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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.

Introduction: The ketogenic diet is a non-pharmacological treatment that has been used as a therapeutic alternative for the management of refractory epilepsy since 1921. It is a diet, high in fats and low in carbohydrates, which is used to treat paediatric refractory epilepsy, and is effective in around 50% of the patients that begin to follow it. It is a nutrition plan that is imbalanced in terms of both macro- and micro-nutrients, which can give rise to nutritional deficits in energy, proteins, minerals and vitamins, as well as an excess of lipids. There is therefore a risk of it causing undesirable side effects both at the outset and in later stages of the treatment., Development: The most frequent adverse effects that may occur either in acute form or in later stages are described, and tools that can be used in their management and prevention during treatment are proposed for paediatricians and ketogenic diet teams., Conclusions: Some adverse effects are difficult to interpret and may manifest due to involvement at different levels of the organism, thereby arousing doubts as to whether they are caused by the diet, the antiepileptic medication or by the patient's own intercurrent complications. It is important to follow the study and evaluation frequency protocol in order to detect and prevent these effects, as well as to consult reference centres in order to evaluate the cost-benefit of continuing the treatment or not.

Aim: To describe a series of patients with drug resistant epilepsy treated with vagus nerve stimulation in a national pediatric hospital, evaluating efficacy, safety and tolerability., Patients and Methods: A retrospective analysis of 158 pediatric patients with epilepsy resistant to pharmacological and non pharmacological treatment including surgery that were treated with vagus nerve stimulation between 2001-2015. Patients with progressive encephalopathies, and congenital heart disease were excluded., Results: 158 patients (80 male) were included, with a mean age at implantation of 11.4 years and a mean age at evolution of epilepsy of 9.5 years. Time of follow-up: 1-15 years (median: 6.9 years). Patient's age at this time: 2-31 years (median: 14.1 years). Effectiveness: 66.5% of patients showed more or equal at 50% of seizure control at 24 months of implant. Just three patients showed severe side effects (1.8%). Minor side effects were seen in 26 patients (16.4%). Without side effects: 129 (81.8%)., Conclusion: Vagus nerve stimulation is an effective, tolerable and safe therapy in our pediatric series with refractory epilepsy.

Introduction: Epilepsy is a chronic disease that affects 0.5-1% of the population. One third of the patients become refractory to antiepileptic drugs. Among the non-pharmacological treatments available, the modified Atkins diet is an effective treatment used since 2003 as another alternative for children and adults with refractory epilepsy., Development: The Ketogenic Diet National Committee, which depends on the Argentine Society of Pediatric Neurology, elaborated this consensus on the modified Atkins diet, basing itself on a review of the literature and on their clinical experience. This consensus in Spanish explains the different aspects to be taken into account regarding the modified Atkins diet, patient selection, implementation, different controls and adverse effects. Unlike the classic ketogenic diet, the modified Atkins diet is initiated without fasting or hospital stay, nor does it require protein, calorie or fluid restriction, thus improving patient palatability and consequently patient tolerability., Conclusions: The modified Atkins diet is a useful treatment for patients with intractable epilepsy. The publication of this consensus offers the possibility for new centers to get oriented regarding this diet implementation.

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.

