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We aimed to investigate the role of interleukin-1 beta (IL-1β) in the mechanisms underlying mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE+HS). We assessed a cohort of 194 patients with MTLE+HS and 199 healthy controls. Patients were divided into those with positive and negative antecedent febrile seizures (FS). We used a multidimensional approach, including (i) genetic association with single nucleotide polymorphisms (SNPs) in the IL1B gene; (ii) quantification of the IL1B transcript in the hippocampal tissue of patients with refractory seizures; and (iii) quantification of the IL-1β protein in the plasma. We found a genetic association signal for two SNPs, rs2708928 and rs3730364*C in the IL1B gene, regardless of the presence of FS (adjusted p = 9.62e–11 and 5.14e–07, respectively). We found no difference between IL1B transcript levels when comparing sclerotic hippocampal tissue from patients with MTLE+HS, without FS, and hippocampi from autopsy controls (p > 0.05). Nevertheless, we found increased IL-1β in the plasma of patients with MTLE+HS with FS compared with controls (p = 0.0195). Our results support the hypothesis of a genetic association between MTLE+HS and the IL1B gene

Abstract Background Focal cortical dysplasias (FCDs) are malformations of cortical development that present cortical dyslamination and abnormal cell morphology and are frequently associated with refractory epilepsy. FCD type IIb presents dysmorphic neurons (DNs) and balloon cells (BCs), which are the hallmarks of this dysplasia. Moreover, hypertrophic neurons (HyNs) may be present in FCD types I, II and III. The objective of this study was to perform a detailed morphology and immunophenotype study of BCs, DNs, and HyNs in a cohort of FCD IIb patients. Methods Cortices resected as a treatment for refractory epilepsy from 18 cases of FCD type IIb were analysed using Bielschowsky method and haematoxylin and eosin as routine stains. Immunophenotype was performed using specific antibodies to detect epitopes differentially expressed by abnormal cells. Results All cases showed cortical dyslamination, BCs, DNs, and HyNs. No cell layer or column could be identified, except for cortical layer I. Lesions predominated in the frontal cortex (11 cases). DNs were large neurons and presented a clumped and or displaced Nissl substance towards the cell membrane, and a cytoplasm accumulation of neurofilament that displaced the nucleus to the cell periphery, as shown by Bielschowsky staining and immunohistochemistry. HyNs were as large as DNs, but without alterations of Nissl substance or dense neurofilament accumulation, with a central nucleus. BCs were identified as large, oval-shaped and pale eosinophilic cells, which lacked the Nissl substance, and presented an eccentric nucleus. BCs and DNs expressed epitopes of both undifferentiated and mature cells, detected using antibodies against nestin, vimentin, class III β-tubulin, pan-neuronal filaments, neurofilament proteins, β-tubulin and NeuN. Only BCs expressed GFAP. Conclusion FCDs present with disorganization of the cerebral cortex architecture, abnormal cell morphology, are frequently associated with refractory epilepsy, and their post-surgical prognosis depends on the type of FCD. The diagnosis of focal cortical dysplasia in a surgical specimen relies on the identification of the abnormal cells present in a dysplastic cortex specimen. The current report contributes to the identification of balloon cells, dysmorphic and hypertrophic neurons in the context of focal cortical dysplasia type IIb.

