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Aim: To determine clinical, electroencephalographic, therapeutic and evolutive characteristics of a series of oncopediatric patients with acute symptomatic seizures., Patients and Methods: We performed a retrospective and prospective descriptive analysis of clinical records of oncopediatric children evaluated by neurology at the comprehensive outpatient Center for Hemato-Oncological Patients during 2017-2021. We included children aged one month to 17 years with intracranial and extracranial tumors who presented with acute symptomatic seizure (ASC). We defined acute symptomatic seizure according to the 2010 International League Against Epilepsy. We classified seizures according to 2017 International League Against Epilepsy classification. We excluded any patient with a diagnosis of previous epilepsy and non-epileptic paroxysmal episodes., Results: We analyzed 44 cases with a median of 4 years (range: 1 month-17 years) and mean of 5.75 months (range: 1 month-11 months) and 8.33 years (2-17 years). The main etiologies were neurotoxicity and post-surgical context. Four patients presented dysnatremias and two associated with endocranial hypertension. Forty-one electroencephalograms were performed with intercritical results with abnormalities in the baseline rhythm, but without foci or paroxysms. There were no critical recordings. Focal seizures were 25 (56.8%) and generalized seizures 19 (43.18%). Levetiracetam was the most commonly used drug for acute management., Conclusions: Our cohort shows that ASC, in this population, do not show considerable differences between focal motor and generalized seizures and occur mostly in neurotoxic and post-surgical contexts. Dysnatremias and endocranial hypertension associated with ASC were also recorded. Postcrisis electroencephalograms were without foci or paroxysms and good seizure evolution.

Objective: The aim of this study was to assess efficacy, safety, and tolerability of highly purified cannabidiol oil (CBD) as add-on therapy for the treatment of a series of patients with infantile epileptic spasms syndrome (IESS) who were resistant to antiseizure medications and ketogenic dietary therapy., Material and Methods: We conducted a retrospective analysis of the medical records of 28 infants with treatment-resistant IESS aged 6 to 21 months who received highly purified CBD between July 2021 and June 2023. Data were collected on neurological examinations, EEG, Video-EEG and polygraphic recordings, imaging studies, laboratory testing, and seizure frequency, type, and duration, and adverse effects. As the primary outcome, a reduction of frequency of epileptic spasms (ES) was assessed. ES freedom was considered after a minimal time of 1 month without ES., Results: Sixteen male and 12 female patients, aged 6-21 months, who received CBD for treatment-resistant IESS were included. The etiology was structural in 10, Down syndrome in seven, genetic in nine, and unknown in two. Initial CBD dose was 2 mg/kg/day, which was uptitrated to a median dose of 25 mg/kg/day (range, 2-50). Prior to CBD initiation, patients had a median of 69 ES in clusters per day (range, 41-75) and of 10 focal seizures per week (range, 7-13). After a mean and median follow-up of 15 and 12.5 months (range, 6-26 months), seven patients were ES free and 12 had a >50 % ES reduction. Five of seven patients (71 %) with Down syndrome and 3/5 (60 %) with cerebral palsy responded well. Adverse effects were mild. EEG improvements correlated with ES reductions., Conclusion: In this study evaluating the use of CBD in children with IESS, 19/28 (67.8 %) had a more than 50 % ES reduction with good tolerability., Competing Interests: Declaration of competing interest The authors declare they have no conflict of interest regarding this study., (Copyright © 2024 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Dravet syndrome is currently considered as an developmental and epileptic encephalopathy and, recently, mandatory, alert, and exclusionary criteria have been proposed. Here, we describe three patients with Dravet syndrome with the typical early presentation including febrile and afebrile alternating hemiclonic seizures due to loss-of-function SCN1A variants. Subsequently, they developed episodes of febrile focal status epilepticus (SE) associated with hemiparesis and cerebral hemiatrophy with posterior focal seizures, as a consequence of Dravet syndrome. This sequence of events has been previously published in patients with Dravet syndrome and does not contradict the recent classification by the International League Against Epilepsy (ILAE). The ILAE guidance identifies "Focal neurological findings" as alert criteria and "MRI showing a causal focal lesion" as exclusionary criteria for making an initial diagnosis of Dravet syndrome at presentation. Our three patients would correspond to a severe phenotype, similar to the well-known presentation of generalized atrophy following prolonged status epilepticus. Common genetic findings in cases of diffuse and unilateral brain involvement may help explain these clinical presentations. Further genotype-phenotype studies may provide additional insights into this electroclinical behavior., (© 2023 International League Against Epilepsy.)

