Dysfunctional personality beliefs and executive performance in patients with juvenile myoclonic epilepsy.

BACKGROUND: This article intends to verify the association of dysfunctional beliefs of personality disorders with the executive performance in people with juvenile myoclonic epilepsy (JME). METHODS: Fifty-two patients (35 women, 67.3%) with JME aged 18-50 yrs. (32.3 ±â€¯9.7) were evaluated between May 2017 and April 2018 and compared with controls. All subjects were submitted to the Personality Beliefs Questionnaire (PBQ) (Beck & Beck, 1991; Savoia et al., 2006), Dysexecutive Questionnaire (DQ; Wilson et al., 1996; Macuglia et al., 2016), estimated intelligence quotient (IQ) using Vocabulary and Block Design tests, attention and executive functions evaluation (Controlled Oral Word Association (COWA), Digit Span, Trail Making Tests (TMT) A and B, Stroop and Wisconsin Card Sorting Test (WCST)). The inclusion criteria were as follows: diagnosis of JME (ILAE, 1989); age ≥18 yrs., schooling ≥ 11 yrs. and IQ ≥70. The inclusion criteria for the control group were the same except diagnosis of epilepsy. RESULTS: Compared with controls, patients presented higher scores in PBQ for personality disorders, namely Narcissistic (z = -0.79; p < 0.001), Borderline (z = -0.58; p = 0.002), Paranoid (z = -0.43; p = 0.017), and Histrionic (z = -0.39; p = 0.041). Executive functions were impaired when compared with controls in TMT A (z = -0.97; p = 0.038), TMT B (z = -0.65; p = 0.023), and COWA (z = -0.51; p = 0.001). Patients showed higher WCST scores for Errors (z = -1.62; p ≤ 0.001), Perseverative Errors (z = -0.77; p = 0.001), Non-Perseverative Errors (z = -1.01; p = 0.001), Conceptual Level Response (z = -1.56; p ≤ 0.001), Completed Categories (z = -2.12; p = 0.002), and Failure to Maintain Context (z = -0.49; p = 0.015). Personality Beliefs Questionnaire results showed correlation with lower values in TMT A, Antisocial (r = -0.298; p = 0.032), Narcissistic (r = -0.303; p = 0.029), Schizoid (r = - 0.410; p = 0.003), Histrionic (r = -0.341; p = 0.013), Passive-aggressive (r = -0.341; p = 0.015), and Obsessive-compulsive (r = -0.319; p = 0.021); TMT B results showed a trend for Obsessive-compulsive traits (r = -0.261; p = 0.052); COWA was correlated to Dependent (r = 0.319; p = 0.021); and Digit Span to Passive-aggressive (r = 0.287; p = 0.039). On WCST, Failure to Maintain Context was correlated to Avoidant (r = 0.335; p = 0.017). The DQ was not correlated with PBQ. CONCLUSION: People with JME presented dysfunctional beliefs of personality disorder that were correlated with executive dysfunction. These findings reinforce the need for psychological rehabilitation in these patients.

Epilepsy for primary health care: a cost-effective Latin American E-learning initiative.

A lack of neurologists in Latin America forces primary health care providers to manage epilepsy. With the main goal of improving diagnostic and therapeutic management of patients with epilepsy through training of physicians in the primary health care level, the International League Against Epilepsy Education Commission (2013-2017) created a low-cost, regional, virtual course. The course, set-up in Moodle platform, was structured in eight modules, each lasting for a week. Teaching was based on written didactic material, videos, and interactive discussions, both in Spanish and Portuguese. Topics included epidemiology, diagnosis, classification, treatment, prognosis, social issues, and epilepsy policies. Each course was limited to 50 participants and priority was given to general practitioners. Certification was given to those approving the final examination. Since 2015, five courses have been developed, involving 143 participants from 17 countries and 21 tutors. Of the participants, 61% worked in primary health care services. A total of 129 participants (90%) completed the course, and 110 submitted the final examination with an approval rate of 95%. From 85 participants completing the course evaluation, 98% would recommend the course to other colleagues, and 99% showed interest in taking other similar courses. High self-confidence for the management of patients with epilepsy increased from 21% at baseline to 73% after the course. The online course on epilepsy for primary care physicians in Latin America was shown to be a cost-effective course, with good retention and excellent approval rates. Our current challenges include periodic updating, complete self-sustainability, and exploring different strategies to reach our target audience more effectively.