ObjectiveTo evaluate clinical data, electroencephalogram, etiology, classification, treatment, morbidity, and mortality in acute refractory status epilepticus.MethodsFifteen patients, mean age of 41.3 years-old, six males, with refractory status epilepticus, were retrospectively studied. All of them were followed by serial electroencephalogram or continuous electroencephalographic monitoring.ResultsThe most common comorbidity was hypertension. Seven (46.7%) patients were diagnosed with previous symptomatic focal epilepsy. More than one etiology was identified in 40.0% of the cases. Status epilepticus partial complex was the most common (n=14, 93.3%), and discrete seizures were the most observed initial ictal pattern. Continuous intravenous midazolam was used in nine (60.0%) patients and continuous thiopental in three (20.0%). Nine (60.0%) participants died, one (6.6%) had neurological sequelae, and five (33.3%) presented no neurological sequelae.ConclusionsHigher mortality rate was associated with advanced age and periodic lateralized epileptiform discharges. Midazolam proved to be a safe drug. The refractory status epilepticus is related to high mortality.ObjetivoAvaliar os dados clínicos, o eletroencefalograma, a etiologia, a classificação, o tratamento, a morbidade e a mortalidade do estado de mal epiléptico.MétodosQuinze pacientes, idade média de 41,3 anos, seis masculinos, foram avaliados retrospectivamente. Todos eles foram acompanhados por eletroencefalogramas seriados ou monitoração eletrencefalográfica contínua.ResultadosA comorbidade mais comum foi hipertensão arterial. Sete (46,7%) pacientes tinham epilepsia focal sintomática prévia. Mais de uma etiologia foi identificada em 40,0% dos casos. O estado de mal epiléptico parcial complexo foi o mais frequente (n=14; 93,3%) e discrete seizures foram os padrões ictal inicial mais observados. Midazolam contínuo foi usado em nove (60,0%) pacientes e tiopental contínuo em três (20,0%). Nove (60,0%) participantes morreram, um (6,6%) teve sequelas neurológicas e cinco (33,3%) não apresentaram sequelas.ConclusõesAlta taxa de mortalidade foi associada com idade avançada e com a presença de descargas periódicas epileptiformes lateralizadas. Midazolam provou ser uma droga segura. Estado de mal epiléptico refratário está associado à alta mortalidade.

Objective To evaluate clinical data, electroencephalogram, etiology, classification, treatment, morbidity, and mortality in acute refractory status epilepticus. Methods Fifteen patients, mean age of 41.3 years-old, six males, with refractory status epilepticus, were retrospectively studied. All of them were followed by serial electroencephalogram or continuous electroencephalographic monitoring. Results The most common comorbidity was hypertension. Seven (46.7%) patients were diagnosed with previous symptomatic focal epilepsy. More than one etiology was identified in 40.0% of the cases. Status epilepticus partial complex was the most common (n=14, 93.3%), and discrete seizures were the most observed initial ictal pattern. Continuous intravenous midazolam was used in nine (60.0%) patients and continuous thiopental in three (20.0%). Nine (60.0%) participants died, one (6.6%) had neurological sequelae, and five (33.3%) presented no neurological sequelae. Conclusions Higher mortality rate was associated with advanced age and periodic lateralized epileptiform discharges. Midazolam proved to be a safe drug. The refractory status epilepticus is related to high mortality.

OBJECTIVE: Nonconvulsive status epilepticus (NCSE) is currently considered as one of the most frequent types of status epilepticus (SE). The objective of the present study was to identify the natural history of the electrographical evolution of refractory NCSE and to establish the relationship between ictal patterns and prognosis. METHODS: We analyzed, retrospectively, 14 patients with loss of consciousness and NCSE. The ictal patterns were classified as discrete seizures (DS), merging seizures (MS), continuous ictal discharges (CID), continuous ictal discharges with flat periods (CID-F), and periodic lateralized epileptiform discharges (PLEDs). RESULTS: The ictal patterns were DS (n=7; 50.0%), PLEDs (n=3; 1.4%), CID (n=2; 14.3%), MS (n=1; 7.1%), and CID-F (n=1; 7.1%). CONCLUSIONS: NCSE electrographic findings are heterogeneous and do not follow a stereotyped sequence. PLEDs were related to a higher probability of neurological morbidity and mortality.

Epilepsy is the main neurological condition in children and adolescents. Unfortunately patients with medical refractory epilepsy are more susceptible for clinical complications and death. We report a prospectively evaluated cohort of children followed for approximately 10 years. Fifty-three of 1012 patients died. Forty-two patients died due to epilepsy or its clinical complications and the main causes of death were pneumonia (in 16 cases), sepses (in 9 patients), status epilepticus (in 8 patients). In 11 patients cause of death was sudden unexpected death in epilepsy (SUDEP). Mental retardation was significantly more frequent in patients who did not die from SUDEP. SUDEP may be a significant condition associated with mortality in children and adolescents with epilepsy.

Epilepsy is the most common neurological disorder in humans. People with epilepsy are more likely to die prematurely than those without epilepsy, with the most common epilepsy-related category of death being sudden unexpected death in epilepsy (SUDEP). The central mechanisms underlying the fatal process remain unclear, but cardiac and respiratory mechanisms appear to be involved. Recently, cerebellar, thalamic, basal ganglia and limbic brain structures have been shown to be implicated in respiratory and cardiac rate regulation. We discuss here the potential mechanisms underlying the fatal process, with a description of cerebellar actions likely failing in that SUDEP process.