Objective: The study was conducted to analyze the possible diagnostic value of the electroclinical semiology of the epileptic seizures., Methods: We evaluated the medical records of 17 females and 5 males with CDKL5 deficiency disorder (CDD) considering the long-term evolution, including the polygraphic video-EEG recordings., Results: We recognized three disease phases. We found that the seizure semiology was already recognizable in the first phase of the syndrome. In the short-term evolution, all patients had focal motor and 12/21 hypermotor seizures. Both epileptic spasms and myoclonic seizures were already present in more than half of the cases in the first 2 months after onset. In the second phase, the intermediate period, the polymorphic pattern was maintained, but in eight patients the electroclinical pattern of epileptic encephalopathy with hypsarrhythmia appeared. In the long-term period, the seizure polymorphism continued but myoclonic and epileptic spasms diminished. Tonic seizures appeared in the last 2 phases. Progressively, with the aggravation of seizures and paroxysmal EEG abnormalities impairment of the neurocognitive status was observed. Severe behavioral disturbances were seen in eight and autistic-like features in 14., Conclusion: CDD is a true developmental and epileptic encephalopathy with a specific etiology characterized by the early appearance of epileptic seizures that quickly become polymorphic and drug resistant in infants that are most often female and already have neurological impairment. Polygraphic video-EEG recordings are important to recognize ictal events of the association of hypermotor seizures, epileptic spasms in clusters, and massive myoclonic jerks, already present at onset., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Introduction: Initiation of ketogenic diet therapies (KDT) for pediatric epilepsy is usually done on an inpatient basis and the diet is managed during clinical appointments following a protocol of visits and routine tests. Because of the 2019 coronavirus disease (COVID-19) pandemic and the associated lock-down measures, we switched from outpatient to telemedicine-based KDT initiation., Objective: To explore the feasibility, effectiveness, and safety of online KDT initiation and follow-up by comparing a group of children with drug-resistant epilepsy that was managed by telemedicine compared to a group that was treated on an outpatient basis., Materials and Methods: An observational study was conducted in two groups of patients with drug-resistant epilepsy who initiated KDT and were followed up with an online versus an outpatient modality by the interdisciplinary KDT team of Hospital Pediatria JP Garrahan in Buenos Aires, Argentina. Dietary compliance, ketosis, retention rate, adverse effects, number of contacts, and clinical outcome were evaluated at 1, 3, and 6 months on the diet., Results: Overall, 37 patients were included, of whom 18 started the KD by telemedicine and 19 on an outpatient basis. Minimum follow-up of the patients was 6 months. All patients received the classic ketogenic diet. No statistical differences between the two groups regarding efficacy and safety of the diet were found., Conclusions: Our results support the feasibility and safety of initiating and management of KDT by telemedicine. Patients and their families should be carefully selected in order to guarantee a good outcome., (Copyright © 2022. Published by Elsevier Ltd.)