Olfactory stimulation induces delayed responses in epilepsy.

Precipitation and inhibition of seizures and epileptic discharges by sensory stimuli are receiving increasing attention because they provide insight into natural seizure generation in human epilepsies and can identify potential nonpharmacological therapies. We aimed to investigate modulation (provocation or inhibition) of epileptiform discharges (EDs) in mesial temporal lobe epilepsy (MTLE) versus idiopathic generalized epilepsy (IGE) by olfactory stimulation (OS) compared with standard provocation methods. The underlying hypothesis was that any response would be more likely to occur in MTLE, considering the anatomical connections of the temporal lobe to the olfactory system. This multicenter, international study recruited patients with either MTLE or IGE who were systematically compared for responses to OS using an EEG/video-EEG protocol including a 30-min baseline, twice 3-min olfactory stimulation with ylang-ylang, hyperventilation, and intermittent photic stimulation. The 95% confidence interval (CI) for the baseline EDs in each patient was calculated, and modulation was assumed when the number of EDs during any 3-min test period was outside this CI. A total of 134 subjects (55 with MTLE, 53 with IGE, and 26 healthy controls) were included. Epileptiform discharges were inhibited during OS in about half the patients with both MTLE and IGE, whereas following OS, provocation was seen in 29.1% of patients with MTLE and inhibition in 28.3% of patients with IGE. Olfactory stimulation was less provocative than standard activation methods. The frequent subclinical modulation of epileptic activity in both MTLE and IGE is in striking contrast with the rarity of reports of olfactory seizure precipitation and arrest. Inhibition during OS can be explained by nonspecific arousal. The delayed responses seem to be related to processing of olfactory stimuli in the temporal lobe, thalamus, and frontal cortex.

Higher mortality rate is associated with advanced age and periodic lateralized epileptiform discharges in patients with refractory status epilepticus.

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.

Higher mortality rate is associated with advanced age and periodic lateralized epileptiform discharges in patients with refractory status epilepticus / Alta taxa de mortalidade está associada com idade avançada e descargas periódicas lateralizadas em pacientes com estado de mal epiléptico refratário

To evaluate clinical data, electroencephalogram, etiology, classification, treatment, morbidity, and mortality in acute refractory status epilepticus. 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. 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. 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. .

Avaliar os dados clínicos, o eletroencefalograma, a etiologia, a classificação, o tratamento, a morbidade e a mortalidade do estado de mal epiléptico. Quinze pacientes, idade média de 41,3 anos, seis masculinos, foram avaliados retrospectivamente. Todos eles foram acompanhados por eletroencefalogramas seriados ou monitoração eletrencefalográfica contínua. 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. 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. .

[Post-operative progress of patients with Rasmussen's encephalitis who have undergone hemispherotomy surgery]. / Evolucion posquirurgica en pacientes con encefalitis de Rasmussen operados por hemisferotomia.

