The Important Role of Hemispherotomy for Rasmussen Encephalitis: Clinical and Functional Outcomes.

BACKGROUND: Rasmussen encephalitis (RE) is characterized by pharmacoresistant epilepsy and progressive neurological deficits concurrent with unilateral hemispheric atrophy. Evidence of an inflammatory autoimmune process has been extensively described in the literature; however, the precise etiology of RE is still unknown. Despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, surgical disconnection of the affected hemisphere is considered the treatment of choice for these patients. The aim of this study was to report a series of children and adolescents who underwent hemispheric surgery (HS) for the treatment of RE, analyzing their clinical, electrographic, and neuroimaging features pre-operatively, as well as their postoperative status, including seizure and functional outcomes. METHODS: All patients with RE who underwent HS in the Epilepsy Surgery Center (CIREP) of the University Hospital of Ribeirão Preto Medical School, between 1995 and 2020 were retrospectively reviewed. Preoperative and postoperative analyses included gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery; duration of epilepsy; surgical complications; duration of follow-up; histopathological findings; and postoperative seizure, cognitive, and functional outcomes. RESULTS: Forty-four patients were evaluated. Mean age at seizure onset and epilepsy duration was 6 years and 2.5 years, respectively. Mean age at surgery was 9 years, with an average follow-up ranging from 3 months to 23 years. All patients presented with severe epilepsy and distinct neurological abnormalities on MRI. Before HS, different degrees of abnormal intellectual performance as well as hemiparesis were seen in 86% and 90%, respectively. Histopathology examination confirmed this diagnosis in 95% patients. At the last follow-up, 68% of patients were seizure free, and 70% were classified as Engel Class I or II. Postoperatively, the cognitive status remained unchanged in 64% of patients. Likewise, the gross motor function remained unchanged in 54% of patients and 74% had functional hand ability after HS. CONCLUSIONS: Considering the progressive damage course of RE, hemispheric surgery should be offered to pediatric patients. It has manageable risks and results in good seizure outcome, and the preoperative functional status of these children is often preserved (even when the left hemisphere is involved), thus improving their quality of life.

Clinical and Surgical Approach for Cerebral Cortical Dysplasia.

The present article describes pathophysiological and clinical aspects of congenital malformations of the cerebral tissue (cortex and white matter) that cause epilepsy and very frequently require surgical treatment. A particular emphasis is given to focal cortical dysplasias, the most common pathology among these epilepsy-related malformations. Specific radiological and surgical features are also highlighted, so a thorough overview of cortical dysplasias is provided.

Long-term seizure outcome and mobility after surgical treatment for Rasmussen encephalitis in children: A single-center experience.

OBJECTIVE: Rasmussen Encephalitis (RE) is a rare inflammatory neurodegenerative disease associated with refractory seizures, hemiparesis, and cognitive deterioration, due to lateralized cortical atrophy. Hemispheric surgery (hemispherotomy) is the mainstay of treatment, but its unavoidable motor deficits and lack of long-term data regarding seizure outcomes can make patients and families apprehensive to undergo this procedure. The present study aimed at analyzing the results of surgical treatment for RE from a motor and epilepsy standpoint, and mitigate such concerns. METHODS: Clinical and operative data were retrospectively collected from medical records of pharmacoresistant patients treated with functional hemispherectomy at a tertiary reference center for epilepsy surgery, during a 24-year period (1996-2020). Variables such as age of epilepsy onset, seizure semiology, seizure frequency, immunomodulatory therapy, age at surgery, duration of epilepsy, surgical procedures and complications, number of medications used preoperatively and postoperatively were described and statistically analyzed. RESULTS: Forty-three (43) patients were included in this study. Mean age of epilepsy onset was 6.14 years, the average interval between epilepsy onset and hemispherotomy was 2.21 years. and the mean age at surgery was 8.28 years. Thirty patients (69.7%) were Engel I at their last follow-up, of whom 23 (56.4%) were Engel Ia, within a mean follow-up of 11.3 years. Duration of epilepsy, seizure frequency, and age at surgery, among others, did not correlate with seizure outcome, except the use of immunotherapy which led to worse outcomes (p < .05). Also, after surgery, motor functionality was significantly recovered (i.e., most patients returned to their previous status) with time. SIGNIFICANCE: This study tackled some issues regarding the surgical treatment of this disease, particularly showing that hemispherotomy is safe and leads to potentially recoverable disability of motor functions while providing high rates of effective and long-lasting seizure control; therefore, early surgical indication should be warranted once medical refractoriness has been established.