Objective: We report our findings regarding effectiveness, safety, and tolerability of cannabidiol (CBD)-enriched medical cannabis as add-on therapy in children with drug-resistant epileptic encephalopathies (DEEs) after a median follow-up of 20 months., Methods: A prospective cohort study was conducted to assess effectiveness, safety, and tolerability of CBD-enriched medical cannabis oil added to standard antiseizure medications in children with drug-resistant DEE seen at a single center., Results: Between October 2018 and March 2020, 59 patients were enrolled. Mean age at enrollment was 10.5 years (range, 2-17 years). Median treatment duration was 20 months (range, 12-32). Median age at first seizure was 8 months (range, 1 day - 10 years). At the end of follow-up, 78% of the children had a ≥ 50% decrease in seizure frequency and 47.5% had a > 75% decrease. Seven patients (11.9%) were seizure free. The number of seizures was reduced from a median of 305/month to 90/month, amounting to a mean reduction of 57% and a median reduction of 71% (p < 0.0001). Adverse effects were mostly mild or moderate. CBD was discontinued in 17 patients (28.8%) due to lack of response to treatment, increased seizure frequency, intolerance to the drug, or poor compliance., Conclusion: In children with drug-resistant DEEs, long-term treatment of CBD-enriched medical cannabis as an adjuvant therapy to antiseizure therapy was found to be safe, well tolerated, and effective. Sustained reductions in seizure frequency and improvement of aspects of daily living were observed compared to our preliminary findings., (Copyright © 2022 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Objective: We studied cases with long-lasting epileptic spasms (ES) considered as a spasm status analyzing type of epilepsy, epileptic syndrome, etiology, treatment, and outcome in 21 patients., Methods: We evaluated the charts of 21 patients seen between June 2006 and July 2017 who met the electroclinical diagnostic criteria of a spasm status. The spasm status was defined as continuous ES lasting 30 min or longer., Results: The type of ES was mixed in nine patients, flexion in seven, and extension in five. Epileptic spasms were asymmetric in three patients and unilateral in two. They occurred on awakening in all patients, while during sleep they decreased in all and disappeared in three cases. The duration of the spasm status ranged from 40 min to 15 days according to the seizure diaries of patients and video-EEG recordings. Two well-defined subgroups of patients were recognized; the first included patients with West syndrome (WS) and the second other types of severe non-West epilepsy syndromes. The spasm status responded well to oral vigabatrin (VGB) in four patients, oral topiramate (TPM) in three, oral corticosteroids in one, and cannabidiol in another patient. A good response was observed with benzodiazepines in six patients, with phenytoin (PH) in two, and with phenobarbital (PB) in one. Adrenocorticotropic hormone (ACTH) was effective in one patient and the ketogenic diet in two. Prognosis depends on the etiology., Conclusion: In this study we identified patients with WS and other types of severe non-West epilepsy syndromes who had a particular type of long-lasting ES that, in spite of its long duration does not strictly meet the criteria of the International League against Epilepsy (ILAE) classification of status epilepticus, may be considered a spasm status., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: Ketogenic diet therapy (KDT) is a metabolic treatment with proven effectiveness for the treatment of drug-resistant epilepsy in children. Although previously not used in infants under 2 years of age, recent studies have shown KDT to be highly effective and well tolerated in infants with epilepsy, especially those with epileptic encephalopathies. Here, we describe the effectiveness and tolerability of the diet in infants up to 2 years of age., Material and Methods: A prospective study was conducted in a cohort of infants younger than 2 years of age with drug-resistant epilepsy who received the classic ketogenic diet using a specific protocol at a single center in Argentina., Results: 56 infants with treatment-refractory epilepsy were evaluated. The etiology was genetic in 21.4%, structural in 28.6%, unknown in 44.7%, and metabolic in 5.4%. At 3 months, a > 50% decrease in seizure frequency was observed in 35 patients (62.4%), of whom 11 (19.6%) became seizure free. At 6 months, 34 patients (60.7%) had a decrease in seizure frequency of > 50%, of whom 10 (17.8%) were seizure free. At the one-year follow-up, 27 patients (48.2%) had a > 50% decrease in seizure frequency, of whom six (10.7%) were seizure free. At two years, 14 patients (25%) had a > 50% seizure control, of whom four (7.1%) were seizure free. The most common early adverse effects were hypoglycemia and vomiting, while after 1 month and beyond metabolic acidosis, vomiting, and constipation more commonly found. A trend towards a higher rate of acute adverse events in infants younger than 1 year was observed., Conclusions: CKD showed to be a useful option in infants with treatment-resistant epilepsy. Adverse effects were common, but not a reason to discontinue the diet. Further studies are necessary to evaluate in which epilepsy syndromes and etiologies KDT is most effective., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objective: Here we present a series of patients with WS that were refractory to antiseizure medications and the ketogenic diet and who were treated with cannabidiol-enriched cannabis oil (CBD) as add-on therapy analyzing efficacy, safety, and tolerability., Material and Methods: Medical records of eight patients with WS treated with CBD at a ratio of cannabidiol:Δ-9-tetrahydrocannabinol (CBD:THC) of 25:1 seen between May 2020 and March 2021 were retrospectively analyzed. In all patients CBD was started as add-on therapy., Results: Eight patients (six female and two male) who received CBD for treatment-resistant WS were evaluated. Ages ranged from 16 to 22 months. The etiology was unknown in five and structural in three. Initial CBD dose was 2 mg/kg/day which was uptitrated to a median dose of 12 mg/kg/day (range, 2-25). Prior to CBD initiation, patients had a mean of 63 seizures per day (range, 31-79). After a follow-up of between 6 and 13 months, a 75-99% decrease in seizure frequency was observed in two patients, a 50-74% decrease in two, a less than 50% decrease in three, and no changes in seizure frequency were seen in the remaining patient. The index of EEG abnormalities improved between 20 and 80% in seven patients concurrently with the reduction in seizures. Adverse effects were mild and transient. Somnolence was observed in one patient, nausea and vomiting in one, and behavior disturbances and irritability in another patient., Conclusion: This study evaluating the use of cannabidiol-enriched cannabis oil in children with WS showed that four (50%) of eight had a more than 50% seizure reduction with good tolerability., (Copyright © 2021 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Purpose: The aim of this study was to assess parent satisfaction three years after the implementation of a pediatric epilepsy telemedicine program., Methods: The program was developed with support from the Ministry of Health through Hospital Nacional de Pediatría J.P. Garrahan. A secure internet connection was used for high-speed, high-definition video and audio. Synchronous face-to-face consultation between the patient and the neurologist was used. To evaluate the success of the program, at the end of the consultation the parent or caregiver of the patient was asked to complete a survey evaluating satisfaction with health care, cost, and privacy issues. The survey was administered by community health workers to the parents of patients that were in follow-up or first seen at the rural health posts during 2019., Results: Between January and December 2019, 116 consecutive parents of patients seen at the epilepsy telemedicine clinic filled out a semistructured questionnaire. Mean age of the patients was 8.5 years (range, 1-17 years); 85 % of the patients had a follow-up of more than 6 months. All the families felt supported by the team and were satisfied with the program in terms of epilepsy management. Less work- and schooltime lost, less travel time and costs, better access to medication, and more regular follow-up visits were reported. The parents considered they had received clear information about the epilepsy of their child., Conclusion: Telemedicine proves to be an important tool in the comprehensive management of people with epilepsy., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Despite the rapid increase of clinical and basic-science knowledge on ketogenic diet therapies over the past years, it has not always been easy to determine the adequate indications of this treatment. Over the nearly 100 years of use, from being a last resource in the therapeutic algorithm, the diet has become one of the four main treatments for patients with difficult-to-control epilepsy together with antiepileptic drugs, surgery, and vagus nerve stimulation. The use of the diet has also changed. The current paper will briefly discuss the history of the diet together with a review of the literature regarding its most important indications and how they have evolved. The concept of the importance of defining the type of seizure, type of syndrome, and etiology in the selection of patients and timing of diet initiation has been gaining importance. This paper explores how the indications of the diet changed together with the shifting focus of epilepsy teams towards its use in different types of epilepsy and epilepsy syndromes and according to etiologies and as an alternative option in refractory and superrefractory status epilepticus., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: To provide information on the effect of the coronavirus disease of 2019 (COVID-19) pandemic on people with epilepsy and provide consensus recommendations on how to provide the best possible care for people with epilepsy while avoiding visits to urgent care facilities and hospitalizations during the novel coronavirus pandemic., Methods: The authors developed consensus statements in 2 sections. The first was "How should we/clinicians modify our clinical care pathway for people with epilepsy during the COVID-19 pandemic?" The second was "What general advice should we give to people with epilepsy during this crisis? The authors individually scored statements on a scale of -10 (strongly disagree) to +10 (strongly agree). Five of 11 recommendations for physicians and 3/5 recommendations for individuals/families were rated by all the authors as 7 or above (strongly agree) on the first round of rating. Subsequently, a teleconference was held where statements for which there was a lack of strong consensus were revised., Results: After revision, all consensus recommendations received a score of 7 or above. The recommendations focus on administration of as much care as possible at home to keep people with epilepsy out of health care facilities, where they are likely to encounter COVID-19 (including strategies for rescue therapy), as well as minimization of risk of seizure exacerbation through adherence, and through ensuring a regular supply of medication. We also provide helpful links to additional helpful information for people with epilepsy and health providers., Conclusion: These recommendations may help health care professionals provide optimal care to people with epilepsy during the coronavirus pandemic., (© 2020 American Academy of Neurology.)