INTRODUCTION: Rasmussen's encephalitis (RE) is a progressive pathology affecting the brain that causes unilateral hemispheric atrophy, neurological dysfunction and refractory epilepsy. Hemispherotomy is considered the most effective treatment today, but some cases present certain peculiarities that can seriously affect the decision to go ahead with this procedure. AIMS: To evaluate the post-operative progress made by children with RE who have undergone hemispherotomy surgery, and who, in the pre-operative assessment, presented certain characteristics that complicated the decision to perform surgery. PATIENTS AND METHODS: The sample selected for study consisted of the cases of RE attended in the Hospital Sao Paulo between 2003 and 2012 who, in the pre-surgery evaluation, presented clinical, electroencephalographic or neuroimaging evidence of involvement of both brain hemispheres, compromise of the dominant brain hemisphere, absence of severe neurological deficit and absence of criteria for refractory epilepsy and atypical crises. The post-operative assessment of the epileptic seizures was evaluated using the Engel scale; motor function was analysed with the Gross Motor Function Classification System and Manual Ability Classification System scales, and language was evaluated clinically. RESULTS: Six cases were selected (four girls), with a mean age at clinical onset of 3.3 ± 1.2 years (range: 2-7 years) and a mean age at hemispherotomy of 6.7 years (range: 2.3-16.5 years). The mean post-surgery follow-up time was three years (range: 0.5-7.2 years). In the post-surgery evaluation of the epileptic seizures, four cases were classified as Engel class I (66%); there was some improvement in motor functioning in five of them, and language improved in all cases. CONCLUSIONS: Hemispherotomy must be considered an efficient option for treatment in children with RE.

Evolución posquirúrgica en pacientes con encefalitis de Rasmussen operados por hemisferotomía / Post-operative progress of patients with Rasmussen’s encephalitis who have undergone hemispherotomy surgery

Introducción. La encefalitis de Rasmussen (ER) es una afección cerebral progresiva que provoca una atrofia hemisférica unilateral, disfunción neurológica y epilepsia refractaria. La hemisferotomía se considera actualmente el tratamiento más efectivo, pero algunos casos presentan particularidades que dificultan la decisión de realizarla. Objetivo. Evaluar la evolución posquirúrgica de niños con ER operados por hemisferotomía, que en la evaluación prequirúrgica presentaron algunas características que dificultaron la decisión quirúrgica. Pacientes y métodos. Se seleccionaron los casos con ER, atendidos en el Hospital São Paulo entre 2003 y 2012, que en la evaluación prequirúrgica presentaran evidencias clínicas, electroencefalográficas o en la neuroimagen de participación de ambos hemisferios cerebrales, afectación del hemisferio cerebral dominante, ausencia de déficit neurológico grave y ausencia de criterios de epilepsia refractaria y crisis atípicas. Para la evaluación posquirúrgica de las crisis epilépticas se utilizó la escala de Engel; para analizar la función motora, las escalas Gross Motor Function Classification System y Manual Ability Classification System, y el lenguaje se evaluó clínicamente. Resultados. Se seleccionaron seis casos (cuatro niñas), con edad media de inicio clínico de 3,3 ± 1,2 años (rango: 2-7 años) y edad media de hemisferotomía de 6,7 años (rango: 2,3-16,5 años). El tiempo medio de seguimiento posquirúrgico fue de tres años (rango: 0,5-7,2 años). En la evaluación posquirúrgica de las crisis epilépticas, cuatro casos se clasificaron como Engel clase I (66%); hubo mejoría en la función motora en cinco, y mejoría en el lenguaje, en todos. Conclusión. La hemisferotomía debe considerarse una opción eficiente de tratamiento en niños con ER (AU)

Introduction. Rasmussen’s encephalitis (RE) is a progressive pathology affecting the brain that causes unilateral hemispheric atrophy, neurological dysfunction and refractory epilepsy. Hemispherotomy is considered the most effective treatment today, but some cases present certain peculiarities that can seriously affect the decision to go ahead with this procedure. Aims. To evaluate the post-operative progress made by children with RE who have undergone hemispherotomy surgery, and who, in the pre-operative assessment, presented certain characteristics that complicated the decision to perform surgery. Patients and methods. The sample selected for study consisted of the cases of RE attended in the Hospital São Paulo between 2003 and 2012 who, in the pre-surgery evaluation, presented clinical, electroencephalographic or neuroimaging evidence of involvement of both brain hemispheres, compromise of the dominant brain hemisphere, absence of severe neurological deficit and absence of criteria for refractory epilepsy and atypical crises. The post-operative assessment of the epileptic seizures was evaluated using the Engel scale; motor function was analysed with the Gross Motor Function Classification System and Manual Ability Classification System scales, and language was evaluated clinically. Results. Six cases were selected (four girls), with a mean age at clinical onset of 3.3 ± 1.2 years (range: 2-7 years) and a mean age at hemispherotomy of 6.7 years (range: 2.3-16.5 years). The mean post-surgery follow-up time was three years (range: 0.5-7.2 years). In the post-surgery evaluation of the epileptic seizures, four cases were classified as Engel class I (66%); there was some improvement in motor functioning in five of them, and language improved in all cases. Conclusions. Hemispherotomy must be considered an efficient option for treatment in children with RE (AU)

Absence of gender effect on amygdala volume in temporal lobe epilepsy.

