Epilepsy and inborn errors of metabolism in adults: The diagnostic odyssey of a young woman with medium-chain acyl-coenzyme A dehydrogenase deficiency.

We describe a case of epileptic encephalopathy in a young woman with undiagnosed medium-chain acyl-coenzyme A dehydrogenase deficiency (MCADD), who presented with an early-onset focal motor status epilepticus (SE) then followed by permanent left hemiplegia and drug-resistant epilepsy with neurodevelopmental delay. Throughout her clinical history, recurrent episodes of lethargy, feeding difficulties, and clustering seizures occurred, progressing into a super refractory SE and death at the age of 25 years. Although epilepsy is not a distinctive feature of MCADD, we advise considering this metabolic disease as a possible etiology of epileptic encephalopathy and hemiconvulsion-hemiplegia-epilepsy syndrome of unknown origin, on the chance to provide a timely and targeted treatment preventing development delay and evolution to SE. Adult patients with epilepsy of unknown etiology not screened at birth for inborn errors of metabolism, such as MCADD, should be promptly investigated for these treatable conditions.

The potential impact of CYP2D6 (*2/*4/*10) gene variants among Egyptian epileptic children: A preliminary study.

BACKGROUND: The cytochrome P450 (CYP) isoenzymes have an indispensable role in the metabolic phase of different medications during the treatment of multiple neuropsychiatric disorders. The foremost goal of this study is to evaluate the correlation of the allelic variants within CYP2D6 (*2/*4/*10) gene with the susceptibility for epileptic syndrome as well as the assessment the degree of resistance towards antiepileptic drugs (AEDs). METHODS: This work was designed based on the involvement of 200 participants [100 unrelated healthy controls, 50 AEDs responsive, and 50 AEDs resistant]. Genomic DNA for the CYP2D6 variants was genotyped utilizing the T-ARMS-PCR technique. RESULTS: The distributions of the CYP2D6*2 (rs16947; c.886C > T) and CYP2D6*4 (rs3892097; c.506-1G > A) variants were significantly correlated with elevated risk among epileptic patients compared to healthy controls (P-value < 0.05). Furthermore, the CYP2D6*2 variant was statistically associated with disease risk among AEDs responsive patients, while the CYP2D6*4 variant was statistically correlated with disease risk among AEDs resistant patients (P-value < 0.05). Interestingly, the allelic variants of the CYP2D6*4 (A allele) and CYP2D6*10 (T allele) were associated with elevated risk among AEDs resistant compared to AEDs responsive patients (P-value = 0.008 and 0.040, respectively). CONCLUSIONS: The CYP2D6*2 and CYP2D6*4 variants were recognized as independent risk factors among epileptic patients, but not the CYP2D6*10 variant.

Deep feature fusion based childhood epilepsy syndrome classification from electroencephalogram.

Accurate classification of the children's epilepsy syndrome is vital to the diagnosis and treatment of epilepsy. But existing literature mainly focuses on seizure detection and few attention has been paid to the children's epilepsy syndrome classification. In this paper, we present a study on the classification of two most common epilepsy syndromes: the benign childhood epilepsy with centro-temporal spikes (BECT) and the infantile spasms (also known as the WEST syndrome), recorded from the Children's Hospital, Zhejiang University School of Medicine (CHZU). A novel feature fusion model based on the deep transfer learning and the conventional time-frequency representation of the scalp electroencephalogram (EEG) is developed for the epilepsy syndrome characterization. A fully connected network is constructed for the feature learning and syndrome classification. Experiments on the CHZU database show that the proposed algorithm can offer an average of 92.35% classification accuracy on the BECT and WEST syndromes and their corresponding normal cases.

Burden of seizures and comorbidities in patients with epilepsy: a survey based on the tertiary hospital-based Epilepsy Syndrome Registry in Japan.

