normalization, years of spike persistence and treatment; and 3) to assess final outcomes between groups of patients with or without spikes at the time of medication tapering. METHODS: Retrospective analysis of BECTS patients confirmed by clinical data, including age of onset, seizure semiology and serial electroencephalography (EEG) from diagnosis to remission. Age at spike normalization, years of spike persistence, and time of treatment onset to spike normalization were assessed. Final seizure and EEG outcome were compared between the groups with or without spikes at the time of AED tapering. RESULTS: One hundred and thirty-four patients were included. Mean age at seizure onset was 7.52 ±â€¯2.11 years. Mean age at spike normalization was 11.89 ±â€¯2.11 (range: 6.3-16.8) years. Mean time of treatment onset to spike normalization was 4.11 ±â€¯2.13 (range: 0.24-10.08) years. Younger age of seizure onset was correlated with longer duration of spike persistence (r = -0.41, p < 0.001). In treated patients, spikes persisted for 4.1 ±â€¯1.95 years, compared with 2.9 ±â€¯1.97 years in untreated patients. No patients had recurrent seizures after AED was discontinued, regardless of the presence/absence of spikes at time of AED tapering. CONCLUSION: Years of spike persistence was longer in early onset BECTS patients. Treatment with AEDs did not shorten years of spike persistence. Persistence of spikes at time of treatment withdrawal was not associated with seizure recurrence.

Core Health Outcomes In Childhood Epilepsy (CHOICE): protocol for the selection of a core outcome set.

BACKGROUND: There is increasing recognition that establishing a core set of outcomes to be evaluated and reported in trials of interventions for particular conditions will improve the usefulness of health research. There is no established core outcome set for childhood epilepsy. The aim of this work is to select a core outcome set to be used in evaluative research of interventions for children with rolandic epilepsy, as an exemplar of common childhood epilepsy syndromes. METHODS: First we will identify what outcomes should be measured; then we will decide how to measure those outcomes. We will engage relevant UK charities and health professional societies as partners, and convene advisory panels for young people with epilepsy and parents of children with epilepsy. We will identify candidate outcomes from a search for trials of interventions for childhood epilepsy, statutory guidance and consultation with our advisory panels. Families, charities and health, education and neuropsychology professionals will be invited to participate in a Delphi survey following recommended practices in the development of core outcome sets. Participants will be able to recommend additional outcome domains. Over three rounds of Delphi survey participants will rate the importance of candidate outcome domains and state the rationale for their decisions. Over the three rounds we will seek consensus across and between families and health professionals on the more important outcomes. A face-to-face meeting will be convened to ratify the core outcome set. We will then review and recommend ways to measure the shortlisted outcomes using clinical assessment and/or patient-reported outcome measures. DISCUSSION: Our methodology is a proportionate and pragmatic approach to expediently produce a core outcome set for evaluative research of interventions aiming to improve the health of children with epilepsy. A number of decisions have to be made when designing a study to develop a core outcome set including defining the scope, choosing which stakeholders to engage, most effective ways to elicit their views, especially children and a potential role for qualitative research.

[Cognitive Development in Children with Benign Rolandic Epilepsy of Childhood with Centrotemporal Spikes - Results of a Current Systematic Database Search]. / Kognitive Entwicklung von Kindern mit benigner Epilepsie des Kindesalters mit zentrotemporalen Spitzen (Rolando-Epilepsie) ­ Ergebnisse einer aktuellen systematischen Literaturrecherche.

Benign Rolandic Epilepsy (BRE) is one of the most common epilepsy syndromes in childhood. Although global intellectual performance is typically normal in BRE-patients, problems were found in specific cognitive domains. To summarize recent empirical findings concerning cognitive development in children with BRE a systematic literature search of clinical studies published between 2009 and 2015 was performed. 19 studies of relevance were found.In most recent studies children with BRE consistently showed general intellectual performance within the normal range. However, in two of the studies patients showed a significantly poorer (but still normal) performance in comparison to controls. The studies provide clear indications for a high prevalence of impairments in language (10 out of 12 studies) and academic performance (6 out of 8 studies) in children with BRE. Regarding deficits in other cognitive domains (attention, memory, visual/auditory perception, executive functions) current findings are inconsistent. In addition, no clear results are found in studies examining cognitive development after remission of BRE. Studies on the relationship between selected clinical/electroencephalographic characteristics (e. g. EEG-patterns, focus lateralization) and cognitive performance and studies on potential benefits of anti-epileptic therapy for cognitive functions also have not yielded consistent results. Studies using fMRI and evoked potentials provide evidence for functional reorganization of neural networks in BRE.Due to the developmental risks in children with BRE early cognitive assessment, early treatment and follow-up assessments are important.