Sexual dimorphism has already been described in temporal lobe epilepsy with mesial temporal sclerosis (TLE-MTS). This study evaluated the effect of gender on amygdala volume in patients with TLE-MTS. One hundred twenty-four patients with refractory unilateral or bilateral TLE-MTS who were being considered for epilepsy surgery underwent a comprehensive presurgical evaluation and MRI. Amygdalas of 67 women (27 with right; 32 with left, and 8 with bilateral TLE) and 57 men (22 with right, 30 with left, and 5 with bilateral TLE) were manually segmented. Significant ipsilateral amygdala volume reduction was observed for patients with right and left TLE. No gender effect on amygdala volume was observed. Contralateral amygdalar asymmetry was observed for patients with right and left TLE. Although no gender effect was observed on amygdala volume, ipsilateral amygdala volume reductions in patients with TLE might be related to differential rates of cerebral maturation between hemispheres.

IV Escola Latino-Americana de Verão em Epilepsia: chamada especial aos epileptologistas brasileiros / IV Latin-American Summer School on Epilepsy: special call to the brazilian epileptologists

INTRODUÇÃO: Produzir tratamentos novos e efetivos aliados à prevenção adequada das epilepsias, a doença neurológica crônica grave mais comum e que acomete aproximadamente 1 por cento da população mundial, deve ser o principal objetivo dos sistemas de saúde de uma nação. OBJETIVOS: Nesse sentido, o principal objetivo de nosso artigo é divulgar a IV Escola Latino-Americana de Verão em Epilepsia entre os epileptologistas brasileiros.

INTRODUCTION: The development of new treatments and effective means to prevent the epilepsies, the most prevalent neurological disorder and that affects around 1 percent of worldwide population, must be the main goal of the health systems of a nation. OBJETIVES: Following these reasoning, the goal of our article is to promote the IV Latin-American Summer School on Epilepsy (LASSE) among Brazilian epileptologists.

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.

Can intraoperative electrocorticography patterns predict surgical outcome in patients with temporal lobe epilepsy secondary to unilateral mesial temporal sclerosis?

INTRODUCTION: Intraoperative electrocorticography (ECoG) can be performed in cases of temporal lobe epilepsy due to hippocampal sclerosis (TLE-HS). However, its significance and correlation with surgical outcome are still controversial. OBJECTIVES: To analyze the electrophysiological characteristics of temporal lobe structures during ECoG of patients with TLE-HS, with emphasis on the comparison between pre- and post-resection recordings and surgical outcome. PATIENTS AND METHODS: Seventeen patients with refractory TLE-HS submitted to corticoamigdalohipocampectomy were included in the study. Clinical variables included age at the onset, duration of epilepsy and seizure outcome. The post-operative follow-up ranged from 24 to 36 months. According to outcome subjects were divided in two subgroups: (A) individuals free of seizures (Engel 1A), and (B) individuals not-free of seizures (Engel 1B-IV). Four patterns of ECoG findings were identified: isolated discharges; high frequency spikes (HFS); continuous discharges; combination of isolated discharges and HFS. According to predominant topography ECoG was classified as mediobasal, lateral (or neocortical), mediobasal and lateral. RESULTS: The progressive removal of the temporal pole and the hippocampus was associated with significant decrease of neocortical spikes. No correlation between clinical variables and seizure outcome was observed. Patients who only had isolated spikes on intraoperative ECoG presented a statistical trend for excellent surgical control. Patients who presented temporal pole blurring on MRI also had better post-surgical seizure outcome. CONCLUSIONS: This study showed that out of diverse clinical and laboratory variables, only isolated discharges on intraoperative ECoG and temporal pole blurring on MRI predicted excellent post-surgical seizure outcome. However, other studies with larger number of patients are still necessary to confirm these findings.