OBJECTIVE: To examine the current medical and psychosocial status of patients with epilepsy, aiming to facilitate appropriate application of the Intractable/Rare Diseases Act of Japan. METHODS: By analysing the cross-sectional data of patients registered in the tertiary hospital-based Epilepsy Syndrome Registry of Japan, we investigated the proportion of patients who met the severity criteria as defined by the Act (seizure frequency of at least once a month, or presence of intellectual/neurological/psychiatric symptoms, or both) and whether there are candidate syndrome/diseases to be added to the existing list in the Act. RESULTS: In total, 2,209 patients were registered. After excluding self-limited/idiopathic epilepsies, 1,851 of 2,110 patients (87.7%) met the severity criteria. The patients were classified into eight main epilepsy syndromes (594 patients), 20 groups based on aetiology (1,078 patients), and three groups without known aetiology (427 patients). Most of the groups classified by syndrome or aetiology had high proportions of patients satisfying the severity criteria (>90%), but some groups had relatively low proportions (<80%) resulting from favourable outcome of surgical therapy. Several small groups with known syndrome/aetiology await detailed analysis based on a sufficiently large enough number of patients registered, some of whom may potentially be added to the list of the Act. SIGNIFICANCE: The registry provides data to examine the usefulness of the severity criteria and list of diseases that are operationally defined by the Act. Most epilepsy patients with various syndromes/diseases and aetiology groups are covered by the Act but some are not, and the list of designated syndromes/diseases should be complemented by further amendments, as suggested by future research.

Trends and Costs Associated With the Diagnosis and Treatment of Infantile Spasms: A 10-Year Multicenter Retrospective Review.

OBJECTIVE: Early treatment of infantile spasms (IS) may be imperative for improvement of neurodevelopmental outcomes. Existing studies have led to inconclusive recommendations with variation in treatment. Our objective was to determine the national average cost, initial diagnostic workup, treatments, and hospital length of stay for patients with IS. METHODS: This retrospective cohort study was designed to review data of patients < 2 years from 43 non-profit institutions. Data obtained included patient demographics, length of stay, admission cost, and treatments used from 2004 to 2014. Cost data were collected and adjusted to 2014 dollars, the year data were analyzed. RESULTS: A total of 6183 patients met study criteria (n = 3382, 55% male). Three-quarters of patients (n = 4684, 76%) had an electroencephalogram, 56.4% had brain imaging (n = 3487), and 17% (n = 1050) underwent a lumbar puncture. Medication for IS was initiated during inpatient hospital stay in two-thirds of all patients (n = 4139, 67%). Most patients were initiated on corticotropin (n = 2066, 33%) or topiramate (n = 1804, 29%). Average length of stay was 5.8 days with an average adjusted cost of $18,348. Over time there was an 86.6% increase in cost from an average $12,534.54 (2004) to $23,391.20 (2014), a significant change (p < 0.01). This correlated with an increase in average length of stay. CONCLUSIONS: Variability exists in diagnostic workup and pharmacotherapy initiated for IS, which may lead to differences in the cost of hospital stay. Further studies may help determine contributing factors to increased cost and improve health care utilization for IS patients.

How soon should urgent EEG be performed following a first epileptic seizure?

PURPOSE: Patients with a first unprovoked epileptic seizure are often seen in emergency services. Electroencephalography (EEG) is indicated for diagnosing epilepsy, but the optimal time to perform this test has not been defined. This study aimed to determine the time interval following a seizure within which EEG has the greatest diagnostic yield. METHODS: We conducted a retrospective study of all adult patients with a first unprovoked seizure who had undergone emergency EEG (July 2014-December 2019). Data collection included demographics, seizure type, time interval to EEG study, EEG pattern identified, and the prescription after emergency assessment. An optimal cut-off point for time to EEG was obtained, and an adjusted regression model was performed to establish associations with the presence of epileptiform abnormalities. RESULTS: A total of 170 patients were included (mean age: 50.7 years, 40.6% women). Epileptiform discharges were identified in 34.1% of recordings, nonepileptiform abnormalities in 46.5%, and normal findings in 19.4%. A lower latency from seizure to EEG was associated with a higher probability of finding epileptiform discharges (median: 12.7 in the epileptiform EEGs vs. 20 h in the nonepileptiform EEGs, p < 0.001). The time interval associated with the highest probability of detecting an epileptiform EEG pattern was within the first 16 h after seizure onset: 52.1% of recordings performed before the 16-h cut-off showed these abnormal patterns compared with 20.2% performed after (p < 0.001). These findings were not related to the presence of an epileptogenic lesion in neuroimaging or to other clinical variables. The finding of epileptiform abnormalities was followed by a greater prescription of antiseizure drugs (96.4% vs. 66% in nonepileptiform patterns, p < 0.001). CONCLUSION: The diagnostic yield of EEG following a first unprovoked epileptic seizure is highest when this test is performed within the first 16 h after onset of the event.