Las alteraciones neuropsicológicas son frecuentes en la epilepsia rolándica y sus evoluciones atípicas / Neuropsychological alterations are frequent in rolandic epilepsy and its atypical developments

Introducción. La epilepsia rolándica o epilepsia benigna de la infancia con puntas centrotemporales se denomina benigna debido a lo favorables que suelen ser sus crisis y a la espontánea normalización del electroencefalograma al llegar a la pubertad, aunque se ha demostrado el impacto sobre el desarrollo cognitivo con la presencia de déficits cognitivos heterogéneos, relacionados especialmente con las descargas intercríticas persistentes durante el sueño no REM. El objetivo de este trabajo es estudiar las redes epileptógenas involucradas en los trastornos neuropsicológicos de esta patología. Desarrollo. Las evoluciones atípicas tienen en común una actividad epiléptica persistente durante el sueño lento, que desempeña un papel importante en el desarrollo de los déficits neurocognitivos que se asocian a esta patología. Factores como la edad de inicio de la epilepsia, el inicio de la evolución atípica, la localización de las descargas interictales y la actividad epiléptica continua durante el sueño que persista durante más de dos años pueden provocar cambios en el funcionamiento de las redes neurocognitivas, con los consecuentes déficits en las funciones neuropsicológicas, que incluso pueden resultar irreversibles. Conclusiones. Es necesario un seguimiento cercano tanto clínico como electroencefalográfico; además, deben realizarse estudios neuropsicológicos formales desde el inicio de la epilepsia benigna de la infancia con puntas centrotemporales y más en los casos que es evidente una evolución atípica para detectar y prevenir los déficits neuropsicológicos antes de que se instauren definitivamente (AU)

Introduction. Rolandic epilepsy or benign childhood epilepsy with centrotemporal spikes is called benign because its seizures are usually favourable and due to the spontaneous normalisation of the electroencephalogram on reaching puberty. Nevertheless, evidence has been found of the impact on cognitive development with the presence of heterogeneous cognitive deficits, especially related to persistent intercritical discharges during non-REM sleep. The aim of this study is to examine the epileptogenic networks involved in the neuropsychological disorders of this pathology. Development. A common feature of the atypical developments is persistent epileptic activity during slow sleep, which plays an important role in the development of the neurocognitive deficits that are associated to this pathology. Factors such as the age at onset of the epilepsy, the onset of the atypical development, the location of the interictal discharges and the continuous epileptic activity during sleep that persists for more than two years can trigger changes in the functioning of the neurocognitive networks. This may result in deficits in the neuropsychological functions, which may even be irreversible. Conclusions. A close clinical and electroencephalographic follow-up is necessary. Moreover, formal neuropsychological studies must be conducted as of the onset of benign childhood epilepsy with centrotemporal spikes. This is even more necessary in cases in which there is an obvious atypical development in order to detect and prevent the neuropsychological deficits before they establish themselves on a definitive basis (AU)

Averaged EEG spike dipole analysis may predict atypical outcome in Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS).

PURPOSE: Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS) is the most common childhood idiopathic focal epilepsy with a good outcome. However, an increasing number of reports suggest a 'not so benign' outcome showing atypical evolution. METHODS: We analyzed the electroencephalographic (EEG) spike source dipoles of BCECTS patients to compare patients with confirmed typical and atypical outcome showing intractable epilepsy and cognitive decline. Thirty-seven patients that were diagnosed for BCECTS and whose clinical information was sufficient enough to confirm long-term outcome were included in the study. Eight patients (22%, 7 patients for poor seizure control and 1 patient for cognitive decline) were classified as showing atypical outcome. Forty-seven averaged spike dipole sources were analyzed using the single equivalent current dipole (ECD) method. The inverse problem was solved using the 4 shells ellipsoidal model. RESULTS: The coordinate value of yori differed significantly between the two groups (mean±standard deviation, 0.32±0.33 for the typical group and -0.33±0.77 for the atypical group, P<0.001). These findings suggest that the averaged spike source dipoles were oriented anteriorly in patients with typical outcome and posteriorly in patients with atypical outcome. Spike source dipoles seemed to be located higher in patients with atypical presentation but this finding was insignificant. CONCLUSION: We can conclude that there are significant differences in spike source dipole in patients with BCECTS that shows atypical outcome, and we can use this finding as a marker for predicting an outcome at the time of diagnosis.