Occipital intermittent rhythmic delta activity in absence epilepsy.

Occipital intermittent rhythmic delta activity (OIRDA) is considered good prognostic factor in typical absences (TA). We report electroclinical evolution in 14 patients with TA and OIRDA, which performed video-EEG. Seven patients were female; 9 had childhood absence epilepsy and the others did not present electroclinical characteristics for syndromic classification according to ILAE's classification (1989). Pyknolepsy was referred to in 13; TA was the only seizure type in 13; one had generalized tonic-clonic seizures (GTCS) and three had myoclonic jerks during TA. VPA monotherapy controlled seizures in 11, diVPA and ESM, in one each. After seizure control EEG normalized in 10 while in three, spike-wave complexes (SWC) persisted, accompanied by OIRDA in one. Finally in another, seizures were not controlled and SWC and OIRDA persisted. In conclusion, we observed in this series of TA and OIRDA with onset before 10 years, pyknolepsy as common finding and few GTCS. VPA controlled seizures in most cases and EEG normalized in 76.92%. We suggest that OIRDA could be considered good prognostic factor in TA associated with SWC and of epileptiform nature leading to appropriate investigation.

Occipital intermittent rhythmic delta activity in absence epilepsy

Atividade occipital delta rítmica intermitente (AODRI) é considerada fator de bom prognóstico em crises de ausência típica (AT). Neste estudo relatamos a evolução eletroclínica de 14 pacientes com AT e AODRI que realizaram vídeo-EEG. Sete pacientes eram do sexo feminino; nove tinham epilepsia ausência da infância e os outros não apresentavam características eletroclínicas para classificação sindrômica de acordo com a classificação da ILAE (1989). Picnolepsia foi relatada em 13; AT foi o único tipo de crise em 13; um tinha crises generalizadas TCG e três, abalos mioclônicos durante AT. Monoterapia com VPA controlou as crises em 11, diVPA e ESM, em um cada. Após o controle das crises, o EEG normalizou em 10; em três, complexos de espícula-onda (CEO) persistiram, acompanhados por AODRI em um. Finalmente em outro, as crises não foram controladas, persistindo CEO e AODRI. Concluindo, observamos nesta série de AT e AODRI, com início antes dos 10 anos, picnolepsia freqüente e poucas crises TCG. VPA controlou as crises na maioria dos casos e o EEG normalizou em 76,92%. Sugerimos que AODRI possa ser considerada um fator de bom prognóstico em AT associada a CEO e de possível natureza epileptiforme, levando por sua vez, à investigação apropriada.

Neuropsychiatric profiles of patients with juvenile myoclonic epilepsy treated with valproate or topiramate.

In this cross-sectional study, the neuropsychiatric profiles of 42 patients with juvenile myoclonic epilepsy (JME) who were treated with valproate (VPA) or topiramate (TPM) in monotherapy were compared with the aim of verifying the relationship between cognitive dysfunction, psychiatric disorders, and factors related to epilepsy. Patients with JME taking VPA 500-1750 mg/day or TPM 50-175 mg/day were selected. For all patients, psychiatric profiles were evaluated with the Scheduled Clinical Interview, axes I and II (SCID I and SCID II), or the Brazilian version of the Schedule for Affective Disorders and Schizophrenia for School-Aged Children (K-SADS-PL). Neuropsychological measures included intellectual functions, attention, memory, executive functions, and language. Patients taking TPM exhibited worse neuropsychological performance on attention, short-term memory, processing speed, and verbal fluency functions related to frontal lobes, which may be dysfunctional in JME. Anxiety disorders were associated with lack of seizure control and having had more than 20 lifetime generalized tonic-clonic seizures.