Purpose: The aim of this study was to analyze patients whose only manifestation of epilepsy were generalized tonic-clonic seizures (GTCS) during childhood and discuss its validity as separate syndrome of childhood., Methods: We included children with at least two unprovoked GTCS between 3 and 11 years of age, no other seizure types at diagnosis, normal psychomotor development and neurological examination, an EEG with normal background and paroxysms of generalized spikes and waves with a frequency 2.5 Hz or above, and an unknown cause for epilepsy. Only patients with a follow-up >2 years were included., Results: Over a 12-year period (2005-2017) 26 patients met the inclusion criteria of epilepsy with GTCS only. Mean age at onset was 5 years. The seizures occurred while awake in 16 patients, on awakening in two, and during sleep in eight patients. The duration of seizures was around 3 min. Generalized spike-and-wave discharges were observed in all patients when awake and during sleep in eight and 26 patients, respectively. Nineteen responded well to valproic acid or levetiracetam. Two patients who received clobazam initially did not respond well; however, a switch to valproic acid resulted in excellent seizure control. Antiepileptic treatment was discontinued in sixteen patients who remained seizure free over a period of 2-9 years of follow-up., Conclusion: Epilepsy with GTCS alone in childhood is a type of epilepsy; however, it may be considered as a well-defined epileptic syndrome. Patients responded well to valproic acid or levetiracetam., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Encephalopathy with continuous spike-waves during slow-wave sleep (CSWS) evolves over time, and three stages can be recognized: before the onset of CSWS, during CSWS, and after the CSWS period. Clinical seizures tend to remit spontaneously around puberty. This pattern is independent of the etiological lesion. The CSWS also disappears in all cases. Focal abnormalities instead, may persist for some time after the disappearance of CSWS. The disappearance of the clinical seizures and CSWS may be simultaneous or seizures may disappear before or after disappearance of the CSWS pattern on the EEG. Electroclinical parameters in the pre-CSWS period that have been proposed to predict a poor outcome are early-onset seizures, appearance of new seizures, and a significant increase in seizure frequency. From the electrical point of view, an increase in the frequency of the interictal EEG paroxysms while awake and during sleep and bilateral spike-and-wave paroxysms may also be predictive of a poor evolution in CSWS. When CSWS disappears, neurocognitive and behavioral status improve, but in most patients, residual moderate to severe neurocognitive impairments remain. In non-lesional epilepsy, cognitive recovery after cessation of the CSWS depends on the severity and duration of the initial regression. The duration of the CSWS seems to be the most important predictor of cognitive outcome. Early recognition and effective therapy to reduce the seizures and resolve the CSWS may be crucial to improve long-term prognosis. Cognitive recovery is observed in patients who respond well to AED treatment and outcome depends on the etiology.