Aims: In this paper we describe the clinical characteristics, and particularly the epileptic seizures and electroencephalographic findings, in 15 patients with a pathology diagnosis of late infantile neuronal ceroid lipofuscinosis (NCL)., Patients and Methods: Nine female and six male patients were studied and their clinical records covering the period February 1990 to June 2003 were analysed. Neuroimaging, neurometabolic studies, ERG, PE and repeated EEG were carried out in all cases., Results: The mean age on onset of the disease was 3 years (range: 1-5 years). The initial symptom was epilepsy in all cases. Massive myoclonias and myoclonic-atonic seizures were the most frequent kinds of attacks. Focal myoclonias were observed in six patients. Other types of epileptic seizures observed included generalised tonic-clonic, absence, motor focal and complex focal. The epileptic seizures were resistant to therapy. Progressive neurological and visual impairment, pyramidal and cerebellar signs, as well as mental retardation were present in all cases. Intercritical EEG recordings showed diffuse paroxysms with spike and polyspike waves, multifocal spikes and, less often, focal spikes that were predominant in posterior regions. Photostimulation showed high amplitude (300-450) occipital spikes during the application of light stimulation between 1 and 8 Hz. ERG, VEP and SSEP results were pathological. Images showed signs of brain and cerebellar atrophy. Seven of the patients died between 8.5 and 11 years of age., Conclusions: Late infantile NCL must be considered in the case of a child aged between 1 and 5 years who presents seizures that are predominantly generalised myoclonias and myoclonic-atonic, in association with progressive neurological deterioration including pyramidal, cerebellar and visual signs and an EEG trace showing occipital paroxysms triggered by low frequency photostimulation.

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.

Objective: We described the electroclinical features, evolution and family history of two patients with definitive diagnosis of pyridoxine dependency., Case Reports: The first patient is a 15-month-old girl who at 1 month of age started with seizures and irritability. At two months of age, pyridoxine was prescribed with a good control of seizures. At five months of age withdrawal response provoked 7 days after seizures recurrence. Pyridoxine was reintroduced and seizures disappeared. Her sister, at two months of age, started with refractory seizures. This sister also had mental retardation and at four years, she died. Her brother, 16 years old, presents mental retardation, refractory epilepsy and progressive motor and cognitive impairment. At 3 months of age, he started with seizures and at 15 years of age, pyridoxine was prescribed with a significative improvement the number of seizures and a better visual connection. The second patient is a 4-month-old girl who started with clonic seizures at 3 days of age and she had a good response to pyridoxine. Withdrawal response provoked seizure recurrence at 48 hours. Pyridoxine was introduced immediately with total control of seizures. She had two cousins with seizures who died at 3 months and 3 years of age respectively., Conclusion: When dealing with an infant with refractory seizures which start in the first two years of life and without etiology, we should consider the diagnosis of pyridoxine dependency. Early diagnosis and treatment with pyridoxine is crucial to avoid high risk morbidity and mortality. All infants in the two first years of life with refractory seizures without etiology must be prescribed oral pyridoxine (50-200 mg per day).

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.

Aims: The purpose of this study is to report on 35 patients with Angelman syndrome (AS) in whom we evaluated the electroclinical characteristics and the progression of their epilepsy., Patients and Methods: The following factors were evaluated: sex, family background, neurological examination, age at onset and semiology of the epileptic seizures, EEG, types of epilepsy according to the international classification and response to therapy. We investigated the karyotype, and conducted FISH and methylation tests for AS., Results: The 35 patients had an average follow up time of 5.6 years. Epilepsy was diagnosed in 25 cases, with an average age of onset of 1.6 years. The epileptic syndromes were: epilepsy with myoclonic seizures in 13, of which seven presented a myoclonic state in their history, focal epilepsy in seven, West's syndrome in three, and Lennox Gastaut syndrome in two. Intercritical EEG showed generalised MSW and SW paroxysms in 13, unilateral spikes in seven, hypsarrhythmia in three, generalised fast rhythm paroxysms and slow SW activity in two. Basal electroencephalographic activity was: slow hypervoltage waves with or without inserted spikes situated at the rear in 19, at the front in six, diffuse in six, and normal in four cases., Conclusions: 71.4% of patients with AS suffered epileptic seizures; epilepsy with myoclonic seizures was the most frequently observed epileptic syndrome and hypervoltage slow wave activity with or without spikes inserted in the posterior quadrants was a characteristic encephalographic pattern. In patients with mental retardation, with or without epilepsy and these electroencephalographic findings, even in the absence of characteristic clinical signs, methylation and FISH analyses for AS should be performed.