Thermolabile polymorphism of carnitine palmitoyltransferase 2: A genetic risk factor of overall acute encephalopathy.

OBJECTIVES: Acute encephalopathy is an acute brain dysfunction after preceding infection, consisting of multiple syndromes. Some syndromes, such as acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), are severe with poor outcome, whereas others, such as clinically mild encephalitis/encephalopathy with reversible splenial lesion (MERS), are mild with favorable outcome. Previous study reported the association of the thermolabile polymorphism in Carnitine Palmitoyltransferase 2 (CPT2) gene and severe syndromes of acute encephalopathy. To further explore the pathogenetic role of CPT2 in acute encephalopathy, we conducted a case-control association study of a typical thermolabile CPT2 polymorphism, rs2229291, in 416 patients of acute encephalopathy, including both severe and mild syndromes. METHODS: The case cohort consisted of 416 patients, including AESD, MERS, and other syndromes. The control subjects were 100 healthy Japanese. rs2229291 was genotyped by Sanger sequencing. Genetic distribution was compared between the patients and controls using Cochran-Armitage trend test. RESULTS: Minor allele frequency of rs2229291 was significantly higher in AESD (p = 0.044), MERS (p = 0.015) and entire acute encephalopathy (p = 0.044) compared to the controls. The polymorphism showed no significant association with influenza virus, or with outcome. CONCLUSIONS: This study provided evidence that CPT2 is a susceptibility gene for overall acute encephalopathy, including both severe and mild syndromes, and suggested that impairment of mitochondrial metabolism is common to various syndromes of acute encephalopathy.

[Vomiting as a symptom of epilepsy. Panayitopoulos Syndrome - review of the literature and own experience].

Vomiting is a common sign of illness in the pediatric population. Its etiology is diversified, ranging from mild functional disorders to severe life-threatening systemic diseases. Vomiting most often occurs in the course of gastrointestinal tract diseases, however, it may also coexist with numerous other ailments located outside the GI tract. Due to its diverse etiology encompassing various systems and organs, it can sometimes cause diagnostic difficulties. The present paper illustrates a case of Panayiotopoulos syndrome, which is an early-onset childhood occipital epilepsy (EOCOE). Characteristic of this syndrome are seizures with symptoms originating from the autonomic nervous system or the occurrence of vegetative status epilepticus. The dominant signs and symptoms are vomiting and nausea, which in the first place most frequently suggest inflammation of the stomach or intestines, migraine, or a proliferative process in the central nervous system. Rarely is the possibility of vomiting taken into account as an element of epileptic seizure in the differential diagnosis. The aim of this paper is to draw attention to the difficulty in defining the precise cause of recurrent vomiting. Many times, despite collecting a detailed medical history and extensive physical examination, it is only observation-based diagnosis that allows the doctor to make a final evaluation.

Causes of mortality in early infantile epileptic encephalopathy: A systematic review.

INTRODUCTION: Early infantile epileptic encephalopathy syndrome (EIEE), also known as Ohtahara syndrome, is an age-dependent epileptic encephalopathy syndrome defined by clinical features and electroencephalographic findings. Epileptic disorders with refractory seizures beginning in the neonatal period and/or early infancy have a potential risk of premature mortality, including sudden death. We aimed to identify the causes of death in EIEE and conducted a literature survey of fatal outcomes. METHODS: We performed a literature search in MEDLINE, EMBASE, and Web of Science for data from inception until September 2017. The terms "death sudden," "unexplained death," "SUDEP," "lethal," and "fatal" and the medical subject heading terms "epileptic encephalopathy," "mortality," "death," "sudden infant death syndrome," and "human" were used in the search strategy. The EIEE case report studies reporting mortality were included. RESULTS: The search yielded 1360 articles. After screening for titles and abstracts and removing duplicate entries, full texts of 15 articles were reviewed. After reading full texts, 11 articles met the inclusion criteria (9 articles in English and 2 in Japanese, dated from 1976 to 2015). The review comprised 38 unique cases of EIEE, 17 of which had death as an outcome. In all cases, the suppression-burst pattern on electroencephalographies (EEGs) was common. Most cases (55%) involved male infants. The mean (standard deviation [SD]) age at onset of seizure was 19.6 ±â€¯33 days. The mean (SD) age at death was 12.9 ±â€¯14.1 months. Most infants (58.8%) survived less than one year. The cause of death was described only in eight (47%) patients; the cause was pneumonia/respiratory illness or sudden unexpected death in epilepsy (SUDEP). DISCUSSION: The results show EIEE as a severe disease associated with a premature mortality, evidenced by a very young age at death. Increasing interest in the detection of new molecular bases of EIEE is leading us to a better understanding of this severe disease, but well-reported data are lacking to clarify EIEE-related causes of death.