Executive functioning in children with posterior cortex epilepsy compared to temporal and frontal lobe epilepsies.

OBJECTIVE: People with epilepsy are at an increased risk of experiencing executive dysfunction, particularly those with frontal lobe epilepsy (FLE). The literature has also demonstrated alterations in executive functioning (EF) in patients with temporal lobe epilepsy (TLE). However, few studies have examined the neuropsychological profile of posterior cortex epilepsy (PCE), and little attention has been given to cognitive impairments in the pediatric population with PCE. This study aims to investigate EF performance in children with drug-resistant PCE compared to patients with FLE and TLE. METHODS: We analyzed neuropsychological data from 217 patients aged 6-18 years who underwent preoperative evaluation for epilepsy surgery. The EF of patients with PCE was compared to patients with FLE and TLE. RESULTS: There was no significant difference in Full-Scale Intelligence Quotient (FSIQ) means between groups. However, we found a significant effect of brain region on the Coding task, in which patients with PCE and FLE performed worse than those with TLE (p = 0.034). We also observed performance differences between groups on the Stroop test (p = 0.005), with patients with PCE and FLE performing worse than the TLE group. SIGNIFICANCE: These findings suggest that children with PCE have alterations in their EF that are similar to the deficits found in FLE compared to patients with TLE. This emphasizes the importance of understanding the neuroanatomy of executive functions and the model of neural networks extending beyond the prefrontal cortex.

Reduction in anti-seizure medications use in pediatric patients with pharmacoresistant epilepsy submitted to surgical treatment.

PURPOSE: We aimed to analyze the potential for postoperative (PO) medication suspension and reduction, emphasizing passive withdrawal. METHODS: Retrospective study of patients under 18 years old submitted to surgical treatment for pharmacoresistant epilepsy and classified as Engel I during the first year of PO follow-up. Therapeutic management was evaluated through discontinuation or reduction of medications, both in terms of the number of ASM prescribed and in daily maintenance dosages in mg/kg. RESULTS: ASM withdrawal started in the first year PO and occurred in 1.2% of cases, with a significant yearly reduction in the number of ASM during follow-up (p < 0.001). A comparison of the most commonly used ASM in daily mg/kg between the preoperative period (preop) and PO showed a reduction of ASM maintenance dosages during PO. Even though recurrence of seizures was observed 5 years after surgery, 125 patients (85%) were still classified as Engel I, albeit a higher number of ASM per patient was observed. Most patients showed no changes in cognitive and adaptive behavior evaluation between preop and PO, even in those who were able to reduce ASM. CONCLUSION: Significant reduction observed both in the number and daily maintenance dosages of ASM following each year of PO may be an indirect measure of the effectiveness of epilepsy surgery.

Differential diagnosis between autism spectrum disorder and other developmental disorders with emphasis on the preschool period.

BACKGROUND: Neurodevelopmental disorders are a heterogeneous group of conditions that manifest as delays or deviations in the acquisition of expected developmental milestones and behavioral changes. Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by deficits in communication and social interaction and by repetitive and restricted patterns of behavior, interests and activities. The aim of this review is to discuss the clinical features of the differential diagnoses of ASD that are prevalent among preschoolers, focusing on their similarities and disparities. DATA SOURCES: The international medical literature search was conducted using PubMed and was revised regarding the subject using single and/or combined keywords as follows: differential diagnosis, preschoolers, diagnostic challenge, attention deficit hyperactivity disorder, intellectual disability, high abilities/giftedness, childhood apraxia of speech, social communication disorder, Landau-Kleffner syndrome, stereotyped movement disorder and excessive screen time. RESULTS: We describe conditions commonly found in clinical practice, taking ASD as a reference. We addressed converging and divergent aspects of behavior, cognition, communication, language, speech, socialization, and stereotypes for the diagnosis of ASD and other disorders identified as potential differential or comorbid diagnoses. CONCLUSIONS: The ranking and characterization of symptoms appear to be essential for better understanding the underlying common ground between children with developmental disorders and children with ASD, thus properly diagnosing and directing social, professional, or medication interventions. This detailed discussion adds to the literature since, although ASD differential diagnoses are frequently mentioned and discussed in textbooks and journal articles, they rarely occupy a prominent place as we aimed herein.