The impact of an exercise therapy on psychosocial health of children with benign epilepsy: a pilot study.

OBJECTIVES: The purposes of the current study were to test the feasibility of exercise therapy for children with benign epilepsy with centrotemporal spikes (BECTS) and to collect pilot data about the impact of exercise therapy on neurocognitive, emotional, and behavioral outcomes. METHODS: Ten children with BECTS (9.7±1.42 years) participated in a therapeutic exercise program consisting of ten supervised exercise sessions and home-based exercises for five weeks. Electroencephalography (EEG), seizure frequency, and neurocognitive and psychological factors, including attention, executive function, depression, anxiety, behavioral problems, and quality of life, were assessed before and after the exercise program. RESULTS: No clinical symptoms were observed to worsen during the study, demonstrating that the exercise therapy was safe and also feasible. After five weeks of exercise therapy, significant improvements in neurocognitive domains such as simple visual and auditory attention, sustained attention, divided attention, psychomotor speed, and inhibition-disinhibition were observed. Furthermore, parent ratings of internalizing behavioral problems and social problems and mood-related well-being from quality of life improved after exercise therapy. Although not statistically significant, trends were noted toward improvement in children's self-reports of negative mood/somatization, parent reports of somatic complaints, and general health on a quality-of-life measure. CONCLUSIONS: A five-week structured exercise program was successfully implemented, with preliminary data suggesting beneficial impact on neurocognitive and psychobehavioral function. Exercise therapy should be further evaluated as a part of a comprehensive treatment program for children with benign epilepsy.

Panayiotopoulos syndrome with convulsive status epilepticus at the onset: a long-term study.

PURPOSE: To better define the convulsive status epilepticus (CSE) as a possible manifestation at the onset of Panayiotopoulos syndrome (PS) and to assess its prognostic value in these children. METHODS: Children with CSE and diagnostic criteria of PS were identified, followed clinically and compared with a group of patients with PS without CSE from 1993 to 2012. RESULTS: We identified 37 patients with CSE at the onset of PS. During the same period we identified 72 children with autonomic symptoms of PS without CSE. The first episode of CSE occurred at a mean age of 6.5 years. Generalized clonic seizures were the most common ictal event and one-third of the patients required admission to Intensive Care Units. Interictal EEGs showed occipital spike activity in 31 (83.7%) subjects. Only 14 (37.8%) patients were treated with valproic acid and for two of them (5.40%) it was necessary to administer other drugs. There were no intractable cases. The overall prognosis was excellent. After the first event, 15 subjects (40.54%) experienced at least another typical PS seizure, but all patients were seizure free at the last follow-up. CONCLUSION: CSE is not uncommon in PS and it may occur at the onset of benign childhood epilepsy, without leading to a poor prognosis.

Panayiotopoulos syndrome: a case series from Turkey.

The aim of the study was to evaluate the demographic, clinical, and EEG characteristics of patients with Panayiotopoulos syndrome (PS) and the course of their illness. Thirty-eight patients followed up with a diagnosis of PS between January 2011 and December 2013 were evaluated. We found high rates of personal history of febrile convulsions, breath-holding spells, and family history of febrile convulsions, afebrile convulsion/epilepsy, migraine, and breath-holding spells. Seizures started before the age of eight in 87% of the patients, and the mean age at seizure onset was 4.6 years. Seizures were sleep-related in 81.5%, and autonomic status was seen in a third of the patients. The number of seizures was between 2 and 10 in 66% of the patients. The most common symptoms were ictus emeticus, eye/head deviation, and altered consciousness. Rolandic features were seen in 26% of the patients, and visual symptoms in 5%. Multifocal epileptiform discharges on EEG were identified in 84% of the patients. Two or more antiepileptic drugs were required in only 13% of the patients. Evolution to electrical status epilepticus in sleep and Gastaut-type epilepsy were seen in patients with more than ten seizures. The high rates of febrile convulsions, afebrile convulsions/epilepsy, migraine, and breath-holding spells in the patients and families suggest the importance of genetic factors and, perhaps, a common pathogenesis. However, the high rates of febrile convulsions and breath-holding spells in patients can be related to a misdiagnosis because of the similar symptoms. Despite its disturbing symptoms, PS is a benign epileptic syndrome requiring multiple antiepileptic drug use only in a small proportion of patients.