The concept of epilepsy syndromes, introduced in 1989, was defined as "clusters of signs and symptoms customarily occurring together". Definition of epilepsy syndromes based on electro-clinical features facilitated clinical practice and, whenever possible, clinical research in homogeneous groups of patients with epilepsies. Progress in the fields of neuroimaging and genetics made it rapidly clear that, although crucial, the electro-clinical description of epilepsy syndromes was not sufficient to allow much needed development of targeted therapies and a better understanding of the underlying pathophysiological mechanisms of seizures. The 2017 ILAE position paper on Classification of the Epilepsies recognized that "as a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking". The concept of "epilepsy syndromes" evolved, incorporating issues related to aetiologies and comorbidities. A comprehensive update (and revision where necessary) of the EEG diagnostic criteria in the light of the 2017 revised terminology and concepts was deemed necessary. Part 2 covers the neonatal and paediatric syndromes in accordance with the age of onset. [Published with educational EEG plates at www.epilepticdisorders.com].

The concept of epilepsy syndromes, introduced in 1989, was defined as "clusters of signs and symptoms customarily occurring together". Definition of epilepsy syndromes based on electro-clinical features facilitated clinical practice and, whenever possible, clinical research in homogeneous groups of patients with epilepsies. Progress in the fields of neuroimaging and genetics made it rapidly clear that, although crucial, the electro-clinical description of epilepsy syndromes was not sufficient to allow much needed development of targeted therapies and a better understanding of the underlying pathophysiological mechanisms of seizures. The 2017 ILAE position paper on Classification of the Epilepsies recognized that "as a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking". The concept of "epilepsy syndromes" evolved, incorporating issues related to aetiologies and comorbidities. A comprehensive update (and revision where necessary) of the EEG diagnostic criteria in the light of the 2017 revised terminology and concepts was deemed necessary. The work was commissioned by the Neurophysiology Task Force of the ILAE Committee on the Diagnostic Methods. Diagnostic criteria and recording procedures were developed by group consensus, reached through an "informal", internal decision-making process. Each working group member was allocated a number of syndromes, and a standard structured template was used. International literature was extensively reviewed. We developed a simple diagnostic system that is applicable to all epilepsy syndromes which allows the physician (i) to rate the strength of EEG diagnosis (degree of diagnostic certainty) by weighting EEG findings in relation to the available clinical information or the specific clinical question, and ii) to suggest further EEG diagnostics where conclusive diagnostic evidence is lacking. We also propose a system of syndrome-specific recording protocols that, used with the relevant clinical presentation or specific clinical question, may maximize activation of epileptic discharges and ultimately help with standardization of EEG recording across departments, worldwide. Because recording methodology also depends on available resources, a two-tier system was developed to embrace clinical EEG services in resource-limited and industrialized countries. A clinical practice statement for each of the epilepsy syndromes discussed underscores the crucial role of the clinical information with regards to both the optimization of the EEG recording and mainly its meaningful interpretation. Part I covers Genetic (Idiopathic) generalized epilepsies and syndromes, Reflex epilepsies, structural and genetic focal (lobar) syndromes and Progressive Myoclonus Epilepsies [Published with educational EEG plates on www.epilepticdisorders.com].