Attitudes and practices in the management of attention deficit hyperactivity disorder among Brazilian pediatric neurologists who responded to a national survey: a cross-sectional study

ABSTRACT BACKGROUND: Attention deficit hyperactivity disorder (ADHD) has a prevalence of 5.3% among children and adolescents. It is characterized by attention deficit, hyperactivity, and impulsivity. OBJECTIVE: We aimed to conduct a survey involving pediatric neurologists in the management of ADHD and compare the results with the current literature and guidelines. DESIGN AND SETTING: Descriptive analytical study of a virtual environment, was used Test of equality of proportions for comparison between two groups of pediatric neurologists (working as specialists for > 6 versus ≤ 6 years), with a significance level of P = 0.05. METHODS: This cross-sectional study used a virtual questionnaire covering the steps in the diagnosis and treatment of children with ADHD. The inclusion criteria were professionals who had completed their residency/specialization in pediatric neurology and clinical neurologists working in pediatric neurology. RESULTS: Among the 548 electronic invitations sent, 128 were considered valid. For all participants, the diagnosis was clinically based on the disease classification manuals. Combination treatment promotes improvement of symptoms (96.9%). Among psychostimulants, short-acting methylphenidate was the most commonly prescribed medication (85.2%). Headache was the most common side effect (77.3%). Altogether, 73.4% of the participants requested laboratory tests, 71.1% requested an electrocardiogram, and 42.2% requested an electroencephalogram. Pediatric neurologists working as specialists for ≤ 6 years had more frequent referrals to psycho-pedagogists for diagnosis (P = 0.03). CONCLUSIONS: The participants complied with clinical guidelines, emphasizing the relevance of diagnostic manuals and treatment guidelines for an eminently clinical situation and enabling uniformity in quality treatment.

Attention-Deficit Hyperactivity Disorder (ADHD) versus Specific Learning Disorder-Reading Subtype (Dyslexia): performance in writing tasks / Transtorno do Déficit de Atenção e Hiperatividade (TDAH) versus Transtorno Específico de Aprendizagem - Subtipo Leitura (Dislexia): desempenho em tarefas de escrita

ABSTRACT Purpose: to analyze and compare the writing performance between students with attention-deficit/hyperactivity disorder (ADHD) and students with dyslexia. Methods: altogether, 27 children participated in the study, divided into the groups G-ADHD and G-Dyslexia. Their writing was assessed with a test that uses word and pseudoword dictation. The analysis addressed their writing level, word/pseudoword writing performance, and misspelling types. The groups were compared with the two-proportion z-test between two samples and the Mann-Whitney test (α = 0.05). Results: only one child in G-Dyslexia out of the 27 participating children was classified at the syllabic-alphabetical level. The others were classified at the alphabetical level, with no statistical difference between the groups in this item. The analysis of word/pseudoword writing performance revealed a difference between mean total scores, in which G-ADHD performed better. This group also had a higher percentage of children whose performance was classified as adequate for their age. There was a difference in misspellings between the groups in the omission of syllables, omission/addition of letters in complex syllables, and total performance - G-dyslexia made such errors more often. Conclusion: children with ADHD performed better in writing than the ones with dyslexia. However, writing cannot be used as a diagnostic marker between these conditions.

RESUMO Objetivo: analisar e comparar o desempenho em escrita entre escolares com Transtorno do Déficit de Atenção e Hiperatividade (TDAH) ou escolares com Dislexia. Métodos: participaram 27 crianças, divididas nos grupos: G-TDAH; G-Dislexia. Para avaliação da escrita, utilizou-se um teste que se baseia no ditado de palavras/pseudopalavras e foram analisados o nível de escrita, o desempenho em escrever palavras/pseudopalavras e os tipos de erros ortográficos presentes. Para comparação entre os grupos foram utilizados os testes Igualdade de Proporções entre duas amostras e Mann-Whitney (α=0,05). Resultados: das 27 crianças participantes, apenas uma do G-Dislexia foi classificada no nível silábico-alfabético. Todas as outras foram classificadas no nível alfabético, não havendo diferença estatística deste item entre os grupos. Em relação à análise do desempenho em escrever palavras/pseudopalavras, observou-se diferença entre os valores médios totais, com melhor desempenho do G-TDAH. Neste mesmo grupo, há maior porcentagem de crianças classificadas com desempenho adequado para a idade. Em relação aos erros ortográficos, houve diferença entre os grupos em omissão de sílabas, omissão/adição de letras em sílabas complexas e no desempenho total, sendo o G-dislexia com maior número destes tipos de erros. Conclusão: crianças com TDAH apresentaram melhor desempenho em escrita do que crianças com dislexia, porém, a escrita não pode ser utilizada como um marcador diagnóstico entre essas condições.