Evaluation of a neurotherapy program for a child with ADHD with Benign Partial Epilepsy with Rolandic Spikes (BPERS) using event-related potentials.

BACKGROUND: We hypothesized that there would be a good response to relative beta training, applied to regulate the dynamics of brain function in a patient with benign partial epilepsy with Rolandic Spikes (BPERS), associated with neuropsychiatric deficits resembling the symptoms of attention deficit-hyperactivity disorder (ADHD). CASE REPORT: The patient, E.Z., age 9.3, was suffering from neuropsychiatric symptoms, cognitive dysfunction, especially attention deficits, and behavioral changes, rendering him unable to function independently in school and in many situations of everyday life. He was treated for epilepsy, but only slight progress was made. The patient took part in 20 sessions of relative beta training combined with behavioral training. We used standardized neuropsychological testing, as well as ERPs before the experiment and after the completion of the neurotherapy program. Neuropsychological testing at baseline showed multiple cognitive deficits. Over the course of neurotherapy, E.Z.'s verbal and non-verbal IQ increased significantly. His cognitive functions also improved, including immediate and delayed logical and visual recall on the WMS-III, maintaining attention on the WMS-III, and executive functions, but remained below norms. Physiologically, the patient showed substantial changes after neurotherapy, including fewer spikes and an increased P300 NOGO component. CONCLUSIONS: The cognitive deficits characteristic for ADHD in a child with BPERS may be unresponsive to antiepileptic treatment, but are reversible after a carefully selected neurotherapy program, combined with antiepileptic treatment. Event Related Potentials (ERPs) in the GO/NOGO task can be used to assess functional brain changes induced by neurotherapeutical programs.

[Rolandic epilepsy clinical variants and their influence on the prognosis]. / Variantes clínicas de epilepsia rolándica y su implicación en el pronóstico.

INTRODUCTION: Benign epilepsy with central-temporal spikes (BECTS) is the most common epileptic syndrome in childhood. It is an age-dependent, genetically determined and benign condition. The aim of this study is to describe the clinical course and prognosis in 60 patients with BECTS diagnosed in our hospital. PATIENTS AND METHODS: We made a retrospective review of patients diagnosed with BECTS in a University Hospital (1995-2009). They were divided into 2 groups: 1) Patients who met all BECTS classical criteria. 2) Patients who met all the criteria but one (less than 4 years; diurnal seizures; atypical EEG abnormalities). RESULTS: A total of 60 patients, 34 males and 26 females were included, with 31 patients in group 1 and 29 in group 2. The mean age at onset in group 1: 7.45 years, group 2: 6.55 years. Medical treatment was indicated in 32.2% of patients in group 1 and 41.3% in group 2. The outcome was favourable in the majority: 58% in group 1 and 62.1% in group 2 were free of seizures after 1 year. Average age in which it disappeared: 8.54 years in group 1 and 7.84 years in group 2. There were no statistically significant differences in any of these parameters. CONCLUSIONS: Unlike that published by some authors, we have not identified any poor outcome factors in patients with BECTS in this study, meaning that an accurate diagnosis correlates with a good prognosis and excellent neurological outcome.

Cognitive and linguistic abnormalities in benign childhood epilepsy with centrotemporal spikes.

AIM: To assess the cognitive function and language ability in children with benign partial epilepsy with centrotemporal spikes. METHODS: Twenty-five patients with benign partial epilepsy with centrotemporal spikes were included. They were divided into two subgroups. Group I: 10 patients with rolandic focus who were not treated. Group II: 15 patients with rolandic focus receiving treatment. A third Group of 12 healthy subjects have been studied. All children underwent standardized neuropsychological testing: electroencephalogram recording, Wechsler Intelligence Scale for Children-revised, Peabody Picture Vocabulary Test-III (PPVT-III) and Boston Naming Test (BNT), both during active disease (T1) and 2 years after recovery from epilepsy (T2). RESULTS: At T1 evaluation, no significant differences in group I and II patients about general intelligence, when compared with controls, were found. Group I and II patients were impaired with respect to controls in the receptive and expressive vocabulary evaluated with PCVT-III and BNT, respectively. At T2 evaluation, group I and II patients showed a normalization of the language abnormalities. CONCLUSION: Deficits of speech-related abilities can be detected in children with this type of epilepsy: these dysfunctions seem to be independent of the effects of antiepileptic treatment and are reversible after remission of epilepsy.