Epilepsy is one of the most common and widespread neurological disorders affecting over 65 million people worldwide. Although estimates vary considerably, the annual incidence is considered to be almost 50 per 100,000 and prevalence around 700 per 100,000. It is thought, however, that more people are affected in low- and middle-income countries where resources to improve the care for people with epilepsy are limited. Of all people with epilepsy, around 80% live in resource-limited countries and up to 90% of these patients receive no treatment at all. National epilepsy programs to organize comprehensive care and cover educational, economic, and research aspects are necessary. A referral network will enable local healthcare workers to consult patients with more complex diseases and may ensure routine availability of inexpensive AEDs. Adequately identifying people with epilepsy and delivering cost-effective care in resource-limited countries is an important challenge for epileptologists and healthcare policy makers alike. Here we give an overview of the present situation and review the needs and the efforts currently being made in the field.

Introduction: Epilepsy is a chronic disease with onset in infancy affecting 0.5-1% of the population. One third of the patients is refractory to antiepileptic drugs and they pose a challenge for the health care team. The ketogenic diet is an effective, non-pharmacological, alternative treatment for the management of refractory epilepsy., Aims: There is a need to establish guidelines for the adequate and increased use of the ketogenic diet in Spanish-speaking countries. The National Committee on the Ketogenic Diet, consisting of paediatric neurologists, clinical nutritionists, and dietitians, of the Argentine Society of Child Neurology has developed this consensus statement to standardize the use of the ketogenic diet based on the literature and clinical experience., Development: Patient selection, pre-treatment family counseling, drug interactions, micronutrient supplementation, adverse effects, and discontinuation of the diet are discussed., Conclusions: The ketogenic diet is an effective treatment for children with refractory epilepsy. Education and collaboration of the patient and their family is essential. The patient should be managed by an experienced multidisciplinary team using a protocol. The formation of a national multidisciplinary team and the publication of this document provide possibilities for new centers to integrate the ketogenic diet into their treatment options.

Objective: In this study we report the clinical outcomes of hemispherectomy for epilepsy in pediatric patients with special emphasis on the epileptic syndromes and their etiologies., Material and Methods: We retrospectively studied 45 patients with medically refractory epilepsy with hemispheric lesions who underwent hemispherectomy at the "Hospital de Pediatría Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina between February 1990 and February 2010. Patients had been assessed using a standard protocol involving clinical, neuroradiological, neurophysiological, and neuropsychological teams., Results: Twenty-seven males and 18 females with a mean age of 8.5 years (range, 2 months to 18 years) who underwent epilepsy surgery for refractory epilepsy were assessed. The mean time of follow-up was 9.5 years (range, 1 to 16 years). The following epileptic syndromes were recognized: West syndrome in 15 patients (33.5%), Rasmussen syndrome in 13 (29%), focal symptomatic epilepsy in 8 (17.5%), startle epilepsy in 6 (13.5%), Lennox-Gastaut syndrome in 2 (5%), and continuous spikes and waves during slow sleep in 1 (2%). The surgical specimens revealed malformations of cortical development in 18 patients (40%), Rasmussen encephalitis in 13 (29%), porencephalic lesions in 10 (22%), gliosis in 2 (4.4%), tumor in 1 (2.2%), and Sturge-Weber syndrome in 1 (2.2%)., Conclusion: The outcome of hemispherectomy in pediatric patients is good for those with refractory epilepsies, such as West syndrome, Lennox-Gastaut syndrome, epileptic encephalopathy with continuous spikes and waves during slow sleep, and startle epilepsy arising from a hemispheric lesion associated with hemiplegia.