Case Reports: We report a clinical and EEG study of 8 children with reflex myoclonic epilepsy of infancy to further confirm the existence of this syndrome first described by Ricci et al in 1995., Results: Between February 1990 to July 2002, we identified 64 epileptic patients with myoclonic seizures with an onset in the first six years of life. Eight (12.5%) of these patients had myoclonic seizure stimuli sensible. The seizures were characterized by generalized, myoclonic jerks triggered by tactile stimuli in six patients and acoustic stimuli in two, in one of them myoclonic jerks were triggered by both types of stimuli. The seizures appeared between 5 and 20 months of age. Two of the 8 patients had spontaneous myoclonic attacks during sleep. Interictal EEG was normal during wakefulness and occasional discharges were evident during sleep. In contrast, the ictal EEG during both wakefulness and sleep showed generalized spike wave and polyspike slow wave paroxysms. Neurologic examination, neuroimaging and neurometabolic studies were normal. Myoclonic jerks disappeared in 6 patients after valproic acid administration and in two after clobazan administration. Antiepileptic treatment was discontinued in 6 patients and no seizure recurrence was observed during a median follow up of 6 years., Conclusion: Our patients presented electro clinical criteria compatible with the syndrome of reflex myoclonic epilepsy of infancy. This syndrome could be considered to be a new reflex epileptic syndrome or a variant of benign myoclonic epilepsy in infancy.

Objectives: To stress that different atypical evolutions occur in the course of disease in some patients with the so called benign partial epilepsies of childhood (BPEC). To promote interest in finding clinical and/or electroencephalographic clues to recognize which patients might be prone to present these risks., Methods: Follow up of 26 pediatric patients who started with typical clinical and EEG features of benign childhood epilepsy with centro-temporal spikes (BCECTS) but presented reversible or persistent serious epileptic events including status epilepticus and language, cognitive or behavioral impairments. Repeated neurological examinations, EEG records and neuropsychological evaluations were done in the course of up to 14 years. Brain imaging studies (CAT and/or MRI) were obtained in all patients., Results: Four groups of patients were recognized: 11 children with atypical benign partial epilepsy of childhood (ABPEC) followed during 4 to 13 years. All have finally recovered and attend normal schools, although five have learning difficulties. Three patients met diagnostic criteria for Landau-Kleffner syndrome (LKS). Two of them recovered from aphasia and in one, some language difficulties persist. Seven children showed status epilepticus of BCECTS but all of them are now normal after 3 to 14 years of follow-up. Five children showed mixed features of the three groups mentioned above and three of them fulfilled the criteria for diagnosis of the syndrome of continuous spike-wave during slow sleep (CSWS)., Conclusions: A small proportion of cases starting with BCECTS evolve into either ABPEC, LKS, status of BCECTS or the syndrome of CSWS. Then, BCECTS is not always benign. Clinical and EEG markers should be sought to predict these atypical evolutions.

Objective: To evaluate clinical and EEG features, as well as treatment and progression in fifteen patients with a diagnosis of acquired epileptic aphasia., Patients and Methods: The population comprised nine male and six female patients, whose mean age was 14.5 (r = 8.11-20 years). All were on routine antiepileptic drugs. Inclusion criteria were acute, subacute or chronic aphasia, lacking signs of motor deficit or demonstrable brain lesion, but displaying bilateral spikes or generalized spike-wave discharges. Throughout, sleep and waking EEG, neuroimaging, brainstem auditory evoked potentials and neuro-psychological evaluations were performed. Cortical brainstem auditory evoked potentials were carried out in ten cases., Results: Median age at onset of verbal auditory agnosia was 5.6 years (r = 1.1-8.6 years), which eleven cases developed epileptic seizures at a median age of five years. Waking and sleep EEG were abnormal but brainstem auditory evoked potentials were normal throughout. Cortical brainstem auditory evoked potentials in ten patients displayed P300 wave and vertex potential alterations. Five cases received 1-3 mg/kg/day prednisone during 6-12 months, with almost complete speech recovery in four. At the last follow-up, language impairment was mild in five patients, moderate in five and severe in three, while two children recovered normal speech., Conclusions: Early acquired epileptic aphasia treatment is advisable with valproic acid, benzodiazepines or ethosuximide alone or in combinations, supplementary with corticoids for at least six months in the absence of clinical response and/or EEG improvement. Globally, seven out of fifteen patients overcame their speech disorder.