Treatment of benign focal epilepsies in children: when and how should be treated?

Benign focal epilepsies represent almost one-fourth of all childhood epilepsies and are a frequent occurrence in clinical practice. They include benign infantile seizures (BIS), Panayiotopoulos syndrome (PS), and benign childhood epilepsy with centrotemporal spikes (BCECTS) in this order of the onset age. Because the prognosis is always excellent in patients with benign focal epilepsies, we must consider the risks and benefits of chronic antiepileptic drug (AED) administration. AED treatment is usually not recommended for the patients with a first attack, but should be considered for those with a second or third attack. A choice of AED has been based on the expert opinion. Carbamazepine (CBZ) is recommended for both acute and chronic treatment of seizure clusters in patients with BIS. Valproic acid (VPA), CBZ or clobazam (CLB) appears to be a first option of AED for patients with PS. A common first choice for BCECTS is CBZ in the USA and Japan, and VPA in the EU. The treatment period should be as short as possible without waiting for EEG normalization, possibly within 2 years after the initiation of AED. We must remember that some patients with BCECTS may have an "atypical evolution". In conclusion, when and how to treat this benign condition should be determined in an individual manner based on the length and frequency of seizures, circadian rhythm of the attacks, interictal EEG findings, cognitive and behavioral functions in daily life and the attitude of the parents toward seizure recurrences and AED side effects.

Benign epilepsy of childhood with centrotemporal spikes (BECTS): to treat or not to treat, that is the question.

The goal of this review is to assess the value of treating versus not treating benign epilepsy (of childhood) with centrotemporal spikes (BECTS). Among 110 recommendations from 96 articles, two-thirds generally favored and one-third generally did not favor treatment with antiepileptic drugs (AEDs). Two studies concluded that all patients should be treated, but most investigators added qualifications, for example, treating those with early onset, multiple seizures at onset, and large numbers of seizures, especially generalized tonic-clonic seizures, and limiting treatment to 1 year. Other studies made treatment dependent on epileptiform discharges or amelioration of symptoms. Specific AEDs were reviewed, and in the largest number of positive studies, valproic acid or carbamazepine was favored. Among the studies generally opposing treatment, none opposed treatment for all patients in all circumstances. Usually, qualifications to treat were added, for example, if generalized tonic-clonic seizures occurred or if there was a change in quality of life. One AED associated with negative effects was carbamazepine, treatment with which can result in the development of epileptic negative myoclonus, absence seizures, and generalized spike-wave complexes on the EEG. Thus, if treatment is planned, valproic acid may be considered the drug of choice in BECTS. Although many neurologists oppose treatment; twice as many studies concluded in favor of treatment. The typical benign aspect of this disorder may allow for nontreatment to be without serious consequences.

Epilepsia rolándica: características epidemiológicas, clínicas y evolutivas / Rolandic epilepsy: Epidemiological and clinical characteristics and outcome

Objetivo: Analizar las características epidemiológicas, clínicas y evolutivas de la epilepsia rolándica para facilitar su sospecha diagnóstica en la práctica clínica diaria. Pacientes y métodos: Se han revisado 56 historias clínicas de pacientes con epilepsia rolándica y se han registrado características epidemiológicas y clínicas, exploraciones complementarias y datos evolutivos. Los criterios diagnósticos aplicados fueron los de la International League Against Epilepsy. Resultados: La edad media al diagnóstico era de 7,7 años. El 62,5 % fueron diagnosticados en edad escolar, con mayor prevalencia del sexo masculino (58,9 %). El 80,4 % de los pacientes tuvieron crisis exclusivamente durante el sueño caracterizadas por contracciones hemifaciales con desviación ocular y/o cefálica (76,8 %), sialorrea (44,6 %), sonidos guturales (30,6 %), crisis motoras secundariamente generalizadas (35,7 %) y/o hemicorporales (26,8 %), disartria (17,9 %) y parestesias unilaterales (16,1 %). Se constataron paroxismos de localización centrotemporal, preferentemente unilaterales (78,6 %). El 50,7 % de las recurrencias se dieron en los primeros 12 meses tras el diagnóstico, el 24,6 % entre los 12 y 24 meses, y el 24,6 % restante entre los 2 y 4 años. Se objetivaron 2 casos de evolución atípica: uno de actividad continua de punta-onda en sueño lento, y otro de afasia adquirida (síndrome de Landau-Kleffner). Conclusiones: La epilepsia rolándica constituye un síndrome epiléptico específicamente pediátrico que afecta preferentemente a varones en edad escolar. Su secuencia semiológica es bastante característica, y es imprescindible documentar paroxismos centrotemporales para su diagnóstico. Su pronóstico es excelente; sin embargo, dado que algunos pacientes cursan una evolución atípica y/o una afectación cognitiva transitoria sería conveniente mantener un riguroso control evolutivo (AU)

