The semiology of benign focal epilepsy with affective symptoms.

Benign focal epilepsy with affective symptoms (BFEAS) is a rare childhood epilepsy syndrome essentially characterized by "epileptic attacks with affective symptoms of a terrifying type". Since the original description, approximately 50 cases have been reported. To our knowledge, however, none of the studies included video-EEG data. Herein, we detail the electroclinical features of a neurodevelopmentally normal 9-year-old boy with epilepsy since the age of 2 years. His seizure semiology essentially consisted of nocturnal focal seizures featuring abrupt fear and autonomic phenomena (such as excessive sweating, repeated swallowing, and coughing), associated with impaired consciousness. These seizures were often secondary generalized, and he had multiple episodes of convulsive status epilepticus. He has been seizure-free for the past year and a half on dual antiepileptic therapy with sulthiamine and valproate. His intellectual and social abilities are excellent (IQ of 116), although he does have difficulties particularly in language learning, and was recently diagnosed with phonological dyslexia with dysorthography. By presenting our patient's history and video-EEG, we intend to further detail the semiology of seizures with affective symptomatology. [Published with video sequence on www.epilepticdisorders.com].

Patient satisfaction with temporal lobectomy/selective amygdalohippocampectomy for temporal lobe epilepsy and its relationship with Engel classification and the side of lobectomy.

PURPOSE: The aim of this study was to investigate not only the effectiveness of epilepsy surgery in improving seizure control but also patient satisfaction with the result of the procedure in a sample of patients operated on at a specialized epilepsy unit. METHODS: Patients with temporal lobe epilepsy who had undergone epilepsy surgery (temporal lobectomy/amygdalohippocampectomy) were interviewed in a standardized telephone survey about their satisfaction with the results of the surgery. The morbidity of the surgery was also analyzed retrospectively. The initial study population consisted of 6 amygdalohippocampectomy and 102 temporal lobectomy patients and was reduced to a final sample consisting of 4 amygdalohippocampectomy and 67 lobectomy patients, as the other patients were not available for interview. Surgical results were based on the Engel classification, and satisfaction with the surgery was assessed by asking patients to rate their result and state whether they would make the same decision (to be operated on) again. RESULTS: A significant number of patients classified as Engel I or II, who considered the surgical outcome good or excellent, said they would have the surgery again (p<0.001). Left temporal lobectomy patients whose results fell in the Engel III/IV bracket were less satisfied (p=0.001) than right temporal lobectomy patients with the same Engel classifications (0.048). Left temporal lobectomy patients who were classified as Engel class III and IV were less likely to have the surgery again if they had the choice (p=0.016). DISCUSSION: Patient satisfaction with the results of epilepsy surgery may depend not only on achieving seizure control but also on the temporal lobe resected. Since worse results were associated with lower satisfaction rates only for left temporal resection patients, it is possible that the cognitive consequences of this procedure compound the worse surgical result, leading to decreased satisfaction.

Vagus nerve stimulation may be a sound therapeutic option in the treatment of refractory epilepsy.

INTRODUCTION: Refractory epilepsy accounts for 20 to 30% of epilepsy cases and remains a challenge for neurologists. Vagus nerve stimulation (VNS) is an option for palliative treatment. OBJECTIVE: It was to study the efficacy and tolerability of VNS in patients implanted with a stimulator at the Curitiba Institute of Neurology (INC). METHODS: A case study of six patients with refractory epilepsy submitted to a VNS procedure at the INC in the last four years was described and discussed. RESULTS: Mean age at time of implantation was 29 years. Mean follow-up was 26.6 months. Seizure frequency decreased in all patients (40-50% (n=2) and ≥80% (n=4)). Three patients no longer required frequent hospitalizations. Two patients previously restricted to wheelchairs started to walk, probably because of improved mood. CONCLUSION: In this population, VNS proved to be a sound therapeutic option for treating refractory epilepsy.