Introduction and Objective: West's syndrome (WS) is an epileptic encephalopathy of the first year of life, associated with different aetiologies. MRI of the brain allows precise determination of the type and extent of the lesions. The aetiology must be recognised in order to establish the prognosis and a suitable therapeutic approach. The objective of this study is to analyse the aetiologies of a population of children with WS and compare the results of cases diagnosed before and after using MRI., Patients and Methods: We analyzed the clinical histories of 448 patients fulfilling the diagnostic criteria for WS (infantile spasms and a EEG with a pattern of hypsarrhythmia), 217 in pre-MRI era (group 1) and 231 in the post-MRI era (group 2). The following parameters were analyzed: type of WS, sex, duration of follow-up, age of onset of infantile spasms and particularly the neuroradiological studies., Results: Group 1: symptomatic WS, 157 patients; cryptogenic WS, 60 patients. Group 2: symptomatic WS, 169 patients; cryptogenic WS, 62 patients. The aetiologies of symptomatic WS were: cortical dysplasias, neurocutaneous disorders, cerebral malformation and prenatal clastic lesions, hypoxic ischaemia, post-infection, metabolic, tumors, Down's syndrome, others and unknown cause., Conclusions: It is known that cerebral MRI gives better definition of these types of cerebral lesions than cerebral CT does. We emphasize the importance of MRI in patients with symptomatic WS for precise determination of the aetiology, and speculate as to whether some of the 21 cases of unknown aetiology of group 1 could have been diagnosed if studied nowadays.

Introduction: Loiseau and Orgogozo first described benign partial convulsions of adolescence (BPCA)., Objective: To analyze the clinical and electroencephalographic characteristics of adolescent patients diagnosed as having BPCA., Patients and Methods: We reviewed the clinical histories of 125 patients with onset of partial epilepsy between the ages of 10 and 18 years, followed-up at the J.P. Garrahan Hospital, Buenos Aires, between 1990 and 1997. Fourteen of these patients fulfilled diagnostic criteria for BPCA. We analyzed the following clinical and electroencephalographic parameters: sex, personal and family history, age of onset, semiology, distribution, duration and frequency of crises, neurological examination, neuro-images (brain CT/MR) using conventional techniques, time of follow-up and evolution., Results: Ten patients were boys (71.4%) and four girls (28.6%). The follow-up period was from one to seven years (average 3.1 years). Convulsions had started at between 10 and 16 years old, average 12 years old, with a peak of maximum incidence at between 12 and 13 years old. The crises were of partial simple type in 12 patients (85.7%) and partial complex type in 2 (14.3%); nine cases had secondarily generalized tonic-clonic convulsions (64.2%). Crises were brief in 100%, in 13 cases (93%) they occurred whilst awake and in one case (7%) during sleep., Conclusions: BPCA occur in a transient condition which affects males more often than females, has a peak onset at between 12 and 13 years of age and is characterized by simple motor partial crises, frequently with secondary generalization, single or inclusters, during waking and is of a benign course. Neurological examination, EEG between crises and neuro-radiological studies were normal.

