Américo Ceiki Sakamoto

Cognitive decline in temporal lobe epilepsy due to unilateral hippocampal sclerosis

OBJECTIVEnWe assessed the cognitive performance of patients with temporal lobe epilepsy (TLE) caused by unilateral hippocampal sclerosis (HS), in comparison with that of matched, healthy controls. We report the relationship between cognitive measures and duration of epilepsy, correlating with hippocampal volumes, and the impact of educational level on cognitive decline.nnnMETHODSnThis study involved 61 outpatients (40 with < or = 8 years and 21 with >8 years of formal education) with unilateral HS and 61 controls. Volumetric MRI was performed on all patients and 10 controls. The results (mean, SD) of the neuropsychological tests of healthy subjects and patients were compared using the Student t and Mann-Whitney tests.nnnRESULTSnPatients performed worse than controls in the neuropsychological evaluation. When adjusted z scores were used to calculate the impairment index, patients had a greater percentage of abnormal tests compared with controls. The cognitive decline, assessed through the impairment index, correlated with duration of epilepsy. Higher level of education did not protect against this decline, thus not supporting the hypothesis of cerebral reserve in this population. A significant correlation between hippocampal volumetric measures and duration of epilepsy was observed only in patients with left HS.nnnCONCLUSIONnPatients with TLE caused by HS present with cognitive morbidity that extends beyond memory deficits. Cognitive decline is associated with duration of epilepsy, and in patients with left-sided HS, duration may correlate with volumetric hippocampal loss.

Magnetic resonance spectroscopy reveals an epileptic network in juvenile myoclonic epilepsy

Purpose:u2002 To investigate the cerebral metabolic differences between patients with juvenile myoclonic epilepsy (JME) and normal controls and to evaluate to what extent these metabolic alterations reflect involvement of an epileptic network.

Validity and reliability of the Portuguese version of the quality of life in epilepsy inventory (QOLIE-31) for Brazil

PURPOSEnWe report the cultural adaptation and psychometric properties of the Quality of Life in Epilepsy-31 Inventory (QOLIE-31) for the Portuguese language and Brazilian culture.nnnMETHODSnThis study involved 150 outpatients: 50 presurgical patients with refractory temporal lobe epilepsy (TLE) related to mesial temporal sclerosis (MTS), 50 patients with juvenile myoclonic epilepsy (JME), and 50 seizure-free patients with TLE. They completed the QOLIE-31, Nottingham Health Profile (NHP), Beck Depression Inventory (BDI), and Adverse Events Profile (AEP) and underwent a neuropsychological evaluation (NE). Internal consistency reliability, interrater and test-retest reliability, and construct validity were assessed.nnnRESULTSnQOLIE-31 mean scores were 33.1 (Social Function), 68.9 (Overall Quality of Life), 56.5 (Seizure Worry), 64.1 (Emotional Well-Being), 63.7 (Energy/Fatigue), 38.9 (Cognitive Function), and 49.7 (Medication Effects). Internal consistency was high (Cronbachs alpha), as were the associations between QOLIE-31 and the BDI, NHP, AEP, and NE.nnnCONCLUSIONnThe Portuguese/Brazilian version of the QOLIE-31 inventory showed good reliability, validity, and construct validity.

Psychiatric comorbidity in epilepsy: a study comparing patients with mesial temporal sclerosis and juvenile myoclonic epilepsy.

We evaluated the frequency of psychiatric disorders (PDs) in a homogenous series of patients with temporal lobe epilepsy with mesial temporal sclerosis (TLE-MTS), as compared with patients with juvenile myoclonic epilepsy (JME), aiming to determine possible differences in psychiatric diagnoses between these two epileptic syndromes. Data from 170 patients with refractory TLE-MTS and from 100 patients with JME were reviewed and compared. The prevalence of PDs was high in both groups of patients with epilepsy: PDs were present in 85 patients with TLE-MTS (50%) and 49 patients with JME (49%). Among the TLE-MTS group, mood (25.8%), psychotic (15.8%), and anxiety (14.1%) disorders were the most frequent diagnoses, whereas anxiety and mood disorders (23 and 19%, respectively) were the most common among patients with JME. Psychoses were significantly associated with MTS (P<0.01) and anxiety disorders with JME (P<0.05). These findings suggest the existence of an anatomic correlation between PDs and brain structures involved in both epilepsy syndromes.

The renin-angiotensin system is upregulated in the cortex and hippocampus of patients with temporal lobe epilepsy related to mesial temporal sclerosis.

Purpose: As reported by several authors, angiotensin II (AngII) is a proinflammatory molecule that stimulates the release of inflammatory cytokines and activates nuclear factor κB (NFκB), being also associated with the increase of cellular oxidative stress. Its production depends on the activity of the angiotensin converting enzyme (ACE) that hydrolyzes the inactive precursor angiotensin I (AngI) into AngII. It has been suggested that AngII underlies the physiopathological mechanisms of several brain disorders such as stroke, bipolar disorder, schizophrenia, and disease. The aim of the present work was to localize and quantify AngII AT1 and AT2 receptors in the cortex and hippocampus of patients with temporal lobe epilepsy related to mesial temporal sclerosis (MTS) submitted to corticoamygdalohippocampectomy for seizure control.