Vagus nerve stimulation may be a sound therapeutic option in the treatment of refractory epilepsy / Estimulação no nervo vago pode ser uma excelente opção no tratamento de epilepsias refratárias

INTRODUCTION: Refractory epilepsy accounts for 20 to 30% of epilepsy cases and remains a challenge for neurologists. Vagus nerve stimulation (VNS) is an option for palliative treatment. OBJECTIVE: It was to study the efficacy and tolerability of VNS in patients implanted with a stimulator at the Curitiba Institute of Neurology (INC). METHODS: A case study of six patients with refractory epilepsy submitted to a VNS procedure at the INC in the last four years was described and discussed. RESULTS: Mean age at time of implantation was 29 years. Mean follow-up was 26.6 months. Seizure frequency decreased in all patients (40-50% (n=2) and >80% (n=4)). Three patients no longer required frequent hospitalizations. Two patients previously restricted to wheelchairs started to walk, probably because of improved mood. CONCLUSION: In this population, VNS proved to be a sound therapeutic option for treating refractory epilepsy.

INTRODUÇÃO: Epilepsias refratárias compreendem de 20 a 30% dos casos de epilepsia e constituem desafio clínico. A neuroestimulação do nervo vago (VNS) é uma opção de tratamento paliativo. OBJETIVOS: Foi estudar a eficácia e a tolerabilidade da VNS nos pacientes implantados no Instituto de Neurologia de Curitiba (INC). MÉTODOS: Um estudo de casos de seis pacientes com epilepsia refratária, submetidos à VNS no INC em quatro anos, foi descrito e discutido. RESULTADOS: A média de idade na implantação foi 29 anos. O seguimento médio foi 26,6 meses. A frequência de crises diminuiu em todos os pacientes (40-50% em um paciente e >80% em quatro). Três pacientes deixaram de internar frequentemente. Dois pacientes restritos a cadeiras de rodas começaram a andar, provavelmente por melhora de seu humor. CONCLUSÃO: Nesta população, a VNS provou ser uma excelente opção no tratamento de epilepsia refratária.

The usefulness of neuronavigation in functional hemispherectomy / Aplicabilidade da neuronavegação em hemisferectomia funcional

Catastrophic epileptic encephalopathies in children comprise devastating conditions that features cerebral dysfunction in association with refractory epileptic seizures. The diagnosis is based on the clinical findings, on magnetic resonance imaging (MRI) of the brain and on electroencephalographic findings. For these conditions, surgery remains essential for attaining seizure control. We report two cases of 5-year-old girls. The first one had a diagnosis of Rasmussen’s syndrome. The second one had a large porencephalic cyst secondary to perinatal cerebral ischemia. Despite trials of anticonvulsants, both patients deteriorated, and a functional hemispherectomy guided by neuronavigation was indicated and performed, with low morbidity and excellent seizure control. The neuronavigation proved to be a valuable guidance tool in performing the functional hemispherectomy, making the disconnections more accurate, and thus decreasing the surgical time and blood loss.

Aplicabilidade da neuronavegação em hemisferectomia funcional As encefalopatias epilépticas catastróficas da infância compreendem condições graves que associam disfunção cerebral e crises epilépticas refratárias. Seu diagnóstico é firmado com base nos dados clínicos e nos achados de ressonância magnética e eletrencefalográficos. Para algumas destas condições o tratamento cirúrgico continua sendo essencial para o controle das crises. Relatamos dois casos de pacientes de 5 anos. A primeira teve diagnóstico de síndrome de Rasmussen. A segunda tinha antecedentes de encefalopatia hipóxico-isquêmica perinatal. Ambas apresentaram epilepsia parcial refratária em associação com rápida deterioração neurológica, e foram submetidas à hemisferectomia funcional com auxílio da neuronavegação, com baixa morbidade e excelente controle das crises. A neuronavegação se mostrou como uma valiosa ferramenta na realização da hemisferectomia funcional, possibilitando desconexões mais precisas, menor tempo de cirurgia e menor perda sanguínea.

Syncope or epileptic fits? Some examples of diagnostic confounding factors.

Syncope is a condition often misdiagnosed as epileptic seizures. However, the differential diagnosis between both conditions can be quite difficult, even for well-trained physicians. Four cases of epilepsy and/or syncope are reported, to exemplify this situation. Each case is discussed individually, and the confounding factors are analyzed.

