Surgical outcome in patients with refractory epilepsy associated to MRI-defined unilateral mesial temporal sclerosis.

INTRODUCTION: Several pre-operative work-up protocols have been used while selecting epileptic patients for surgery among different centers. The relative value of the different available pre-operative tests is still under discussion. OBJECTIVE: We report on the surgical outcome obtained in patients with refractory temporal lobe epilepsy associated to mesial temporal sclerosis (MTS) and who were evaluated pre-operatively by interictal EEG and MRI alone. METHOD: Forty one patients with refractory unilateral temporal lobe epilepsy were evaluated using interictal EEG and MRI. MRI disclosed unilateral MTS in all patients. All patients had at least 4 interictal EEG recordings. All patients were submitted to cortico-amygdalo-hippocampectomy at the side determined by MRI. RESULTS: Interictal EEG showed unilateral epileptiform discharges compatible with MRI findings in 37 patients; in four out of the 41 patients, bilateral discharges were found. Mean follow-up time was 4.3+/-1.1 years. Thirty-nine patients (95.1%) were classified as Engels Class I (70.6% Engel I-A). Two patients (4.9%) were rated as Engel's Class II. All patients in whom bilateral discharges were found were in Engels Class I. Pathological examination showed MTS in all patients. CONCLUSION: It is possible to adequately select good surgical candidates for temporal lobe resection using MRI and interictal EEG alone. In patients with MRI-defined MTS we should expect a postoperative remission rate higher then 90%. The finding of MTS on MRI is the most important good prognostic factor after temporal lobe resection.

Surgical outcome in patients with refractory epilepsy associated to MRI-defined unilateral mesial temporal sclerosis

INTRODUÇÃO: Protocolos diferentes têm sido utilizados para a investigação pré-operatória de pacientes epilépticos nos diferentes centros. No entanto, o valor relativo de cada teste disponível ainda é controverso na literatura.OBJETIVO: Relatamos os resultados cirúrgicos de pacientes com epilepsia refratária do lobo temporal associada a esclerose hipocampal (EH), cuja investigação pré-operatória consistiu exclusivamente de estudo de eletrencefalograma (EEG) inter-crítico e ressonância magnética (RM). MÉTODO: Foram estudados 41 pacientes com epilepsia refratária do lobo temporal, avaliados pré-operatoriamente somente por meio de EEG interictal e RM encefálica. Foram incluídos somente pacientes em quem a RM mostrava apenas EH unilateral. Todos pacientes possuíam ao menos quatro EEG inter-ictais. Todos os pacientes foram submetidos a córtico-amígdalo-hipocampectomia no lado demonstrado pela RM.RESULTADOS: A análise do EEG interictal revelou atividade epileptiforme unilateral, compatível com os achados da RM em 37 pacientes. Nos outros quatro pacientes, o EEG evidenciou comprometimento bilateral. O tempo médio de seguimento pós-operatório dos pacientes foi 4,3±1,1 anos. No período pós-operatório, 95,1% dos pacientes encontravam-se em classe I de Engel (70,6% em Engel I-A) e 4.9% em classe II de Engel. Todos os pacientes com achados bilaterais ao EEG estavam em classe I de Engel. O estudo anátomo-patológico das amostras cirúrgicas revelou EH em todos os pacientes. CONCLUSÃO: É possível selecionar bons candidatos à ressecção temporal utilizando-se apenas dados de RM e EEG inter-ictal. Nos pacientes com EH unilateral na RM, espera-se um índice de remissão pós-operatória das crises superior a 90%. O achado de EH na RM é, isoladamente, o maior fator indicativo de bom prognóstico após ressecção temporal.

Extended, one-stage callosal section for treatment of refractory secondarily generalized epilepsy in patients with Lennox-Gastaut and Lennox-like syndromes.

PURPOSE: We report on the efficacy and safety of extended one-stage callosal section performed in a large and homogeneous series of patients. METHODS: Seventy-six patients with Lennox-Gastaut (n = 28) and Lennox-like (n = 48) syndrome were studied (42 male patients; mean age, 11.2 years). All patients had multiple seizure types per day, including atonic, tonic-clonic, atypical absence, myoclonic, and tonic seizures. All of them were developmentally impaired. The EEG showed intense secondary bilateral synchrony in all of them. All patients were submitted to an extended, one-stage, callosal section, leaving only the splenium intact. Mean follow-up time was 4.7 years. RESULTS: Worthwhile improvement (>50%) was noted in 69 of 76 patients; 52 patients had a > or =90% reduction in seizure frequency. Seven patients were seizure free after surgery. The seizure patterns most responsive to surgery were atonic (92%), atypical absence (82%), and tonic-clonic (57%) seizures. All patients had some degree of a transient acute postoperative disconnection syndrome. A consistent increase in attention level was observed postoperatively. CONCLUSIONS: We report one of the larger homogeneous series of patients submitted to callosotomy and are the first to report on the effectiveness and safety of performing extended callosal section in a single stage in this patient group. Extended callosal section should be considered a good palliative surgical option for suitable candidates. The increase in attention level was as useful as seizure control in improving quality of life of these patients.

[Early functional hemispherectomy in hemimegalencephaly associated to refractory epilepsy]. / Hemisferectomia funcional precoce na hemimegalencefalia associada à epilepsia refratária.

Hemimegalencephaly (HME) is a rare congenital brain malformation of unknown etiology. Patients with HME can present with an early onset epileptic syndrome which is often refractory to medical treatment and associated to impaired neurological development. Functional hemispherectomy (FH) has proven to be a valuable treatment alternative in patients with refractory epilepsy in this setting. Very few children operated under the age of 6 months and bearing HME and catastrophic epilepsy (CE) have been described in the literature. This study reports on 2 kids younger than 6 months with HME and CE submitted to FH with good clinical outcome.

Hemisferectomia funcional precoce na hemimegalencefalia associada à epilepsia refratária / Early functional hemispherectomy in hemimegalencephaly associated to refractory epilepsy

A hemimegalencefalia (HME) é malformação congênita cerebral rara de etiologia desconhecida que pode se apresentar com síndrome epiléptica de início precoce e resistente à terapia com anticonvulsivantes, associada a comprometimento significativo do desenvolvimento neuropsicomotor. A hemisferectomia funcional (HF) tem-se mostrado alternativa eficaz nos casos refratários à terapêutica medicamentosa. Em número diminuto, crianças foram operadas antes dos seis meses de idade. Esse estudo relata duas crianças com idade inferior a 6 meses com HME e síndrome epiléptica catastrófica submetidas a HF e com boa evolução clínica.

Esclerose múltipla: análise clínica e evolutiva de 214 casos / Multiple sclerosis: clinical analysis and development of 214 cases

Os autores apresentam uma série de 214 casos de esclerose múltipla provenientes de levantamento retrospectivo de prontuários na Disciplina de Neurologia da Faculdade de Ciências Médicas da Santa Casa de Säo Paulo e da clínica privada, no período de 1980 a 1993. Os dados encontrados foram analisados quanto a sexo, raça, média de idade de início dos sintomas, sintomas iniciais e sintomas evolutivos da doença. Os resultados foram analisados comparativamente aos encontrados em outras séries nacionais e estrangeiras