Comparação da qualidade de vida e sobrecarga dos cuidadores de pacientes com epilepsia por esclerose mesial temporal e epilepsia mioclônica juvenil

INTRODUCAO: O padrao de relacionamento que se estabelece entre paciente e cuidador pode comprometer a qualidade de vida e o crescimento de ambos. OBJETIVO: Comparar a qualidade de vida e a sobrecarga dos cuidadores de pacientes com epilepsia do lobo temporal (ELT) com esclerose mesial temporal (EMT) e epilepsia mioclonica juvenil (EMJ). CASUISTICA E METODOLOGIA: Foram avaliados 20 cuidadores de pacientes com ELT e 20 cuidadores de pacientes com EMJ, com o Inventario de Qualidade de Vida SF-36 e Questionario de Sobrecarga do Cuidador (Burden Interview - Zarit). RESULTADOS: Houve sobrecarga leve a moderada nos dois grupos (30 no de ELT e 21 pontos para o grupo de EMJ), porem nao foi observada associacao estatisticamente significante entre os grupos. Ao buscarmos uma correlacao entre os dominios do SF-36 e a sobrecarga do cuidador para os dois grupos estudados, observou-se apenas no grupo EMJ: estado geral de saude (p = 0,011), limitacoes por aspectos emocionais (p = 0,037) e saude mental (p = 0,002). CONCLUSAO: Cuidadores de pacientes dos dois grupos sao sobrecarregados de forma semelhante. Esta sobrecarga nao interferiu significativamente na qualidade de vida dos mesmos na maioria dos dominios embora cuidadores de pacientes com EMJ tenham revelado impacto em dominios que medem aspectos da saude emocional e fisica.

Topiramate for the treatment of juvenile myoclonic epilepsy

OBJECTIVEnThe aim of this study was to evaluate the efficacy and tolerability of topiramate (TPM) in juvenile myoclonic epilepsy (JME).nnnMETHODnWe 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).nnnRESULTSnSixteen 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).nnnCONCLUSIONnTPM 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.

Assessment of working memory in patients with mesial temporal lobe epilepsy associated with unilateral hippocampal sclerosis

OBJECTIVEnThe aim of the present study was to investigate whether working memory is impaired in mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS), a controversial and largely unexplored matter.nnnMETHODSnTwenty subjects with left MTLE-HS, 19 with right MTLE-HS, and 21 control right-handed subjects underwent neuropsychological assessment of episodic and semantic memory, executive functions, and specific working memory components.nnnRESULTSnLeft and right epileptogenic foci resulted in impairment of verbal and nonverbal episodic memory (verbal memory deficit greater in left MTLE-HS than in right MTLE-HS). In addition, patients with left MTLE-HS were impaired in learning paired associates, verbal fluency, and Trail Making. No differences were seen in the tests carried out to evaluate the working memory components (except visuospatial short-term memory in right MTLE-HS).nnnCONCLUSIONnIn this study we did not detect reliable working memory impairment in patients with MTLE-HS with either a left or right focus in most tasks considered as tests of working memory components.

EEG recording after sleep deprivation in a series of patients with juvenile myoclonic epilepsy

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 (p<0.05 and p<0.01). As to localization, the number of generalized, bilateral and synchronous ED increased from 21 (56.8%) to 30 (81.1%) (p<0.01). The DI also increased; while 8 patients (21.6%) presented greater rate in the routine EEG, 25 (67.6%) did so in the sleep-deprived EEG mainly during somnolence and sleep (p<0.01). Moreover, the paroxysms were also longer in the sleep-deprived EEG. Sleep-deprived EEG is a powerful tool in JME and can contribute significantly to the syndromic characterization of this syndrome.

Sign of the Cross (Signum Crucis): observation of an uncommon ictal manifestation of mesial temporal lobe epilepsy.

The objective of the study was to describe the clinical characteristics and determine the lateralizing value of ictal Sign of the Cross (SC) as a complex hand automatism (CHA) in patients evaluated by video/EEG monitoring in a comprehensive epilepsy unit. We reviewed video/EEG data of 530 patients with epilepsy recorded in a tertiary epilepsy center from 2002 to 2008. Four patients were found to have manifested a CHA similar to the SC at least once during their complex partial seizures. All patients had unilateral right mesial temporal lobe epilepsy (TLE) refractory to medical treatment. The limbic system is often suggested as the critical site of religious experience. Moreover, it may be localized predominantly to the temporal regions of the right hemisphere. However, this rare and peculiar ictal manifestation may be related not only to the neural substrate and personality characteristics of TLE, but also to the general religious convictions of Brazilians.