Attitudes and practices in the management of attention deficit hyperactivity disorder among Brazilian pediatric neurologists who responded to a national survey: a cross-sectional study.

BACKGROUND: Attention deficit hyperactivity disorder (ADHD) has a prevalence of 5.3% among children and adolescents. It is characterized by attention deficit, hyperactivity, and impulsivity. OBJECTIVE: We aimed to conduct a survey involving pediatric neurologists in the management of ADHD and compare the results with the current literature and guidelines. DESIGN AND SETTING: Descriptive analytical study of a virtual environment, was used Test of equality of proportions for comparison between two groups of pediatric neurologists (working as specialists for > 6 versus ≤ 6 years), with a significance level of P = 0.05. METHODS: This cross-sectional study used a virtual questionnaire covering the steps in the diagnosis and treatment of children with ADHD. The inclusion criteria were professionals who had completed their residency/specialization in pediatric neurology and clinical neurologists working in pediatric neurology. RESULTS: Among the 548 electronic invitations sent, 128 were considered valid. For all participants, the diagnosis was clinically based on the disease classification manuals. Combination treatment promotes improvement of symptoms (96.9%). Among psychostimulants, short-acting methylphenidate was the most commonly prescribed medication (85.2%). Headache was the most common side effect (77.3%). Altogether, 73.4% of the participants requested laboratory tests, 71.1% requested an electrocardiogram, and 42.2% requested an electroencephalogram. Pediatric neurologists working as specialists for ≤ 6 years had more frequent referrals to psycho-pedagogists for diagnosis (P = 0.03). CONCLUSIONS: The participants complied with clinical guidelines, emphasizing the relevance of diagnostic manuals and treatment guidelines for an eminently clinical situation and enabling uniformity in quality treatment.

Coerência em narrativas escritas de crianças com dificuldades escolares ou quecoixas mportamentais: o papel das variáveis linguísticas e a influência da escolaridade / Coherence in written narratives of children with school difficulties or behavioral complaints: the role of linguistic variables and the influence of schooling / Coherencia en narrativas escritas de niños con dificultades escolares o quejas de conducta: el papel de las variables lingüísticas y la influencia de la educación

Introdução: A narrativa escrita deve articular a ideia principal através da relação entre tema, perso-nagens e desfecho, sendo responsabilidade de quem escreve relacionar esses componentes para levá-los à coerência. A coerência consiste de uma dependência de relações macro-linguísticas (associação do tema do texto às estruturas que o compõem) e micro-linguísticas (conectivos que trarão coesão à narrativa), a fim de proporcionar ao texto o poder de interpretação. Objetivo: analisar quais variáveis linguísticas estavam relacionadas ao melhor desempenho em coerência nas narrativas escritas de escolares do ensino fundamental. Métodos: a amostra foi composta por 37 crianças (idade entre 7 ­ 11 anos) sem deficiência intelectual e/ou deficiência auditiva. Cada criança teve sua elaboração escrita classificada em adequada (coerência nível III e IV segundo instrumento utilizado) ou inadequada (coerência nível I ou II). Poste-riormente, foi analisado um conjunto de variáveis que poderiam estar relacionadas ao desempenho da narrativa, a saber: compreensão oral, vocabulário, consciência fonológica, consciência morfossintática, memória de trabalho ­ alça fonológica, leitura e escrita. Todas essas variáveis foram avaliadas através de testes padronizados. Para a análise estatística utilizou-se modelo de regressão logística. Resultados:dentre todas as habilidades linguísticas avaliadas, consciência morfossintática (p = 0,02) foi a variável significativa. Somada a estas, temos também a escolaridade (p = 0,01), porém a consciência morfossin-tática apresentou coeficiente negativo enquanto a escolaridade apresentou coeficiente positivo. Conclu-são: crianças que apresentam alteração em consciência morfossintática apresentam maiores chances de elaborarem narrativas escritas incoerentes. Já as crianças com maior grau de escolaridade, são as que possuem textos mais adequados.