Electroclinical findings and long-term outcomes in epileptic patients with inv dup (15).

OBJECTIVE: To define the electroclinical phenotype and long-term outcomes in a cohort of patients with inv dup (15) syndrome. MATERIAL AND METHODS: The electroclinical data of 45 patients (25 males) affected by inv dup (15) and seizures were retrospectively analysed, and long-term follow-up of epilepsy was evaluated. RESULTS: Epilepsy onset was marked by generalized seizures in 53% of patients, epileptic spasms in 51%, focal seizures in 26%, atypical absences in 11% and epileptic falls in 9%. The epileptic syndromes defined were: generalized epilepsy (26.7%), focal epilepsy (22.3%), epileptic encephalopathy with epileptic spasms as the only seizure type (17.7%) and Lennox-Gastaut syndrome (33.3%). Drug-resistant epilepsy was detected in 55.5% of patients. There was a significant higher prevalence of seizure-free patients in those with seizure onset after the age of 5 years and with focal epilepsy, with respect to those with earlier epilepsy onset because most of these later developed an epileptic encephalopathy (69.2% vs 34.4%; P = .03), usually Lennox-Gastaut Syndrome in type. In fact, among patients with early-onset epilepsy, those presenting with epileptic spasms as the only seizure type associated with classical hypsarrhythmia achieved seizure freedom (P < .001) compared to patients with spasms and other seizure types associated with modified hypsarrhythmia. CONCLUSIONS: Epilepsy in inv dup (15) leads to a more severe burden of disease. Frequently, these patients show drug resistance, in particular when epilepsy onset is before the age of five and features epileptic encephalopathy.

Early childhood myoclonic epilepsy: An independent genetic generalized epilepsy with myoclonic seizures as the main seizure type.

OBJECTIVE: To elucidate the characteristics of the myoclonic seizures alone, or predominant myoclonus combined with generalized tonic-clonic seizures (GTCS) and/or absences, in early childhood, and discuss its classification. METHODS: Forty-two children were retrospectively recruited between January 2006 and June 2015. RESULTS: The mean age of seizure onset was 40.5months. They were divided into 4 groups: myoclonic seizures alone; predominant myoclonus combined with GTCS; predominant myoclonus combined with absences; predominant myoclonus combined with both GTCS and absences. Interictal EEG showed generalized spike- or polyspike-wave discharges at 2-4Hz. Seizures were controlled in 22 patients at a mean age of 60.5months. The psychomotor development was normal (30/37) or mildly delayed (7/37). CONCLUSIONS: We reported a cohort of patients with early childhood myoclonic epilepsy (ECME), with the following characteristics: Seizures started below 5years old in otherwise normal children; Seizure types included myoclonic seizures alone or combined with GTCS and/or absences; Febrile or afebrile GTCS might appear firstly; Interictal EEG showed generalized spike- or polyspike-wave; Seizures usually were in remission before adolescence with normal development or mild cognitive or behavioral deficits in most. SIGNIFICANCE: ECME might be an independent epileptic syndrome not established by International League Against Epilepsy (ILAE) previously.

Descriptive study of symptomatic epilepsy by age of onset in patients with a 3-year follow-up at the Neuropaediatric Department of a reference centre. / Estudio descriptivo de las epilepsias sintomáticas según edad de inicio controladas durante 3 años en una Unidad de Neuropediatría de referencia regional.