OBJETIVO: Comparar o quociente intelectual (QI) em três grupos de crianças com epilepsia: 1) controlados com medicação, 2) não controlados com medicação e 3) controlados com cirurgia. MÉTODOS: Noventa e oito pacientes pediátricos, com idades entre 6 e 12 anos, foram selecionados de dezembro de 2007 a julho de 2008. A Escala de Inteligência Wechsler para Crianças - terceira edição (WISC-III) foi utilizada para a avaliação neuropsicológica dos pacientes. Os resultados foram relacionados com a síndrome epiléptica, a etiologia da epilepsia, o tratamento medicamentoso, a idade do paciente no início da epilepsia e a duração da epilepsia. RESULTADOS: Os escores da WISC foram significativamente melhores no grupo controlado com medicação quando comparados aos do grupo não controlado com medicação. O grupo controlado com medicação obteve um desempenho significativamente melhor na maioria dos subtestes da WISC quando comparado ao grupo não controlado com medicação: vocabulário, aritmética, compreensão, memória de dígitos, completar figuras, arranjo de figuras e cubos. Um número significativamente maior de pacientes com epilepsia idiopática e uso de monoterapia foi observado no grupo controlado com medicação quando comparado ao grupo não controlado. O grupo controlado com cirurgia não apresentou diferença significativa no desempenho do QI quando comparado ao grupo controlado com medicação. CONCLUSÕES: As crianças com um bom controle de crises tiveram um melhor desempenho no QI geral, verbal e de execução quando comparadas às crianças com epilepsia refratária. Esses resultados podem ser influenciados por fatores clínicos como o uso de monoterapia, o tipo de droga antiepiléptica utilizada, a síndrome epiléptica e a etiologia da epilepsia. A cirurgia de epilepsia pode causar um impacto positivo no desempenho cognitivo das crianças que ficaram livres de crises após o procedimento cirúrgico.OBJECTIVE: To compare the intellectual coefficient (IQ) of three groups of children with epilepsy: 1) medically controlled, 2) medically uncontrolled and 3) surgically controlled. METHODS: From December 2007 until July 2008, 98 pediatric patients were selected, with an age range between 6 and 12 years. Neuropsychological assessment included the Wechsler Intelligence Scale for Children - third edition (WISC-III). Results are related to epileptic syndrome, etiology of epilepsy, drug therapy, age at epilepsy onset and epilepsy duration. RESULTS: WISC scores were significantly better in the medically controlled group when compared to the medically uncontrolled group. The medically controlled group performed significantly better in the majority of the WISC subtests when compared to the medically uncontrolled group: vocabulary, arithmetic, comprehension, digit span, picture completion, picture arrangement, and block design. A significantly higher number of idiopathic epilepsy and monotherapy cases was observed in the medically controlled group when compared to the medically uncontrolled group. Surgically controlled children had no significant differences in IQ performance when compared to medically controlled children. CONCLUSIONS: Children with good seizure control have higher general, verbal and performed intelligence when compared to children with refractory epilepsy. These results may be influenced by clinical factors such as use of monotherapy, drug type and epileptic syndrome and etiology. Epilepsy surgery can have a positive impact on cognitive performance of children who were free of seizures after surgery.

The aim was to determine if patients with writer' cramp (WC) have abnormalities in kinesthetic motor imagery of hand movements. We timed the execution and simulation of a "finger tap task" and a "writing task" in 9 patients with simple WC and 9 matched healthy controls. In the "finger tap task, patients tended to be slower than controls to execute without vision (p=0.190) and to simulate the movements (p=0.094). In the "writing task", patients were slower than controls to execute writing with vision (p=0.0001) and without vision of the movements (p=0.0001) and to mentally simulate it (p=0.04). Patients were slower to execute writing than to simulate it (p=0.021) In general, there were not significant correlations between times of execution and simulation of both tasks. In conclusion, patients with WC seem to have slowing in the processes of mental simulation of hand movements that is not specific for writing.O objetivo do estudo foi determinar se pacientes com cãibra do escrivão (CE) teriam anormalidades na imagem motora de movimentos manuais. Foi cronometrado o tempo gasto para a execução e simulação de uma tarefa de "batida dos dedos" e outra de "escrita" em 9 pacientes com CE simples e 9 controles pareados. Na tarefa de "batida dos dedos" os pacientes apresentaram tendência a serem mais lentos que os controles para executá-la com visão dos movimentos (p=0,190) e para simulá-la (p=0,094). Na tarefa de "escrita", os pacientes foram mais lentos que os controles para executá-la com visão (p=0.0001) e sem visão dos movimentos (p=0,0001) e também para simulá-la (p=0,04). Os pacientes foram mais lentos para escrever que para simular a escrita (p=0,021). Não encontramos correlação entre os tempos de execução e simulação das tarefas. Pacientes com CE apresentam alentecimento no processo de simulação mental de movimentos manuais.