Introduction: The written narrative must articulate the main idea through the relation between theme, characters and outcome, being the responsibility of those who write to relate these components to bring them to coherence. Consistency consists of a dependence on macro-linguistic relations (association of the theme of the text with the structures that make it up) and micro-linguistic (connectives that will bring cohesion to the narrative), in order to provide the text with the power of interpretation. Objective: to analyze which linguistic variables are related to the best coherence performance in the written narrati-ves of elementary schoolchildren. Methods: the sample consisted of 37 children aged 7-11 years with no intellectual or hearing deficiency. The written elaboration of each child was classified as adequate (level III and IV coherence according to the instrument used) or inadequate (level I or II coherence). A set of variables that might be related to the performance of the narrative was then analyzed, such as oral comprehension, vocabulary, phonological awareness, morphosyntactic awareness, working memory ­ phonological loop, reading, and writing. All of these variables were evaluated using standardized tests and statistical analysis was performed using a logistic regression model. Results: among all the linguistic skills evaluated, morphosyntactic awareness (p = 0.02) was the significant variable. In addition to these there was also schooling (p = 0.01), although morphosyntacic awareness showed a negative coefficient while schooling showed a positive coefficient. Conclusion: children with changes in morphosyntactic awareness have a greater chance to elaborate incoherent written narratives, whereas children with higher schooling elaborate more adequate texts.

Introducción: La narrativa escrita debe articular la idea principal a través de la relación entre tema, personajes y desenlace, siendo responsabilidad de quienes escriben relacionar estos componentes para llevarlos a la coherencia. La coherencia consiste en una dependencia de relaciones macrolingüísticas y microlingüísticas, para dotar al texto de poder de interpretación. Objetivo: analizar qué variables lingüísticas se relacionaron con un mejor desempeño en coherencia en las narrativas escritas de estudiantes de primaria. Metodos: la muestra estuvo formada por 37 niños (de 7 a 11 años). Cada niño tenía su elabo-ración escrita clasificada como adecuada (nivel de coherencia III y IV, según el instrumento utilizado) o inadecuada (nivel de coherencia I o II). Posteriormente, se analizaron un conjunto de variables que podrían estar relacionadas con el desempeño de la narrativa, a saber: escucha, vocabulario, conciencia fonológica, conciencia morfosintáctica, memoria de trabajo - bucle fonológico, lectura y escritura. Todas estas variables se evaluaron mediante pruebas estandarizadas. Para el análisis estadístico se utilizó un modelo de regresión logística. Resultados: entre todas las habilidades lingüísticas evaluadas, la conciencia morfosintáctica (p = 0,02) fue la variable significativa. Sumado a estos, también tenemos la educación (p = 0.01), pero la conciencia morfosintáctica tuvo un coeficiente negativo mientras que la educación tuvo un coeficiente positivo. Conclusión: los niños con alteración de la conciencia morfosintáctica son más propensos a desarrollar narrativas escritas incoherentes. Los niños con mayor nivel educativo, en cambio, son los que tienen los textos más adecuados.

The intellectual profile of pediatric patients with posterior cortex epilepsy.

BACKGROUND: Cognitive functioning in epileptic syndromes has been widely explored in patients with temporal lobe epilepsy (TLE), but few studies have investigated the neuropsychological profile in posterior cortex epilepsy (PCE). In this study, we investigated the presurgical intellectual profile of children and adolescents with drug-resistant PCE. METHODS: Children and adolescents diagnosed with PCE (n = 25) participated in this study. The data were obtained from medical records, with assessments carried out between the years 2003 and 2019. To compare the intellectual profile, we also included patients diagnosed with frontal (n = 26) and temporal lobe epilepsy (n = 40). The Wechsler Intelligence Scales were used for the assessment of general intelligence. RESULTS: There was an effect of the brain region on the Working Memory Index (p < 0.01), in which patients with TLE had significantly higher scores than groups with FLE (p < 0.01) and PCE (p < 0.05). We also demonstrated that patients with PCE tended to perform worse in the Processing Speed Index than patients with TLE (p = 0.055). The Full-Scale Intelligence Quotient, Verbal Comprehension, and Perceptual Reasoning indexes did not differ among the brain regions. CONCLUSIONS: Children and adolescents with PCE demonstrated significant impairment in working memory and processing speed. The pattern of cognitive dysfunction in PCE was similar to that observed in FLE, which expands the evidence of the involvement of frontoparietal networks on cognitive proficiency.