Introduction: Rasmussen's encephalitis is a neurological disorder probably of immunological origin, characterized by intractable epilepsy, neurological deterioration and the presence of antibodies against R3 glutamate receptors., Objective: To present the experience of an Argentinian Paediatric Neurology Service during the past 7 years., Material and Methods: We analyzed the clinical histories of 12 patients with RS as defined by the diagnostic criteria proposed by Hart et al. We evaluated the following parameters: neurological examination, neuropsychological assessment, types of crises, age of onset, frequency, distribution, electroencephalogram, video EEG, evoked potentials, neuro-radiological studies; CT and cerebral MR using gadolinium, SPECT, lumbar puncture and cerebral biopsy., Results: The crises were of simple partial motor type in 12 cases, secondarily generalized partial crises in 7 cases, complex partial crises in 4 cases and partial and generalized motor states in 8 cases. RESULTS. The ictal EEGs during the continuous partial crises showed contralateral focal slow activity of the affected side of the body in 30% of cases. The neuro-radiological studies (CT and MR) showed signs of cerebral hemiatrophy in all 12 cases, hyper-intense images in the T2 sequences of the fronto-temporo-parietal regions in 5 cases, an asymmetric megacisterna magna in 1 case and hypoplasia of the cerebellar vermis in another case., Conclusions: The continuous partial crises showed little correlation with the electro-encephalography. Following administration of i.v. gammaglobulin there was a transient halt in progression of the neurological deterioration and control of the crises in 55% of the cases. Surgery continued to be the treatment recommended for control of the epileptic crises.

Objective: To evaluate the clinical, electro-encephalographic and evolutionary characteristics of a series of patients diagnosed as having benign neonatal sleep myoclonus (BNSM)., Material and Methods: The clinical histories of 21 patients with BNSM were analyzed. Criteria for inclusion in the study were: neonates who had had more than one episode of myoclonia during sleep and in whom neurological examination and psychomotor development were normal. CRITERIA FOR EXCLUSION: Patients with myoclonia while awake, a perinatal history which included significant pathology and/or the diagnosis of epilepsy. The period of evolution varied from 6 months to 5 years, and the following parameters were considered: clinical features of the myoclonia, neurological examination, psychomotor development and evolution. EEG were done between crises in all patients. In 5 cases we recorded EEG during crises and in 2 cases video-EEG were done., Results: The study group was made up of 11 girls and 10 boys. The myoclonia started between the first and twenty third day of age (average = 7 days). Fifteen (71.4%) of the patients had generalized myoclonia, mainly in the distal part of the upper limbs in 13 and in the lower limbs in two. In 20 cases (95.2%) jerking was of short duration, lasting 10 to 20 seconds. In one case, the jerks were repeated in series lasting 30 minutes. Two patients later developed benign myoclonia of early infancy. The myoclonia disappeared before the age of 7 months in all cases., Conclusions: BNSM is seen in healthy newborns and disappear spontaneously during the first months of live. Differential diagnosis with epileptic seizures is imperative in order to avoid unnecessary medication.

Introduction: It has been known that patients with symptomatic West Syndrome (WS) generally show poor responses to classic treatment, either steroids or antiepileptic drugs (AED). However new evidences arose in the last few years showing that particular association of WS with Down Syndrome (DS), type 1 Neurofibromatosis (NF1) and cerebral palsy (CP) with periventricular leukomalacia (PL) tend to behave as cryptogenic or idiopathic cases showing control of infantile spasms (IS) and disappearance of hypsarrhythmia after treatment. The special case of vigabatrin as treatment for IS in babies with Tuberous Sclerosis is also an example., Material and Methods: We studied 15 patients with WS and DS with a follow-up of 1.5 to 9 years, 5 patients with WS and NF1 followed-up form 2 to 6 years and 10 patients with WS associated with CP and PL followed-up during 2.5 to 12 years. Seizures frequency, EEG and responses to treatment were evaluated., Results: 1. WS and DS: IS had onset at a mean age of 6.5 months. No other type of seizures was registered. EEG showed typical hypsarrhythmia in 12 cases and asymmetric hypsarrhythmia 3. IS disappeared and EEG were normalized after 15 days of treatment in 10 patients and after 6 months in other 4 patients. Three patients presented myoclonic seizures and one motor partial seizures during follow-up, all of them responding to AED. 2. WS and NF1: Onset of IS took place between 4 and 9 months of age. EEG were hypsarrhythmic in all 5 patients. Four of them were seizure free after usual steroids treatment and showed no relapse during follow-up. 3. WS and CP associated with PL: These were 9 males and 1 female with IS starting at a mean age of 6.5 months, without history prior seizures. All EEG showed hypsarrhythmia. IS stopped and EEG became normal during the first month of treatment in 9 patients. In the other one the IS arrested with the use of vigabatrin 10 months after onset., Conclusions: Patients with these particular etiologies associated with WS do not behave as the usual symptomatic cases of WS in their response to classic treatment of IS. In fact the control of IS and normalization of EEG had been as good or better than in cases of cryptogenic WS. Care should them be taken not to generalized the usual concept about poor prognosis of WS in the so called symptomatic cases.