OBJECTIVE: We conducted a descriptive study of symptomatic epilepsy by age at onset in a cohort of patients who were followed up at a neuropaediatric department of a reference hospital over a 3-year period PATIENTS AND METHODS: We included all children with epilepsy who were followed up from January 1, 2008 to December 31, 2010 RESULTS: Of the 4595 children seen during the study period, 605 (13.17%) were diagnosed with epilepsy; 277 (45.79%) of these had symptomatic epilepsy. Symptomatic epilepsy accounted for 67.72% and 61.39% of all epilepsies starting before one year of age, or between the ages of one and 3, respectively. The aetiologies of symptomatic epilepsy in our sample were: prenatal encephalopathies (24.46% of all epileptic patients), perinatal encephalopathies (9.26%), post-natal encephalopathies (3.14%), metabolic and degenerative encephalopathies (1.98%), mesial temporal sclerosis (1.32%), neurocutaneous syndromes (2.64%), vascular malformations (0.17%), cavernomas (0.17%), and intracranial tumours (2.48%). In some aetiologies, seizures begin before the age of one; these include Down syndrome, genetic lissencephaly, congenital cytomegalovirus infection, hypoxic-ischaemic encephalopathy, metabolic encephalopathies, and tuberous sclerosis. CONCLUSIONS: The lack of a universally accepted classification of epileptic syndromes makes it difficult to compare series from different studies. We suggest that all epilepsies are symptomatic because they have a cause, whether genetic or acquired. The age of onset may point to specific aetiologies. Classifying epilepsy by aetiology might be a useful approach. We could establish 2 groups: a large group including epileptic syndromes with known aetiologies or associated with genetic syndromes which are very likely to cause epilepsy, and another group including epileptic syndromes with no known cause. Thanks to the advances in neuroimaging and genetics, the latter group is expected to become increasingly smaller.

[A study of epilepsy according to the age at onset and monitored for 3 years in a regional reference paediatric neurology unit]. / Estudio de las epilepsias según la edad de inicio, controladas durante 3 años en una unidad de neuropediatría de referencia regional.

OBJECTIVE: A study of epilepsy, according to the age at onset of the crisis and its causes, monitored by a Paediatric Neurology Unit over a period of three years. PATIENTS AND METHODS: Historical cohorts study was conducted by reviewing the Paediatric Neurology medical records data base of epileptic children followed-up from 1 January 2008 to 31 December 2010. RESULTS: A total of 4,595 children were attended during the study period. The diagnosis of epilepsy was established in 605 (13.17%): 277 (45.79%) symptomatic, 156 (25.79%) idiopathic, and 172 (28.43%) with cryptogenic epilepsy. Absence epilepsy and benign childhood epilepsy with centro-temporal spikes are the idiopathic epileptic syndromes most prevalent, and the most prevalent symptomatic epilepsies are prenatal encephalopathies. More than one-quarter (26.12%) of epilepsies began in the first year of life, and 67.72% were symptomatic. Refractory epilepsy was observed in 25.29%, 42.46% with cognitive impairment, 26.45% with motor involvement, and 9.92% with an autism spectrum disorder, being more frequent at an earlier age of onset. CONCLUSIONS: The absence of a universally accepted classification of epileptic syndromes makes tasks like this difficult, starting with the terminology. A useful classification would be aetiological, with two groups: a large group with established aetiology, or very likely genetic syndromes, and another with no established cause. The age of onset of epilepsy in each aetiological group helps in the prognosis, which is worsened by refractoriness and associated neurodevelopmental disorders, and are generally worse at an earlier onset and in certain aetiologies.

Pre-operative evaluation in pediatric patients with cortical dysplasia.

INTRODUCTION: Focal cortical dysplasia (FCD) is an important cause of refractory seizures and catastrophic epilepsy in infants and children who had epilepsy surgery. AIMS OF THE REVIEW: This manuscript will discuss age-related unique clinical characteristics in evaluation of infants and young children because the understanding of these age-related features is critical in selecting children who can benefit from epilepsy surgery. In addition, we will review the non-invasive tools available for the presurgical evaluation of children with FCD and their individual contribution to the formulation of the presurgical hypothesis.

The spectrum of epileptic syndromes with fixation off sensitivity persisting in adult life.