[Surgical treatment of temporal lobe epilepsy: a series of forty-three cases analysis]. / Tratamento cirúrgico da epilepsia do lobo temporal: análise de 43 casos consecutivos.

Forty-three patients with epilepsy resistant to drug therapy were submitted to temporal lobe epilepsy surgery at the Instituto de Neurologia de Curitiba, from 1998 to 2003. Thirty-nine patients (90.6%) had mesial temporal sclerosis, and four had brain tumors. According to Engel's rating, 83.7% from 37 patients with complete postoperative evaluation were classified as Class I (free of disabling seizure). Postoperative complications (18.6%) were evaluated, with one case of surgical wound infection, one case of hydrocephalus, one case of cerebrospinal fluid fistula, two cases of transient palsy of the trochlear nerve and one case of transient hemiparesis. No death related to epilepsy surgery was found in our study.

Syncope or epileptic fits? Some examples of diagnostic confounding factors

Síncope é uma condição freqüentemente diagnosticada equivocadamente como crise epiléptica. No entanto, existem algumas situações nas quais a diferenciação entre ambas pode ser difícil até mesmo para alguns médicos ou especialistas bastante familiarizados com essas condições. Quatro casos de pacientes com epilepsia e/ou síncope procuraram os autores para elucidação diagnóstica. Cada caso é discutido individualmente, assim como os potenciais fatores de confusão são analisados.

Tratamento cirúrgico da epilepsia do lobo temporal: análise de 43 casos consecutivos / Surgical treatment of temporal lobe epilepsy: a series of forty-three cases analysis

Quarenta e três pacientes com epilepsia refratária ao tratamento medicamentoso foram submetidos à cirurgia de epilepsia do lobo temporal no Instituto de Neurologia de Curitiba, entre os anos de 1998 a 2003. Trinta e nove (90,6 por cento) pacientes apresentavam esclerose mesial temporal, e quatro (9,4 por cento), tumores cerebrais. Dos trinta e sete pacientes que possuíam avaliação pós-operatória completa, 83,7 por cento apresentaram classificação I, segundo Engel (livres de crises incapacitantes). Complicações pós-operatórias ocorreram em 18,6 por cento: uma infecção da ferida operatória, um caso de hidrocefalia, um de fístula liquórica, dois casos de paralisia transitória do IV nervo craniano e um de hemiparesia transitória. Não houve nenhum óbito relacionado à cirurgia de epilepsia no presente estudo.

["Hot-water epilepsy ", "warm-water epilepsy", or bathing epilepsy? Report of three cases and considerations regarding an old theme].

Partial and generalized tonic-clonic reflex seizures related to hot water bathing have been described as temperature-related. We describe three cases of bathing epilepsy: a 28 year-old white male and a 30 year-old white female with spells triggered either by warm or hot water, and a 32 year-old female with spells triggered by hot water. The later two of the three cases presented localized epilepsy and a familial history of epilepsy. A complex tactile stimuli might play the most relevant role on seizure triggering, as well as water temperature with an additive effect over cutaneous stimulation.

O Methohexital Sódico (Brevital) no Teste de Wada: relato de dois casos / The sodium methohexital (Brevital) in Wada's test: report of two cases

O Teste de Wada continua sendo um exame frequentemente utilizado, para a avaliação qualitativa e quantitativa da lateralidade das funções de linguagem e das funções de memória verbal, e do possível déficit residual, uma vez que simula o efeito da cirurgia na investigação pré-operatória de candidatos a lobectomia temporal. No Brasil, há consideráveis dificuldades impostas pelas autoridades sanitárias para obtenção do Amytal (amobarbital sódico). Descreve o protocolo do Teste de Wada realizado com Brevital (methoexital sódico) em dois candidatos a lobectomia temporal, comentar sua eficácia e suas diferenças em relação ao realizado com o Amytal. Relatou-se o uso do Brevital em dois pacientes submetidos a determinação da lateralidade da linguagem e da memória através de protocolo adaptado pata tal. O Brevital, um anestésico de ação curta, mostrou-se eficiente em ambos os casos como substituto ao Amytal. O Brevital pode ser utilizado no Brasil para a realização do Teste de Wada, com a vantagem de possibilitar um exame mais breve, assim como uma investigação abrangente das funções de memória