Objective: To analyse the epidemiological, clinical and developmental characteristics of Rolandic epilepsy as an aid to its suspected diagnosis in daily clinical practice. Patients and methods: The medical records of 56 patients with Rolandic epilepsy were reviewed in order to collect epidemiological and clinical features, results of complementary examinations and developmental data. The criteria defined by the International League Against Epilepsy (ILAE) were used in the diagnosis. Results: Mean age at diagnosis was 7.7 years. In all, 62.5 % were diagnosed at school age, with a higher prevalence of males (58.9 %). Seizures occurred during sleep in 84.4 % of patients, and they were mainly characterised by hemifacial seizures with eye deviation and/or headaches (76.8 %), hypersalivation (44.6 %), guttural sounds (30.6 %), secondary generalised tonic-clonic (35.7 %) and/or unilateral clonic or tonic seizures (26.8 %), dysarthria (17.9 %) and unilateral paresthesias (16.1 %). Inter-ictal EEG showed paroxysms in the centrotemporal regions, frequently unilateral (78.6 %). Of all recurrences, 50.7 % occurred during the first 12 months after diagnosis, 24.6 % between 12 and 24 months after diagnosis, and 24.6 % between 2 and 4 years of follow up. Two patients with atypical progression were recorded: a case with epilepsy with continuous spikes and waves during slow-wave sleep, and another case with a Landau-Kleffner syndrome. Conclusions: Rolandic epilepsy is a common type of epilepsy in the pediatric age group and generally begins at school-aged children. Its semiological sequence is fairly characteristic, and finding centrotemporal spikes is considered as necessary for the syndromic diagnosis. The prognosis is excellent; however, as a few patients may progress to atypical outcomes and/or neuropsychological deficits, a rigorous developmental control of these patients should be of the highest priority (AU)

Acupuncture treatment of Jacksonian epilepsy--a report of 98 cases.

OBJECTIVE: To evaluate the effect of acupuncture treatment for controlling Jacksonian epilepsy. METHOD: Penetrating needling is adopted together with scalp acupuncture and strong/electric needling on body points. RESULTS: Of the 98 cases treated for 5 courses, 52 cases were markedly effective, 22 effective, 10 poorly effective and 14 ineffective. The total effective rate was 85.7% (95% CI = 78.8%-92.6%). CONCLUSION: Acupuncture is quite effective for treating Jacksonian epilepsy.

Panayiotopoulos syndrome: a benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis.