PURPOSE: The term "fixation off sensitivity" (FOS) was proposed by Panayiotopoulos to describe epilepsy/electroencephalography (EEG) changes evoked by the suppression of central vision and fixation. The EEG pattern usually consists of spike/polyspike and waves localized in occipital regions. FOS occurs mainly in children with idiopathic occipital partial epilepsies and rarely in adults. In this retrospective study we evaluated the clinical data, EEG, and magnetic resonance imaging (MRI) findings of patients with epilepsy and FOS persisting in adult life to better define the spectrum of syndromes. METHODS: We selected 15 consecutive patients (12 female/3 male; age range 19-59 years). The main inclusion criterion was the diagnosis of epilepsy with FOS persisting in adult life. We retrospectively analyzed clinical EEG and neuroimaging data. KEY FINDINGS: We observed a female prevalence (F/M = 12/3). Eight patients presented both simple and complex partial seizures, whereas seven had only complex partial seizures. Partial seizures evolved into generalized seizures/hemiconvulsions in nine cases. The FOS pattern consisted of spike-and-wave and slow-wave abnormalities with posterior localization (bilateral in eight/monolateral in seven). We recorded seizures in 10/15 patients. All showed a posterior onset (bilateral in 2/left in 2/right in 6). FOS was prevalent in symptomatic epilepsy (cortical malformations in 7; celiac disease in 3; calcified vascular malformation in 1). One patient presented cryptogenic epilepsy and only three idiopathic epilepsy (Gastaut syndrome). SIGNIFICANCE: FOS can be observed in adult life in idiopathic epilepsy, representing the "prolongation" of the same phenomenon arisen during childhood. Nevertheless, it often represents the EEG expression of symptomatic epilepsies (cortical malformations/celiac disease).

Spiritual/religious coping in patients with epilepsy: relationship with sociodemographic and clinical aspects and quality of life.

One hundred and ten patients with epilepsy with a mean age of 45.9 were assessed by a clinical-neurological evaluation, Quality of Life in Epilepsy Inventory-31 (QOLIE-31), and the Spiritual/Religious Coping (SRCOPE) Scale. The objective of this study was to evaluate if patients with epilepsy used positive and/or negative spiritual/religious coping and the relationships between this type of coping and the sociodemographic and clinical aspects of epilepsy and the QOLIE-31. A greater use of positive coping (3.0±0.7) than negative coping (2.3±0.7) was found. The use of the positive factor was greater in mesial temporal lobe epilepsy (MTLE) than in other types of epilepsy. The ratio of negative/positive coping was associated with lower scores in the QOLIE-31 (-0.222; p=0.036). Patients with epilepsy appear to use spiritual/religious coping, especially those with MTLE, and a predominance of negative coping was associated with a reduced quality of life. Future studies should evaluate interventions considering the knowledge of spiritual/religious strategies by the patients.

Epilepsy and quality of life: socio-demographic and clinical aspects, and psychiatric co-morbidity / Epilepsia e qualidade de vida: aspectos sociodemográficos, clínicos e comorbidade psiquiátrica

To study socio-demographic and clinical aspects, as well as psychiatric co-morbidity that influence the quality of life of adult epileptic patients.

Estudar os aspectos sociodemográficos, clínicos e comorbidades psiquiátricas que influenciam a qualidade de vida de pacientes adultos com epilepsia.

Síndrome opercular o síndrome perisilviano / Operculum syndrome or perisylvian syndrome

El interés de los autores es llamar la atención sobre el síndrome opercular, y estimular con ello su identificación en la práctica neuropediátrica. Se realizó una búsqueda en PubMed desde febrero de 2005 hasta septiembre de 2010, y se comentaron los artículos que, a consideración de los autores, mostraban los diferentes aspectos del concepto, historia, características clínicas, causas, así como del diagnóstico, evolución y pronóstico. El síndrome opercular puede ser de causa congénita o adquirida; en los adultos es más frecuente por infarto cerebral opercular bilateral, no así en los niños, en los que se puede presentar por diferentes causas, desde trastornos de la migración neuroblástica, hasta en la epilepsia. En niños epilépticos se debe estar atento a su evolución, ya que tanto por el tipo de epilepsia o síndrome epiléptico, como por la medicación antiepiléptica usada, puede presentarse este síndrome, teniendo una gran significación su identificación rápida y tratamiento adecuado

The interest of authors is to attract attention on operculum syndrome and thus to stimulate its identification in the neuropediatric practice. A search in PubMed from February, 2005 to September, 2010 was made commenting on papers that according authors showed the different features of concept, history, clinical features, causes, as well as diagnosis, evolution and prognosis. The operculum syndrome may be congenital or acquired; ion adults is more frequent by bilateral operculum cerebral infarction, but not in children in whom it may be present by different causes, from neuroblast migration to epilepsy. In the case of epileptic children it is necessary to pay attention to its course since due to the type of epilepsy or epileptic syndrome or due to antiepileptic drug used, this syndrome may be present, considering very much its fast identification and appropriate treatment

Anesthetic experience of a patient with Ohtahara syndrome -A case report-.