OBJECTIVE: Early brain insults can cause cavitary lesions including porencephaly (POR) and multicystic encephalopathy (MCE). The objective of this study was to investigate clinical and electrographic correlates associated to these types of destructive brain lesions. METHOD: Patients with POR and MCE were selected and submitted to clinical and Video-EEG monitoring. The following variables were analyzed: demographic data, type of lesion, presence of gliosis, perinatal complications, epilepsy, brain atrophy, and presence and frequency of epileptiform discharges. RESULTS: Twenty patients were included, 65% males, 35% females, ages ranging from 1 to 40 years, 14 with MCE and 6 with POR. Eighteen patients had hemiparesis, 19 had epilepsy (current or in the past), seven of them had refractory seizures, and 16 had epileptiform discharges. All patients with MCE had gliosis while only 2 with POR had it. CONCLUSIONS: No correlation was observed between type of lesion and clinical and electrographical outcome. However, a positive correlation was observed between frequency of discharges and presence of brain atrophy, and between MCE and gliosis.OBJETIVO: Insultos cerebrais precoces podem causar lesões cavitárias incluindo porencefalias (POR) e encefalomalacias multicisticas (EMC). O objetivo deste estudo foi investigar correlatos clínicos e eletrográficos associados a estes dois tipos de lesões destrutivas. MÉTODO: Pacientes com POR e EMC foram selecionados e submetidos à avaliação neurológica e monitorização vídeo-eletrencefalográfica, analisando-se as seguintes variáveis: dados demográficos, tipo de lesão, presença de gliose, complicações perinatais, epilepsia, atrofia cerebral, presença e freqüência de descargas epilépticas. RESULTADO: Vinte pacientes foram incluídos, sendo 65% do sexo masculino, 35% do feminino, idades entre 1 e 40 anos, sendo 14 com EMC e 6 com POR. Dezoito pacientes tinham hemiparesia, 19 tinham ou tiveram epilepsia (7 deles refratários ao tratamento medicamentoso) e 16 deles tinham paroxismos epileptiformes. Todos com MCE tinham gliose associada, contra apenas 2 dos pacientes com POR. CONCLUSÃO: Não houve correlação entre tipo de lesão e evolução clínica e eletrográfica. Houve, entretando, correlação positiva entre freqüência de descargas epilépticas e presença de atrofia cerebral, e entre lesão do tipo EMC e presença de gliose.