BACKGROUND: Panayiotopoulos syndrome is a common idiopathic childhood-specific seizure disorder formally recognized by the International League Against Epilepsy. An expert consensus has defined Panayiotopoulos syndrome as "a benign age-related focal seizure disorder occurring in early and mid-childhood. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms, and by an EEG [electroencephalogram] that shows shifting and/or multiple foci, often with occipital predominance." OBJECTIVE: The purpose of this review is to provide guidance for appropriate diagnosis and management of Panayiotopoulos syndrome. CLINICAL FEATURES: Autonomic epileptic seizures and autonomic status epilepticus are the cardinal manifestations of Panayiotopoulos syndrome. Autonomic seizures in Panayiotopoulos syndrome consist of episodes of disturbed autonomic function with emesis as the predominant symptom. Other autonomic manifestations include pallor (or, less often, flushing or cyanosis), mydriasis (or, less often, miosis), cardiorespiratory and thermoregulatory alterations, incontinence of urine and/or feces, hypersalivation, and modifications of intestinal motility. In approximately one fifth of the seizures the child becomes unresponsive and flaccid (ictal syncope) before or often without convulsions. Cardiorespiratory arrest is exceptional. More-conventional seizure symptoms often appear after the onset of autonomic manifestations. The child, who was initially fully conscious, becomes confused and unresponsive. Eyes turn to one side or gaze widely open. Only half of the seizures end with brief hemiconvulsions or generalized convulsions. Convulsive status epilepticus is extremely rare. Autonomic symptoms may be the only features of the seizures. Half of the seizures in Panayiotopoulos syndrome last for >30 minutes, thus constituting autonomic status epilepticus, which is the more common nonconvulsive status epilepticus in normal children. Two thirds of seizures occur during sleep. EPIDEMIOLOGY: Panayiotopoulos syndrome probably affects 13% of children aged 3 to 6 years who have had 1 or more afebrile seizures and 6% of such children in the 1- to 15-year age group. DIAGNOSTIC TESTS: An electroencephalogram is the only investigation with abnormal results, usually showing multiple spikes in various brain locations. PATHOPHYSIOLOGY: Panayiotopoulos syndrome is probably the early-onset and Rolandic epilepsy the late-onset phenotype of a maturation-related benign childhood seizure-susceptibility syndrome. Ictal epileptic discharges in Panayiotopoulos syndrome, irrespective of their location at onset, activate autonomic disturbances and emesis, to which children are particularly vulnerable. The symptoms/sequence of autonomic seizures and autonomic status epilepticus in Panayiotopoulos syndrome are specific to childhood, and they do not occur in adults. PROGNOSIS: Panayiotopoulos syndrome is remarkably benign in terms of seizure frequency and evolution. Autonomic status epilepticus imparts no residual neurologic deficit. The risk of epilepsy in adult life seems to be no higher than in the general population. However, autonomic seizures are potentially life-threatening in the rare context of cardiorespiratory arrest, an area in which additional study is required. MISDIAGNOSIS: The clinical features of Panayiotopoulos syndrome are frequently mistaken as nonepileptic conditions such as acute encephalitis, syncope, migraine, cyclic vomiting syndrome, motion sickness, sleep disorder, or gastroenteritis. The consequence is avoidable misdiagnosis, high morbidity, and costly mismanagement. MANAGEMENT: Education about Panayiotopoulos syndrome is the cornerstone of management. Prophylactic treatment with antiepileptic medication may not be needed for most patients. Autonomic status epilepticus in the acute stage needs thorough evaluation; aggressive treatment may cause iatrogenic complications including cardiorespiratory arrest.

[Rolandic epilepsy. An analysis of the clinical and electrophysiological characteristics, treatment and prognosis in 87 patients]. / Epilepsia rolándica. Análisis de las características clínicas, electrofisiológicas, tratamiento y pronóstico en 87 pacientes.

INTRODUCTION: Rolandic epilepsy is the most common epileptic syndrome in infancy. It usually has a favourable prognosis and seizures disappear during the teenage years. AIMS: The aim of this study was to describe a sample of children diagnosed with rolandic epilepsy in clinical, electrophysiological and imaging terms. We also intend to compare the results obtained with those described in the literature. PATIENTS AND METHODS: We conducted a retrospective, descriptive study of the children diagnosed with rolandic epilepsy who had been submitted to a follow-up at the Neuropaediatrics Unit at the HGSA since 1989. Children who were less than 2 years old when they suffered their first seizures or who had abnormal neurological/imaging examinations were excluded. Two groups were defined (typical and atypical), several variables were characterised and these were then analysed statistically. RESULTS: A total of 87 children (51 males) were included in this study, their mean age being 13.6 years. The population was divided into two groups: A (typical cases; n = 69) and B (atypical cases; n = 18). The mean age at the onset of seizures was 6.2 and 6 years for groups A and B, respectively. The predominant type of seizures was simple partial for group A and complex partial for group B; they were mainly sporadic and nocturnal in both groups. Medication was administered to 51 (73.9%) of the children in group A, with a good response in 78.4% of them; 13 members of group B (72.2%) received medication, with a positive response in 76.9% of them. CONCLUSIONS: Rolandic epilepsy has a broad clinical spectrum and usually courses with normal psychomotor development, but may coexist with learning disabilities. In this study, no significant differences were found between the typical and atypical forms of presentation, as far as their progress and response to treatment are concerned.