Cognitive and surgical outcome in mesial temporal lobe epilepsy associated with hippocampal sclerosis plus neurocysticercosis : a cohort study

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

Analysis of clinical characteristics and risk factors for mortality in human status epilepticus

Purpose: To analyse clinical data including aetiology, age, antecedents, classification and mortality in human status epilepticus (SE), and to assess prognostic factors for mortality.Methods: A prospective study was performed, including detailed analysis of clinical and laboratorial data of SE in individuals of any age. except neonates.Results: One hundred and eleven SE were included, with patients' age ranging from 3 months to 98 years. SE incidence peaked in the first year of life, and 59.4% of the individuals had previous epilepsy while 40.6% had not. the main underlying causes were noncompliance to treatment in the first group, and CNS infection, stroke and metabolic disturbances in the second group. Overall mortality was 19.8%. and deaths were correlated to aetiology and patient's age. Refractory SE affected 11.7% of the cases. Clinical types included focal, secondarily generalised and generalised SE. Clinical and clinicoelectrographic classifications were convergent, but EEG was essential for the diagnosis in 4.5% of the cases.Conclusions: Epileptic patients are at greater risk to develop SE, however, individuals with no prior history of epilepsy and acute neurological problems can also present SE. Aetiology varies with patient's age, and mortality is high and related to age and underlying causes. Clinical and clinicoelectrographic classifications are usually convergent, but in some cases the diagnosis of SE would not be established without the EEG. (C) 2003 BEA Trading Ltd. Published by Elsevier B.V. All rights reserved.Universidade Federal de São Paulo, Dept Neurol & Neurosurg, São Paulo, BrazilUniv São Paulo, Ribeirao Preto Sch Med, Dept Neurol Psychiat & Psychol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Neurol & Neurosurg, São Paulo, BrazilWeb of Scienc

Alta taxa de mortalidade está associada com idade avançada e descargas periódicas lateralizadas em pacientes com estado de mal epiléptico refratário

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.Objetivo Avaliar os dados clínicos, o eletroencefalograma, a etiologia, a classificação, o tratamento, a morbidade e a mortalidade do estado de mal epiléptico. Métodos Quinze pacientes, idade média de 41,3 anos, seis masculinos, foram avaliados retrospectivamente. Todos eles foram acompanhados por eletroencefalogramas seriados ou monitoração eletrencefalográfica contínua. Resultados A 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. Conclusões Alta 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.Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) MD, PhD, Hospital São PauloUNIFESP, EPM, MD, PhD, Hospital São PauloSciEL

Interchangeability among therapeutic equivalents of lamotrigine: evaluation of quality of life

Epilepsia é o distúrbio neurológico grave mais comum no mundo todo. Aproximadamente 70% dos pacientes com epilepsia têm suas crises controladas com tratamento clínico e farmacológico. Esta pesquisa avaliou a possível interferência da intercambialidade entre equivalentes terapêuticos da lamotrigina na condição clínica e na qualidade de vida dos pacientes com epilepsia refratária. O estudo foi dividido em três períodos de 42 dias e em cada período foi dispensado um equivalente terapêutico, aleatoriamente (dois similares - formulação A e B e o medicamento de referência - formulação C). A dose média de lamotrigina foi de 5,5 mg/kg/dia. A ocorrência de efeitos colaterais tende a ser mais decisiva para a redução da qualidade de vida em epilepsia refratária em relação às variações no número de crises ou alterações nas concentrações plasmáticas. Os resultados demonstram que, independentemente do medicamento prescrito, a intercambialidade entre equivalentes terapêuticos pode interferir no sucesso do controle da epilepsia.Epilepsy is the most common serious neurological disorder worldwide. Approximately 70% of patients with epilepsy have their seizures controlled by clinical and pharmacological treatment. This research evaluated the possible influence of interchangeability among therapeutic equivalents of LTG on the clinical condition and quality of life of refractory epileptic patients. The study was divided into three periods of 42 days, and an equivalent therapeutic LTG randomly dispensed for each period (two similars - formulations A and B, and the reference product - formulation C). The mean dose of LTG was 5.5 mg/kg/day. The presence of side effects tends to have a greater deleterious effect on quality of life of refractory epileptics compared to variations in number of seizures or changes in plasma concentrations. The results showed that independently of the drug prescribed, interchangeability among therapeutic equivalents can negatively impact epilepsy control

Lesões destrutivas precoces no cérebro em desenvolvimento: correlatos clínicos e eletrográficos