Introduction: Prospective study to evaluate efficacy of ketogenic diet (KD) in the treatment of children with intractable epilepsies (IE). Tolerability of the KD was also considered., Material and Methods: Criteria for inclusion were: 1. Epilepsy refractary to treatments with antiepileptic drugs (AED) in monotherapy and combining two or three AED. 2. Acknowledgment of blood levels of these drugs in therapeutic range. 3. Absence of liver or kidney disease, metabolic abnormalities, inborn errors of metabolism or other progressive encephalopathies. 4. Family supposedly in economic and psychologic conditions to accept the difficulties of strictly maintaining KD. We used a classic KD following the criteria suggested by the John Hopkins Pediatrics Epilepsy Center. Baseline neurological and physical examination, EEG, blood chemistry including lipid profile were obtained prior to initiative and during the KD. KD efficacy was measured as percent reduction of baseline seizures frequency, considering positive results as reductions of 50% or over. Acceptance of the diet and quality of life were specially considered. Eighteen patients with ages from 2 to 11 years were admitted. Ten of them were males. Diagnosis followed the last Classification of Epileptic Syndromes of the ILAE, and distribution was: Symptomatic partial epilepsies, 8 cases (one had West syndrome at age 5 months); cryptogenic partial epilepsies, 1 case; Lennox-Gastaut syndrome, 2 cases; severe myoclonic epilepsy of infancy, 6 cases and epilepsy with myoclonic astatic seizures 1 case., Results: Four patients were not able to achieve persistent ketosis either due to patient's rejection of KD or to parents non compliance. KD was kept for at least two months before considering failures. Five patients did not show significant improvement and KD was stopped. At present nine patients have been on KD from 6 to 24 months (average 16 months). Four of them showed a 75-100% reduction in seizures frequency and in three the reduction was of 50-75%., Conclusions: KD was fairly well tolerated by 14 of 18 children and their families. Fifty percent of the 14 patients complying KD showed significant improvement in seizure frequency and in quality of life. Due to the small number of patients and short follow-up, we can not speculate about results in relation to each epileptic syndrome, neither the risk of late complications.

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.

Objective: To evaluate the therapeutic response of West's syndrome (WS) associated with cerebral paralysis (CP) secondary to periventricular leucomalacia (PVL)., Material and Methods: We made a retrospective analysis of the clinical histories of 10 patients with SW and CP secondary to periventricular leucomalacia. We studied 10 patients, 9 boys and 1 girl with a current age of between 3 and 11 years, 8 premature newborn babies and two full term new born babies., Results: The infantile spasms (IS) started between the ages of 4-10 months (average age 8.3 months). In 100% of cases they came in runs. In one patient alone they were associated with partial motor crises. EEG showed typical hypsarrhythmia in 8 cases, asymmetrical hypsarrhythmia in 1 case and modified hypsarrhythmia in another case. Seven patients were given ACTH, associated with valproic acid in 4 cases and benzodiazepines in 2 cases. The remaining 3 patients were treated with valproate as the only drug. The IS disappeared and the EEG became normal within 14 days of the start of treatment in 9 patients who remain symptom-free after between 2 and 11.9 years follow-up. In one patient there was partial control of the spasms, and the clinical picture cleared up 10 months after starting treatment with vigabatrine. All 10 patients had spastic quadriplegia. All had mental retardation of between slight and serious degree. The cerebral CT and MR showed signs compatible with PVL and in 3 cases there was associated diffuse cerebral atrophy., Conclusions: We have found a good electro-clinical response in 10 patients with WS, CP and PVL. The results are in agreement with those of other authors.