Purpose: In this Argentinean retrospective, collaborative, multicenter study, we examine the efficacy and tolerability of the ketogenic diet (KD) for different epilepsy syndromes., Materials and Methods: we evaluated the clinical records of 216 patients started on the KD between March 1, 1990 and December 31, 2010., Results: One hundred forty of the initial patients (65%) remained on the diet at the end of the study period. Twenty-nine patients (20.5%) became seizure free and 50 children (36%) had a 75-99% decrease in seizures. Thus, 56.5% of the patients had a seizure control of more than 75%. The best results were found in patients with epilepsy with myoclonic-astatic seizures, Lennox-Gastaut syndrome, and West syndrome. Good results were also found in patients with Dravet syndrome, in those with symptomatic focal epilepsy secondary to malformations of cortical development, and in patients with tuberous sclerosis. Seizures were significantly reduced in four patients with fever-induced refractory epileptic encephalopathy in school-age children and in two patients with epileptic encephalopathy with continuous spikes and waves during slow sleep. The median period of follow-up after discontinuation of the diet was 6 years. Twenty patients who had become seizure free discontinued the diet, but seizures recurred in five (25%). Of 40 patients with a seizure reduction of more than 50% who discontinued the diet, 10 presented with recurrent seizures., Conclusion: The ketogenic diet is a good option in the treatment of refractory epilepsy. After discontinuing the diet, seizures recurrence occurred in few patients., (Copyright © 2011 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Objectives: We analyzed electroclinical features and evolution in nine patients with hemiparetic cerebral palsy associated with SE., Patients and Methods: Nine patients with mean age 12.3 years and a follow up from 1 to 11 years were studied, analyzing etiology, neurological examination, psychometric evaluation, age at onset and semiology of epileptic seizures, EEGs and neuro-radiological findings, response to treatment and evolution., Results: Etiologies were: porencephaly in 4 cases, hypoxic-ischemic encephalopathy in 2, bacterial meningitis in 1, herpetic encephalitis in 1 and meningo-encephalitis in the last. All cases had mental retardation. Mean age at onset of epileptic seizures was 4 years. Mean age at onset of startle seizures was 6. The startle seizures were characterized by sudden tonic contractions of the paretic hemibody, provoked by auditory stimulus in 6, somatosensory in 2 and both types of stimulus in 1. Falls were observed in 6 patients. Seizures were daily and always when awake. Unprovoked focal seizures with or without secondary generalization were found in 8 cases, and in 6 they presented previously to the startle seizures. Interictal EEGs showed unilateral spikes in 3, bilateral spikes in 3 and generalized polyspike-wave paroxysms in the other three cases. Ictal EEGs were obtained in 8 of the 9 patients and showed diffuse paroxysms of rhythms 6-11 Hz. Cerebral CT scan and/or MRI revealed extensive unilateral encephalomalacia in 5 and porencephaly in 4. The different antiepileptic schedules were unsuccessful in all cases. Surgery was performed in two patients. They are free of seizures after 1 to 4 years of follow up., Conclusion: SE should be considered as a distinctive epileptic syndrome or a particular electro-clinical evolution in patients with a large unilateral brain lesion associated with provoked reflex seizures usually refractory to antiepileptic drugs. Epileptic surgery should be considered.

Introduction: We analyze the clinical, neurological, EEG, neuroradiological features and evolution of two patients with subacute measles encephalitis., Case Reports: The patients, aged five years and eleven months respectively showed an acute, progressive neurological compromise and deterioration of consciousness, epilepsia partialis continua and progressive damage on neuroimaging, with a history of measles in the first case and exposure to the virus in the second. The first patient had Hodgkin's disease and the other had a familial C4 deficit disorder. Fundoscopic examination showed lesions on the retina. The EEG showed unilateral slow waves and spikes. Brain CT and MRI revealed progressive cerebral atrophy and a unilateral corticosubcortical lesion. Measles antibodies in CSF were found in the first child and oligoclonal bands in the second. Our first patient died after three months and the second has a severe neurological damage., Conclusion: In immunocompromised patients with the exposure to a history of measles, acute neurological compromised and deterioration of consciousness, epilepsia partialis continua and progressive damage on neuroimaging, subacute measles encephalitis should be considered.

The syndrome of benign familial infantile convulsions (BFIC) is an autosomal dominant epileptic disorder that is characterized by convulsions, with onset at age 3-12 mo and a favorable outcome. BFIC had been linked to chromosome 19q, whereas the infantile convulsions and choreoathetosis (ICCA) syndrome, in which BFIC is associated with paroxysmal dyskinesias, had been linked to chromosome 16p12-q12. BFIC appears to be frequently associated with paroxysmal dyskinesias, because many additional families from diverse ethnic backgrounds have similar syndromes that have been linked to the chromosome 16 ICCA region. Moreover, one large pedigree with paroxysmal kinesigenic dyskinesias only, has also been linked to the same genomic area. This raised the possibility that families with pure BFIC may be linked to chromosome 16 as well. We identified and studied seven families with BFIC inherited as an autosomal dominant trait. Genotyping was performed with markers at chromosome 19q and 16p12-q12. Although chromosome 19q could be excluded, evidence for linkage in the ICCA region was found, with a maximum two-point LOD score of 3.32 for markers D16S3131 and SPN. This result proves that human chromosome 16p12-q12 is a major genetic locus underlying both BFIC and paroxysmal dyskinesias. The unusual phenotype displayed by one homozygous patient suggests that variability of the ICCA syndrome could be sustained by genetic modifiers.