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.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Departamento de Neurologia e NeurocirurgiaUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Departamento de RadiologiaUniversidade de São Paulo Faculdade de Medicina de Ribeirão Preto Departamento de Neurologia, Psiquiatria e Psicologia MédicaUNIFESP, EPM, Depto. de Neurologia e NeurocirurgiaUNIFESP, EPM, Depto. de RadiologiaSciEL

Tratamento da epilepsia mioclônica juvenil com topiramato

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.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Unidade de Pesquisa e Tratamento das EpilepsiasUNIFESP, EPM, Unidade de Pesquisa e Tratamento das EpilepsiasSciEL

Prevalência de transtornos psiquiátricos em pacientes com esclerose mesial temporal

OBJECTIVE: Behavioral changes in patients with epilepsy can range from depression, anxiety to psychosis and personality traits. We evaluated the frequency of psychiatric disorders (PD) in a homogenous series of patients with refractory temporal lobe epilepsy (TLE) related to mesial temporal sclerosis (MTS) aiming at determining the frequency of PD and possible correlations to clinical variables and to laterality of MTS. METHODS: Data from 106 refractory TLE patients were reviewed. Psychiatric evaluation was based on DSM-IV criteria. Statistical analysis was performed through the chi-square (chi&sup2;), Student's t test and Fisher's exact test. P value considered significant was < 0.05. RESULTS: PD were found in 65 patients (61.3%). Among them, mood disorders were the most frequent (32 patients; 30%), followed by interictal (15 patients; 14%) and postictal (10 patients; 9.4%) psychosis. Postictal and interictal psychosis were significantly associated with left side MTS (p < 0.05), while PD in general and mood disorders were not associated to any side. CONCLUSION: There was a high prevalence of PD in patients with refractory TLE associated to MTS. The most common were mood and psychotic disorders. Psychosis was significantly associated to left side. These findings are concordant with data in literature, confirming the existence of anatomic alterations, and also a possible left laterality effect in the mesial temporal lobe structures in patients with epileptic psychosis.OBJETIVOS: Foi avaliada a freqüência de transtornos psiquiátricos (TP) em uma série homogênea de pacientes com epilepsia do lobo temporal (ELT) associada à esclerose mesial temporal (EMT), com o objetivo de verificar a prevalência e os tipos de TP nessa população e de correlacionar tais transtornos a aspectos clínicos e à lateralidade da EMT. METODOLOGIA: Dados de 106 pacientes com ELT refratária associada a EMT foram revisados. A avaliação psiquiátrica foi baseada nos critérios diagnósticos do DSM-IV. A análise estatística foi realizada através do teste do qui-quadrado (chi&sup2;), do teste t de Student e do teste exato de Fisher. O valor de P considerado significante foi < 0,05. RESULTADOS: TP foram diagnosticados em 65 pacientes (61,3%). Transtornos do humor foram os mais freqüentemente encontrados (32 pacientes; 30%), seguidos por psicose interictal (15 pacientes; 14%) e por psicose pós-ictal (10 pacientes; 9.4%). Tanto a psicose interictal como a psicose pós-ictal foram associadas a EMT à esquerda (p < 0,05), enquanto TP em geral e transtornos do humor não foram correlacionados a nenhum lado. CONCLUSÃO: HOUVE uma elevada prevalência de TP em pacientes com ELT refratária associada a EMT. Os mais freqüentemente encontrados foram transtornos do humor e psicóticos. Quadros psicóticos foram associados a EMT à esquerda. Tais achados são concordantes com dados da literatura atual em relação à prevalência de TP na ELT, confirmando a existência de alterações anatômicas e uma possível associação da EMT à esquerda com quadros psicóticos na ELT.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Universidade Federal de São Paulo (UNIFESP) Unidade de Pesquisa e Tratamento das EpilepsiasUNIFESP, Unidade de Pesquisa e Tratamento das EpilepsiasSciEL

Extreme spindles in magnetic resonance age: clinical and neurphysiological and neuroimaging characteristics