OBJECTIVE: The aim of this study was to evaluate the efficacy and tolerability of topiramate (TPM) in juvenile myoclonic epilepsy (JME). METHOD: We assessed seizure control and adverse effects of TPM in 22 patients (18 females) aged 13 to 53 years. Target TPM dosage was up to 200 mg/day. The patients were subdivided into 3 groups: those treated with seizure control plus side effects (n=4); treated with non-controlled seizures (n=15) and with JME newly diagnosed (n=3). RESULTS: Sixteen patients completed the first year of the follow-up. Generalized tonic-clonic seizures were completely controlled in 10 (62.5%); more than 50% of reduction in 4 (25.0%) and less than 50% in 2 (12.5%). Myoclonia were controlled in 11 (68.8%) and persisted in 5 (31.2%) patients. Absence seizures were present in 5 (22.7%) of whom 2 (9.0%) showed more than 50% of seizure reduction while 3 (13.6%) presented worsening. Discontinuations were due to inadequate seizure control and adverse events (N=4), low compliance and loss of follow-up (N=2) and subject choice (N=1). CONCLUSION: TPM showed to be an effective and well-tolerated drug in the treatment of JME. Although frequently observed, TPM side effects were tolerable and the drug could be maintained in the majority of patients.OBJETIVO: Avaliar a eficácia e tolerabilidade do topiramato (TPM) na epilepsia mioclônica juvenil (EMJ). MÉTODO: Avaliamos a resposta terapêutica e efeitos colaterais do TPM em 22 pacientes (18 mulheres) com idades entre 13 e 53 anos. A dose alvo utilizada foi até 200 mg/dia. Os pacientes foram divididos em 3 grupos no início do tratamento: aqueles com controle das crises mas que apresentavam efeitos colaterais (n=4); com crises não controladas (n=15) e com EMJ recém diagnosticada (n=3). RESULTADOS: Dezesseis pacientes completaram o primeiro ano de acompanhamento. Crises tônico-clonicas generalizadas foram completamente controladas em 10 (62,5%), tiveram redução maior de 50% em 4 (25,0%) e menor de 50% em 2 (12,5%). Mioclonias foram controladas em 11 (68,8%) e persistiram em 5 (31.2%) pacientes. As crises de ausências, presentes em 5 (22,7%) pacientes, tiveram redução maior do que 50% em 2 (9,0%) e agravamento em 3 (13,6%). A retirada do estudo foi devida principalmente ao controle inadequado das crises e efeitos colaterais indesejáveis (n=4), pouca adesão e perda do seguimento (n=2) e escolha do paciente (n=1). CONCLUSÃO: TPM foi considerada droga eficaz e bem tolerada no tratamento da EMJ. Apesar de freqüentemente observados, os efeitos colaterais do TPM foram toleráveis e a medicação pode ser mantida na maioria dos pacientes.

Seizures in Juvenile Myoclonic Epilepsy (JME) are dependent on the sleep-wake cycle and precipitant factors, among which sleep deprivation (SD) is one of the most important. Still an under diagnosed syndrome, misinterpretation of the EEGs contributes to diagnostic delay. Despite this, a quantitative EEG investigation of SD effects has not been performed. We investigated the effect of SD on EEGs in 41 patients, aged 16-50 yr. (mean 25.4), who had not yet had syndromic diagnosis after a mean delay of 8.2 yr. Two EEG recordings separated by a 48-hour interval were taken at 7 a.m. preceded by a period of 6 hours of sleep (routine EEG) and after SD (sleep-deprived EEG). The same protocol was followed and included a rest wakefulness recording, photic stimulation, hyperventilation and a post-hyperventilation period. The EEGs were analyzed as to the effect of SD on the number, duration, morphology, localization and predominance of abnormalities in the different stages. A discharge index (DI) was calculated. Out of the 41 patients, 4 presented both normal EEG recordings. In 37 (90.2%) there were epileptiform discharges (ED). The number of patients with ED ascended from 26 (70.3%) in the routine EEG to 32 (86.5%) in the sleep-deprived exam. The presence of generalized spike-wave and multispike-wave increased from 20 (54.1%) and 13 (35.1%) in the first EEG to 29 (78.4%) and 19 (51.4%) in the second, respectively (pNa epilepsia mioclônica juvenil (EMJ), uma síndrome epiléptica ainda subdiagnosticada, as crises são dependentes do ciclo vigília-sono e de fatores precipitantes, entre os quais a privação de sono (PS) é um dos mais importantes. A interpretação inadequada dos EEGs contribui para atraso no diagnóstico. Ainda não foi realizada investigação quantitativa sobre os efeitos da PS. Avaliamos o efeito da PS nos EEGs de 41 pacientes entre 16 e 50 anos (média 25,4) com EMJ em dois registros eletrencefalográficos, separados por intervalo de 48 horas. Os exames foram realizados às 7 horas da manhã, precedidos por um período de 6 horas de sono (EEG de rotina) e após PS (EEG com PS). Seguimos o mesmo protocolo que incluiu o registro em vigília em repouso, fotostimulação, hiperventilação e pós hiperventilação. O efeito da PS foi analisado sobre o número, duração, morfologia, localização e predominância das anormalidades nos diferentes estágios. Calculamos o índice de descargas por minuto. Dos 41 pacientes, 4 tiveram ambos os registros normais. Em 37 (90,2%) houve algumas descargas epileptiformes (DE). O número de pacientes com DE ascendeu de 26 (70,3%) no EEG de rotina para 32 (86,5%) no exame em PS. A presença de descargas de espícula-onda generalizadas e multispícula-onda aumentou de 20 (54,1%) e 13 (35,1%) no primeiro EEG para 29 (78,4%) e 19 (51,4%) no segundo, respectivamente (p

It is clear that sudden unexpected death in epilepsy (SUDEP) is mainly a problem for people with refractory epilepsy, but our understanding of the best way to its prevention is still incomplete. Although the pharmacological treatments available for epilepsies have expanded, some antiepileptic drugs are still limited in clinical efficacy. In the present paper, we described an experience with vagus nerve stimulation (VNS) treatment by opening space and providing the opportunity to implement effective preventative maps to reduce the incidence of SUDEP in children and adolescents with refractory epilepsy.