"Hot-water epilepsy ", "warm-water epilepsy", or bathing epilepsy? Report of three cases and considerations regarding an old theme

Crises reflexas a banhos quentes, tônicas-clônicas parciais e generalizadas foram descritas como relacionadas à temperatura. Descrevemos três casos de epilepsia do banho quente: um homem de 28 anos e uma mulher de 30 anos com crises provocadas por contato com água morna ou quente e uma mulher de 32 anos com crises ao contato com água quente. Os últimos dois casos apresentaram epilepsia localizada e um histórico familiar de epilepsia. Nesta forma de epilepsia, um estímulo táctil complexo parece ter o papel mais relevante na precipitação das crises, sendo potencializado pela temperatura da água.

Severe hypertriglyceridemia as a carbamazepine-induced paradoxical effect

Carbamazepine is probably the most widely anti-epileptic drug prescribed in the world. Thus, potentially serious adverse events must become well known by the medical community. A severe carbamazepine-induced hyperlipidemia after short-term exposure to the drug is described in a single case report. The severe hypertriglyceridemia presented by the patiet may be justified as a paradoxical effect, distinguished from the after long-term exposure effect reported in the literature as an adverse drug event. The importance of a lipid profile study in patients facing long-term carbamazepine therapy is emphasized, due to the potential long-term noxious effects of hyperlipidemias

[Functional magnetic resonance imaging in the determination of dominant language cerebral area]. / Ressonância magnética funcional na determinação da lateralização da área cerebral da linguagem.

Functional magnetic resonance imaging (fMRI) is a technique for detecting minimal changes in brain perfusion and oxygenation secondary to neuronal activation. Its application in the pre-surgical evaluation of epileptic patients with temporal mesial sclerosis is currently being under investigation in several centers. This study aims to describe an activation paradigm for the evaluation of language and memory functions, as an alternative to the worldwide used Wada test, which is an invasive procedure. In order to propose a paradigm adapted to the Portuguese language, we report our experience in determining the dominant cerebral area for language through fMRI with a verbal fluency task. The results of the fMRI from 19 patients studied in Curitiba in a period of approximately two years were studied. Sixteen of them presented with left hemispheric cerebral language dominance. In five patients, results from fMRI and Wada test could be compared and agreed in localization. Our results reinforce the view that fMRI may become an essential tool for medical practice, perhaps for the determination of eloquent areas in the evaluation of candidates for epilepsy surgery.

Ressonância magnética funcional na determinação da lateralização da área cerebral da linguagem / Functional magnetic resonance imaging in the determination of dominant language cerebral area

A ressonância magnética funcional (RMF) é uma nova técnica capaz de detectar pequenas alterações no fluxo sanguíneo e oxigenação de tecidos cerebrais em que ocorre ativação neuronal. O seu emprego na avaliação pré-cirúrgica de pacientes com epilepsia portadores de esclerose mesial temporal está atualmente em avaliação em alguns centros de neurologia. O principal objetivo é encontrar o melhor paradigma de ativação na avaliação das funções de linguagem e memória, visando a substituição do teste de Wada, largamente utilizado nos dias de hoje. Para formular um paradigma já adaptado ao nosso idioma, apresentamos a nossa experiência em uma tarefa comportamental de fluência verbal na determinação da lateralização da área cerebral da linguagem. Avaliaram-se os exames de RMF de uma clínica de imagem particular em Curitiba por período de aproximadamente dois anos. Dos 19 pacientes estudados, obteve-se sucesso no exame em 16 e, destes, todos apresentavam dominância hemisférica cerebral da linguagem à esquerda. Em um subgrupo com 5 pacientes foi possível comparar os resultados obtidos com a técnica de Wada e RMF, havendo concordância entre as técnicas. A partir deste estudo e de vários outros semelhantes na literatura, acreditamos que a RMF está progressivamente conquistando seu espaço na prática médica.