From June 1988 to June 1998, 60 children with extratemporal epilepsies (EE), most of whom were symptomatic, underwent surgery. All patients were studied by means of CT scanning, MRI and scalp EEG. Video-telemetry was used in 40 cases. Intracranial electrodes were placed in 10. Intraoperative ECoG was used in the 35 children who underwent resective procedures and in the 25 in whom disconnection was performed. Surgical procedures were as follows: 24 lesionectomies, 25 disconnecting procedures, 7 polectomies and/or lobectomies, 3 corticectomies and 1 anatomical hemispherectomy. After at least 1 year's follow-up in 48 children, to date 38 are in Engel class I, 7 in class II, 1 in class III and 2 in class IV. That is to say, in 46 of the 48, surgical outcomes ranges from very good to at least worthwhile, as reflected in their classification in Engel class III.

Purpose: Polymicrogyria (PMG) is often associated with symptomatic focal epilepsy and neurologic dysfunction. We investigated the clinical and laboratory features of a group of children with congenital hemiparesis, unilateral polymicrogyria on magnetic resonance imaging (MRI), and a peculiar epileptic syndrome., Methods: Twelve patients (seven girls and five boys) with a mean age of 7.8 years (range, 5-13 years) were studied. All patients underwent clinical evaluation, computed tomography (CT) and MRI scanning, and neuropsychological assessment at initial examination. Patients were followed up from 1 to 7 years (mean, 4.5 years)., Results: Partial motor seizures with secondary generalization with onset between age 1 and 6 years (mean age, 2 years) were recorded in all patients. The course of epilepsy was similar in all patients with development of atypical absences, negative myoclonus, and gait difficulties. EEG recording demonstrated continuous spike-wave or bilateral abnormality throughout. Frequent relapses of the atonic and myoclonic seizures were seen in seven patients. However, during follow-up, seven patients were seizure free, and the others have not developed this particular seizure pattern. A single case underwent cortical resection 23 months ago and has had no seizures since then. Mental retardation was mild in nine and moderate in three patients., Conclusions: Children with unilateral polymicrogyria may develop a syndrome of negative myoclonus seizures that appears to be age specific and responsive to antiepileptic drug (AED) treatment. Despite limited follow-up time, a good outcome was observed in most cases.

Objective: An important number of epileptic patients who began with epileptic seizures during the first year of life have not just been well classified. The objective is to identify the different types of epilepsies and epileptic seizures which begin during the first year of life, according to the last classification of epilepsy and epileptic syndromes of 1989., Material and Methods: We have studied 471 patients who consulted to our service in the last 5 years, with epileptic seizures during the first year of life. We excluded neonatal seizures and febrile convulsions., Results: 1. Partial epilepsy: a) idiopathic: 12 (2.5%); b) symptomatic: 130 (28%); c) cryptogenic: 25 (5%). 2. Generalized epilepsy: a) idiopathic: benign myoclonic epilepsy in infancy, 6 (1%); b) cryptogenic-symptomatic: symptomatic West syndrome, 155 (33%); cryptogenic West syndrome, 65 (14%); cryptogenic myoclonic epilepsy, 6 (1%); early infantile epileptic encephalopathy, 8 (1.6%); early myoclonic encephalopathy, 4 (0.8%); other symptomatic generalized epilepsies without specific aetiology, 14 (3%). 3. Epilepsies and syndromes undetermined as to whether they are focal or generalized: a) severe myoclonic epilepsy in infancy, 15 (3%); symptomatic epilepsy with multifocal and independent spikes, 3 (0.6%); other undetermined epilepsies not defined above, 3 (0.6%). 4. Special syndromes: occasional convulsions, 15 (3%)., Conclusions: West syndrome is the commonest type of epilepsy during the first year of life, particularly symptomatic West syndrome. Symptomatic partial epilepsies are in the second place in frequency. We identified 12 patients with idiopathic partial epilepsy, who full-filled clinical and EEG characteristics of a recently described epilepsy: benign infantile familial convulsions. The study showed the predominance of symptomatic epilepsy, generalized (represented by West syndrome) and partials. Finally 6% of the patients have a generalized epilepsy, cryptogenic or symptomatic, not defined in the classification.

A study of specific neuropsychologic, neurolinguistic, and behavioral features of acquired epileptic aphasia or Landau-Kleffner syndrome was conducted in a group of 12 patients followed-up for 2-15 years (mean: 8 yr). Seventy-five percent had exhibited some language disturbance prior to acquired epileptic aphasia. Even when 9 patients had normal electroencephalographic findings in the long-term course of the disease, only 3 achieved normal language. No patient with persisting electroencephalographic abnormalities recovered normal or near normal language. The need to perform detailed neurolinguistic and neuropsychologic evaluations in the work-up and follow-up of children with acquired epileptic aphasia is stressed. An adapted neuropsychologic profile battery proved to be practical and objective for the follow-up of these patients.