Febrile seizures (FS) affect almost 2-5% of children and factors related to an increase susceptibility of children to FS may involve an imbalance of inflammatory cytokines and genetic factors. FS had low morbidity, but may be associated with the occurrence of late chronic epilepsy. Here we describe factors related to FS and its possible correlation with SUDEP.

Functional MRI produces a more accurate localization of the language areas for epilepsy surgery purpose, but requires the patient cooperation. We report a 34 years-old woman with mental retardation who underwent two different verbal fluency tasks, category and word naming. We found a strong activation of the Broca’s area in the most difficult task. We suggest that a multi-task fMRI study could be successful in patients with cognitive delay.A ressonância magnética funcional permite uma localização acurada da área da linguagem para fins de cirurgia de epilepsia, mas requer a colaboração do paciente na execução da tarefa proposta, o que pode limitar a sua utilização em pacientes com retardo mental. Nós apresentamos o caso de uma mulher com 34 anos de idade, com retardo mental, que foi submetida a duas tarefas distintas de geração silenciosa de palavras, por categoria e letras. Encontramos forte ativação da área de Broca na tarefa mais complexa. Sugerimos que um estudo com múltiplas tarefas pode ser útil em pacientes com retardo mental.

This study determined whether hippocampal kainate (KA) receptor mRNA levels were increased or decreased in temporal lobe epilepsy patients compared with nonseizure autopsies. Hippocampal sclerosis (HS;n= 17), nonsclerosis (non-HS;n= 11), and autopsy hippocampi (n= 9) were studied for KA1–2 and GluR5–7 mRNA levels using semiquantitativein situhybridization techniques, along with neuron densities. Compared with autopsy hippocampi, HS and non-HS cases showed decreased GluR5 and GluR6 hybridization densities per CA2 and/or CA3 pyramid. Furthermore, HS patients demonstrated increased KA2 and GluR5 hybridization densities per granule cell compared with autopsy hippocampi. These findings indicate that chronic temporal lobe seizures were associated with differential changes in hippocampal KA1–2 and GluR5–7 hybridization densities that vary by subfield and pathology group. In temporal lobe epilepsy patients, these results support the hypothesis that pyramidal cell GluR5 and GluR6 mRNA levels are decreased as a consequence of seizures, and in HS patients granule cell KA2 and GluR5 mRNA levels are increased in association with aberrant fascia dentata mossy fiber sprouting and/or hippocampal neuronal loss.

BACKGROUND: Where neurocysticercosis (NCC) is endemic, chronic calcified neurocysticercosis (cNCC) can be observed in patients with mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS). Considering that both disorders cause recurrent seizures or cognitive impairment, we evaluated if temporal lobectomy is cognitively safe and effective for seizure control in MTLE-HS plus cNCC. METHODS: Retrospective cohort study of neuropsychological profile and surgical outcome of 324 MTLE-HS patients submitted to temporal lobectomy, comparing the results according to the presence or absence of cNCC. FINDINGS: cNCC occurred in 126 (38.9%) of our MTLE-HS patients, a frequency higher than expected, more frequently in women than in men (O.R. = 1.66; 95% C.I. = 1.05-2.61; p = 0.03). Left-side (but not right side) surgery caused impairment in selected neuropsychological tests, but this impairment was not accentuated by the presence of cNCC. Ninety-four (74.6%) patients with MTLE-HS plus cNCC and 153 patients (77.3%) with MTLE-HS alone were Engel class I after surgery (O.R. = 1.16; 95% C.I. = 0.69-1.95; p = 0.58). However, the chances of Engel class IA were significantly lower in MTLE-HS plus cNCC than in patients with MTLE-HS alone (31.7% versus 48.5%; O.R. = 2.02; 95% C.I. = 1.27-3.23; p = 0.003). Patients with MTLE-HS plus cNCC showed higher rates of Engel class ID (15.1% versus 6.6%; O.R. = 2.50; 95% C.I. = 1.20-5.32; p = 0.012). INTERPRETATION: cNCC can be highly prevalent among MTLE-HS patients living in areas where neurocysticercosis is endemic, suggesting a cause-effect relationship between the two diseases. cNCC does not add further risk for cognitive decline after surgery in MTLE-HS patients. The rates of Engel class I outcome were very similar for the two groups; however, MTLE-HS plus cNCC patients achieved Engel IA status less frequently, and Engel ID status more frequently. Temporal lobectomy can be safely performed in most patients with MTLE-HS plus cNCC without affecting cognitive outcome. Long-term surgical seizure control in MTLE-HS plus cNCC is still satisfactory, as long as selected patients remain under medication.