OBJECTIVES: Extreme spindles were described by Gibbs and Gibbs in 1962. They are typically observed in children younger than five years, occurring in 0.05% of normal children and in up to 5 to 18% of children with mental retardation or cerebral palsy. In this study we describe the clinical and neurophysiological characteristics of five children with extreme spindles, correlating these findings to neuroimaging data, obtained in magnetic resonance imaging of the brain. PATIENTS AND METHODS: Eight patients from the children epilepsy outpatient clinic at UNIFESP were initially included, who had extreme spindles in at least one electroencephalogram (EEG) examination. Five out of these eight were selected, since they had MRI of the brain available for analysis. RESULTS: The age of the children varied from two to 15 years. The five children had mental retardation, and three presented associated motor deficits. All had epilepsy; in three children seizures were controlled with antiepileptic drugs, but in two they were considered refractory to medical treatment. In one patient only the MRI of the brain was considered normal. In the other cases, the findings were: bilateral pachygiria, diffuse brain atrophy, right occipital lesion, and bilateral frontal atrophy. The frequency of extreme spindles varied form 8.9 to 16 Hz, and amplitude from 67 to 256 µV. In three patients, frontal fast activity was observed along with extreme spindles. CONCLUSIONS: Extreme spindles are seldom observed in normal children, but may be quite frequent among those with mental retardation or cerebral palsy. They are probably not related to epilepsy, though in our series all children had epilepsy as well as mental retardation. Diagnostic investigation of these children with MRI of the brain showed that extreme spindles may occur either in children with defined structural abnormalities or in those with normal neuroimaging examination, suggesting that this specific electroencephalographic pattern is associated to mental retardation but not to specific etiologies.OBJETIVOS: Fusos Extremos (FE) foram descritos por Gibbs e Gibbs em 1962. São tipicamente observados em crianças menores de cinco anos, ocorrendo em 0,05% de crianças normais e em 5 a 18% das crianças com retardo mental ou paralisia cerebral. Nesse estudo, descrevemos as características clínicas e neurofisiológicas de 5 pacientes com FE, correlacionando esses achados com os aspectos de neuroimagem, através de exame de ressonância magnética (RM) de encéfalo. PACIENTES E MÉTODOS: Foram selecionados inicialmente oito pacientes do ambulatório de epilepsia infantil da UNIFESP, que apresentavam fusos extremos em pelo me-nos um exame de eletroencefalograma (EEG). Dos oito, foram selecionados cinco (três do sexo feminino) que tinham RM de encéfalo disponível para análise. Foram verificados as características clínicas, o desenvolvimento neuropsicomotor e os achados neurofisiológicos, bem como os achados da RM de encéfalo. RESULTADOS: A idade dos pacientes variou de 2 a 16 anos. Os cinco pacientes apresentavam déficits cognitivos, e três apresentavam déficits motores associados. Todas as crianças tinham diagnóstico de epilepsia, sendo que em três as crises estavam controladas com drogas antiepilépticas (DAE) enquanto em dois, eram refratárias ao tratamento clínico. Em apenas um paciente a RM foi normal. Nos demais, os achados foram: paquigiria bilateral, atrofia cortical difusa, lesão focal occipital direita e atrofia frontal bilateral. A freqüência dos FE variou de 8,9 a 16 Hz, a amplitude de 67 a 256 µV. Em três pacientes, além dos FE foi observada atividade rápida de localização frontal. CONCLUSÃO: FE são raramente observados em crianças normais, mas podem ocorrer com maior freqüência naquelas com retardo mental ou paralisia cerebral. FE não parecem estar associados a epilepsia, mas em nossa série todos as crianças tinham diagnóstico de epilepsia, bem como retardo mental. O estudo com RM mostrou que FE podem ocorrer em crianças com anormalidades estruturais definidas bem como naquelas com neuroimagem normal, sugerindo que esse padrão se associa à retardo mental, mas não a etiologias específicas.UNIFESP UNIPETEUNIFESP, UNIPETESciEL

Mortalidade em crianças com epilepsia severa: 10 anos de seguimento

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.Epilepsia é uma das condições neurológicas mais comuns em crianças e adolescentes. Infelizmente, pacientes com epilepsias refratárias ao tratamento medicamentoso estão mais susceptíveis a complicações clínicas e óbito. Neste trabalho reportamos, em uma análise prospectiva, um cohort de crianças acompanhadas por aproximadamente dez anos. Cinquenta e três de 1012 pacientes foram a óbito. Quarenta e dois pacientes morreram em decorrência da epilepsia ou de suas complicações clínicas; as principais causas foram pneumonia (16 casos), sepse (9 casos) e estado de mal epiléptico (8 casos). Em 11 pacientes a causa da morte foi SUDEP. A presença de retardo mental foi significantemente associada a mortalidade em crianças e adolescentes com epilepsia.Universidade de São Paulo Faculdade de Medicina de Ribeirão PretoUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de MedicinaUNIFESP, EPMSciEL