Ohtahara syndrome (OS) is a rare epileptic encephalopathy that is characterized by an abnormal electroencephalogram (EEG) and intractable seizures in the neonatal and early infantile period. The patient of this reported case was delivered normally at 39 weeks of gestation without any complication. One week after birth, seizures that were refractory to anticonvulsants started with repetitive clustered tonic spasms. The child had no abnormal findings on the initial laboratory investigations. But he was diagnosed with OS according to the frequent tonic spasms, an abnormal EEG pattern of suppression-burst and magnetic resonance imaging of cortical dysplasia. He was planned to undergo an operation for brain lesion. This report describes our experience with the general anesthetic management when we performed craniotomy and right hemispherotomy for a patient with OS.

Epilepsias parciales benignas de la infancia: Correlación entre factores de riesgo con la evolución clínica y hallazgos electroencefalográficos y su aplicación en el establecimiento de criterios terapéuticos / Benign partial epilepsy of infancy: The Correlation between risk factors, clinical evolution and electroencephalographic findings and the application it has over the therapeutic criteria

Definición: Las epilepsias parciales benignas de la infancia (EPBI) representan el síndrome epiléptico mas común en niños en edad preescolar y escolar, correspondiendo al 15-24% de las epilepsias, diagnosticadas entre los 3 y 13 años. Remiten espontáneamente al llegar a la adolescencia. Las epilepsias rolándicas constituyen las EPBI de mayor incidencia, su principal característica es ocasionar crisis parciales, con anartria, hemiconvulsiones faciales, en algunos casos con hemigeneralización secundaria. El electroencefalograma interictal evidencia espigas focales centrotemporales. En el 40% de los casos existe historia familiar de convulsiones febriles, epilepsias clínicas, o descargas epilépticas en el electroencefalograma. Además, el 7-10% evidencian antecedentes personales de convulsiones febriles en su primera infancia. Algunos autores sugieren no medicar estos pacientes, sin embargo no existe un consenso al respecto.Objetivos:•Determinar si los antecedentes convulsivos familiares o personales representan una influencia en la evolución de la enfermedad y si su presencia es un criterio para iniciar medicación antiepiléptica. Proponer criterios terapéuticos de medicación y de observación.Metodología: Estudio monocéntrico, longitudinal, tipo cohorte histórico, realizado con pacientes de consulta externa del hospital Pediátrico Dr. Roberto Gilbert Elizalde, durante 3 meses (noviembre/2003 Enero/2004). Criterios de inclusión: convulsiones no febriles de inicio entre 3 y 13 años con neurodesarrollo normal, estudios de imágenes normales, examen neurológico normal y trazado electroencefalográfico compatible con EPBI. Se clasificó a los pacientes en dos subgrupos: con y sin antecedentes convulsivos familiares o personales, y se comparó las diferencias clínicas -en cuanto al intervalo interictal- y electroencefalográficas.

Definition: Benign partial epilepsy of infancy represents the most common epileptic syndrome in preschool and school children. It accounts for 15-24% of epilepsies diagnosed between the ages of 3 and 13 years. Rolandic epilepsy is one of BMEI with a high incidence its main characteristic is that it causes partial seizures, anartia, hemiseizures. The electroencephalogram shows centrotemporal spikes. In the 40% of the cases exist family history of febrile seizures, clinical epilepsy or epileptic discharges in the electroencephalogram. Also, 7-10% have a clinical history of febrile seizures in infancy. Some authors suggest to not medicate this patients. Objectives: XDetermine the family and history of seizures that can influence the evolution of the illness and if its presence is a criteria to start antiepileptic medication. Propose therapeutic criteria to medicate and of observation. Method: Monocentric, longitudinal, cohort study with patients that consulted Pediatric hospital of Dr. Roberto Gilbert Elizalde during the period of three months. (November 2003 V January 2004) Criteria to be included in this study: seizures without fever that began between the ages of 3 and 13 years of age with a normal neurodevelopment, normal image study, neurological exam and electroencephalogram that shows BMEI. Patients were classified in two subgroups: with or without family or clinical history of seizures and clinical differences were compared using.Results: Of the 57 patients 52 people were our study group out of which 67% were men and 33% were woman. The average of age was 9.26 years old +/- 2.99. The 63% of the patients had seizures while they were sleeping. Only 48% of the cases showed discharges are the electroencephalographic reading. Important clinical or electroencephalographic differences did not exist between the two groups. (with or with out clinical or family history).