Os anticorpos antifosfolípides (aFLs) constituem grupo heterogêneo de imunoglobulinas que tem sido relacionado com alterações na coagulabilidade. Indivíduos com títulos elevados teriam maior probabilidade de desenvolver tromboses de repetição, tanto arterial como venosa, e por conseguinte infarto cerebral (IC). Os testes para detecção mais utilizados em estudos clínicos são o inibidor lúpico e a anticardiolipina. Têm-se relatado maiores percentuais de positividade nesses testes em pacientes jovens com IC. Neste estudo procuramos investigar a prevalência desses anticorpos em pacientes com IC entre 15 e 40 anos em nosso Serviço. Examinamos 66 pacientes para presença de aFLs e obtivemos 16,65% de resultados positivos. Confirmamos diagnóstico de síndrome do anticorpo antifosfolípide primária em três (4,55%) casos. Concluímos que a pesquisa de rotina para aFLs em pacientes jovens com IC está indicada neste grupo de pacientes, mas correlacioná-los com o episódio isquêmico nem sempre é possível.The antiphospholipid antibodies (aPLs) are a heterogenous group of immunoglobulins that have been related with alterations in blood coagulability in recent years. Patients with elevated titers of these antibodies have a high probability to develop thrombotic events, including cerebral infarct (CI). The tests currently used to detect these antibodies are the lupus anticoagulant and ELISA for anticardiolipin antibodies which have a larger proportion of positivity among young patients with CI. In our study we tested 66 patients with cerebral infarcts whose ages ranged from 15 to 40 years for the presence of lupus anticoagulant and anticardiolipin antibodies. The results showed that eleven (16.65%) patients were positive for aPLs and three (4.55%) of them fulfilled the diagnostic criteria for primary antiphospholipid syndrome. These data point out to the importance of investigating aPLs in young patients with CI and its high prevalence in this group compared with healthy population.

Os anticorpos antifosfolípides (aFLs) têm sido associados a várias síndromes neurológicas, destacando-se a grande incidência os infartos celebrais. Os mecanismos responsáveis pelo comprometimento vascular encefálico em pacientes com títulos elevados de aFLs permanecem em discussão. A existência de vasculopatia relacionada a este grupo heterogêneo de imunoglobulinas é, no entanto, inquetionável. Relatamos dois casos de síndrome do anticorpo antifosfolípide primário que apresentaram enfartos cerebrais. Os achados angiográficos no primeiro sugeriam vasculite intracraniana, embora houvesse também estenose proximal sugestiva de trombose "in situ". No segundo paciente a angiografia mostrava obstrução de carótida interna, sem sinais de aterosclerose, e na necrópsia foi confirmada a hipótese de vasculopatia trombótica em múltiplos sítios. Nossos achados servem de modelo para discutirmos as controvérsias existentes a respeito da patogênese deste tipo de vasculopatia. Ou seja, se a lesão primária seria vasculite, trombose ou ambas.The antiphospholipid antibodies are associated with a large number of neurologic syndromes, cerebral infarct (CI) being the most common of them. In these cases the pathogenesis of the CI is poorly understood and remains controversial; however, the existence of a vasculopathy is indubitable. We report the cases of two young patients with CI and diagnosis of primary antiphospholipid syndrome who were submitted to cerebral angiograms, and one of them to necropsy. In one case the angiographic findings were similar to those of vasculitis in intracranial vessels. In the other case we observed obstruction in internal carotid artery at the angiography that looked like thrombosis in situ; at necropsy we found non-atherosclerotic obstruction in coronary arteries. In summary, is the primary lesion vasculitis, thrombosis, or both? These cases ilustrate the discussion and demonstrate that vasculitic mechanisms may be involved in the vasculopathy of primary antiphospholipid syndrome eventhough trombosis occur more frequently.