Introduction: Partial benign epilepsy of childhood with occipital paroxysms (EPBI-O) described by Gastaut in 1982, has been recognized in the latest Classification of Epilepsy and Epileptic Syndromes as being a form of idiopathic partial epilepsy. In 1989 Panayiotopoulos described a different form of idiopathic occipital epilepsy., Objective: We analyzed the electro-clinical characteristics of EPBI-O in order to identify the different forms of idiopathic occipital epilepsy., Material and Methods: This prospective study included 74 patients who fulfilled the diagnostic criteria of EPBI-O. The patients were evaluated between January 1990 and June 1996 by means of clinical and electro-encephalic examinations during a follow-up period of between 6 months and 6.5 years., Results: We identified two groups. Group I: eighteen patients with visual crises and post-ictal migrainous episodes, with or without motor crises, predominantly when awake and with the onset of these crises at 8.7 years old. On EEG there were P-O occipital paroxysms which reacted to eyelid opening and group II: fifty-six patients with crises of vomiting followed by oculo-encephalic version, predominantly during sleep and with an average age of 4.9 years when these crises started to occur. On EEG there were occipital spikes with identical morphology to that of the benign epilepsy of childhood with spikes of Roland (EPBI-R). Two patients had associated crises of EPBI-O of Group II and EPBI-R with independent occipital and Roland spikes. These formed a third group., Conclusions: This study confirms the existence of two variantes of EPBI-O; the 'Gastaut' type and the 'Panayiotopoulos' type with a more benign and more frequent course.

Introduction: Magnetic resonance has permitted the recognition of cortical dysplasias in patients with congenital hemiparesia and epilepsy., Objective: To study the clinic-EEG characteristics and course of epilepsy in patients with congenital hemiparesia and unilateral polymicrogyria., Methods and Results: We analyzed the clinical histories of 11 patients seen between 1990 and 1996. We studied 6 girls and 5 boys aged between 5 and 13 years, with a follow-up period of from 1 to 6 years. The epilepsy began at between 1 and 6 years old with partial motor seizures. On EEG there were frontotemporal spikes in 9 cases, temporooccipital in 1 and parieto-occipital in another. All 11 patients had hemiparesia, with slight mental retardation in 9 patients and moderate mental retardation in 2. The CT/MR brain scan showed unilateral polymicrogyria. At between 2 and 8 years of age, all 11 patients developed subintrant atonic crises with a pseudo-ataxic gait, absences in 7 patients and myoclonia in 3. Awake EEG showed bilateral asymmetrical spikes. During sleep 7 patients had continuous spikewave discharges and 4 had frequent asymmetrical bilateral spikes. Four patients relapsed. Five patients are free of crises, five have sporadic crises and one continues to have daily crises., Conclusions: These patients had hemiparetic cerebral paralysis, slight mental retardation and epilepsy. At about the age of 6 a peculiar electro-clinical condition developed. Response to treatment was satisfactory, although the follow-up period is still not long.

Introduction: Benign Infantile Familial Convulsions (HBIFC), are characterized by brief partials seizures, occasionally with secondary generalization, with onset in the first year of life, family history of similar electroclinical seizures and same age of appearance., Material and Methods: We presented 16 patients, 10 girls and 6 boys, evaluated in our Service between 1990-1996. We analyzed, age of onset of the seizures, sex, family history of epilepsy, neurologic exam, semiology, distribution, frequency and duration of the seizures, EEG, neuroradiologic studies and evolution., Results: The patients had partial seizures, which occurred mainly in clusters, with onset ranged from 3 to 8 months, with normal neurological exam and psychomotor development. The interictal EEG was normal and the course was benign. Treatment response to antiepileptic drugs was good., Conclusions: Our presentation confirmed that BIFC are a new partial idiopathic epileptic syndrome, with a genetic predisposition, probably with an autosomal dominant inheritance, which would be recognize in the next international classification of epilepsy and epileptic syndromes.