Infartos cerebrais (IC) em jovens apresentam múltiplas etiologias que diferem do padrão observado nos indivíduos idosos. Deficiências de anticoagulantes naturais têm sido descritas nos últimos anos como causa de IC, principalmente em pacientes com menos de 40 anos. Existe tendência atual de se pesquisar essas deficiências em todos os pacientes jovens com infarto cerebral de causa indeterminada e naqueles com manifestações trombóticas de múltiplos sistemas. Realizamos pesquisa dos níveis de proteína C, proteína S e antitrombina III em pacientes entre 15 e 40 anos com ICs classificados como indeterminados após conclusão de protocolo básico de investigação. Diagnosticamos dois casos de deficiência de proteína C e um caso de deficiência de proteína S. Concluímos que a investigação sistemática de causas hematológicas proporciona decréscimo no número de infartos indeterminados, além de possibilitar a adoção de condutas específicas que diminuem a incidência de novos eventos nos casos diagnosticados.The possible etiologies of cerebral infarcts (CI) in young patients differ from those in the older stroke population. Recently, deficiencies of fibrinolytic factors emerged as an important etiology of stroke in the young population. The literature has emphasized the diagnosis of such conditions especially in stroke cases of undetermined etiology and with history of recurrent thrombosis. We carried out a research on the serum level of protein C, protein S, and antithrombin III in young patients, between 15 and 40 years, with CI of undetermined cause after application of a standard protocol. Three patients had low levels of these proteins; two had protein C deficiency, and one protein S deficiency. None of them had antithrombin III deficiency. We conclude that systematic investigation of such causes of cerebral infarct will reduce the group of undetermined strokes, and will open the possibility of prophylactic treatment in a non-negligible group of patients.

One of the subjects that most concerns physicians is treatment-resistance. About 30%–60% of schizophrenia patients do not respond adequately to antipsychotic treatment and are known as refractory schizophrenia patients. Clozapine has been the drug of choice in such cases. However, approximately 30% of them do not respond to clozapine either. Here, we describe a patient with an initial diagnosis of refractory schizophrenia who had a history of dramatic aggressiveness. However, in this case, “refractoriness” was a wrong diagnosis. A case of psychosis secondary to epilepsy had been treated as schizophrenia for almost 20 years. Reports like this one are important because they remind us of how a thorough investigation can lead to the correct diagnosis and improve the patient's prognosis.

People with epilepsy are more likely to die prematurely and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Several studies have reported a moderate or high seizure frequency among SUDEP cases and SUDEP is considered rare in patients in remission. METHOD: We reviewed the occurrence of SUDEP in our epilepsy unit over an 8-year period to identify a potential association between seizure frequency and SUDEP occurrence in children with epilepsy. RESULTS: From 835 patients evaluated, 12 had suffered SUDEP and nearly all of the SUDEP cases in our children are related to chronic uncontrolled epilepsy (daily - 50.0%, two to four/week - 41.7%, monthly - 8.3%). CONCLUSION: SUDEP is not a rare event in children and increased mortality was recorded in those individuals who had not responded to pharmacologic treatment. Improved seizure control seems to be one of the most important measures to prevent SUDEP.Pessoas com epilepsia têm maior chance de morrer prematuramente e a principal causa de morte relacionada à epilepsia é a morte súbita em epilepsia (SUDEP). Vários estudos têm relatado uma freqüência de crises moderada ou elevada em pacientes com SUDEP e SUDEP é considerada rara em pacientes em remissão. MÉTODO: Revisamos a ocorrência de SUDEP em nossa unidade de epilepsia por um período de oito anos com o objetivo de identificar uma possível associação entre freqüência de crises e a ocorrência de SUDEP em crianças com epilepsia. RESULTADOS: De 835 pacientes avaliados, 12 evoluíram com SUDEP e a maioria das crianças apresentava epilepsia crônica não controlada (50% com crises diárias; 41,7% com duas a quatro crises/semana; 8,3% com crises mensais). CONCLUSÃO: SUDEP não é um evento raro em crianças e maior mortalidade foi observada em indivíduos que não responderam ao tratamento medicamentoso. Melhor controle de crises parece ser uma das principais medidas na prevenção de SUDEP.