Development of language and arithmetic skills: risk and protective factors. Comparative cross-sectional study.

BACKGROUND: In a literate society, linguistic/arithmetic performance is highly valued. Based on defined risk factors, strategies for promotion of better performance can be developed. OBJECTIVE: To ascertain the risk and protective factors relating to development of language and arithmetic. DESIGN AND SETTING: Observational comparative cross-sectional study at a public elementary school in Ribeirão Preto (SP), Brazil. METHODS: A total of 66 children (41% females) attending first to fifth grades participated in this study. They were divided into two groups: G1, children classified as presenting language or arithmetic deficits; G2, average performance. Language (oral and written) and arithmetic skills were assessed through standardized tests. Variables relating to social skills, home environment resources and behavioral problems were assessed through standardized scales. Data on other variables (pre, peri and postnatal complications, maternal variables and others) were collected through interviews. The logistic regression technique with LASSO was used (α = 0.05). RESULTS: Teenage pregnancy and consumption of psychoactive substances during pregnancy or complications during pregnancy were risk factors for performance regarding arithmetic and language. Higher schooling level for the mother was a protective factor in the development of arithmetic and language. Being female and having a history of otitis were risk factors for language. Altered social skills (responsibility and civility) and complaints of inattention were risk factor for arithmetic. Adequate linguistic development was a protective factor for the development of arithmetic. CONCLUSION: The risk/protective factors included variables relating to the gestational period, mother's age when pregnant, mother's schooling, social skills, behavior and development issues.

Seizures in self-limited epilepsy with centrotemporal spikes: video-EEG documentation.

PURPOSE: Self-limited epilepsy with centrotemporal spikes, formerly called benign epilepsy with centrotemporal spikes, or rolandic epilepsy, is an age-related and well-defined epileptic syndrome. Since seizures associated with rolandic spikes are infrequent and usually occur during sleep, and repetitive or prolonged EEG recording for diagnostic purposes is not necessary for diagnosis, reports of ictal video-electroencephalographic seizures in this syndrome are rare. We aimed to show ictal video-EEG of typical rolandic seizures. METHODS: We report the ictal video-EEG recordings of two children with rolandic epilepsy who presented typical rolandic seizures during routine recording. RESULTS: Case 1: A 9-year-old boy, with normal development, had his first seizure at 8 years old, characterized by paresthesia in his left face, blocking of speech, and drooling. Carbamazepine was started with seizure control. Case 2: A 10-year-old boy, with normal development, started with focal seizures during sleep, characterized by eye and perioral deviation, and speech arrest at age of 7. He started using oxcarbazepine. Both patients underwent routine electroencephalography for electroclinical diagnosis and presented a seizure. CONCLUSION: Although self-limited epilepsy with centrotemporal spikes is a very common epileptic syndrome, seizure visualization is very difficult, and these videos may bring didactical information for recognition of this usual presentation of benign childhood focal epilepsy.

Impact of epilepsy surgery on quality of life and burden of caregivers in children and adolescents.

OBJECTIVE: The objective of this study was to analyze the impact of pediatric epilepsy surgery on the quality of life (QOL), determining whether patients improve, worsen, or maintain their preoperative patterns, as it relates to the burden of caregivers, as well as evaluating potential related factors, from both the children and caregivers perspectives. MATERIAL AND METHODS: This is a retrospective study of children and adolescents who underwent epilepsy surgery and were evaluated through clinical data, videoelectroencephalogram (V-EEG), neuroimaging findings, neuropsychological testing, and aspects of QOL. These assessments were performed prior to surgery and after six months and two years of follow-up. Quality of life was assessed with epilepsy-specialized questionnaires, namely Questionnaire health-related quality of life for children with epilepsy (QVCE-50), Autoquestionnaire Qualité de Vie Enfant Image Scale (AUQUEI), Quality of life in epilepsy inventory for adolescents (QOLEI-AD-48); and burden of caregivers with Burden Interview - ZARIT scale. Postoperative changes in QVCE-50 were quantified using measures of the analysis of variance (ANOVA MR) for comparison of the difference between the three times of the scale and domains. RESULTS: Fifty patients were enrolled. Of these, 27 (54%) were male, with a mean age at surgery of 8.2 years (range: 1-18 years). Thirty-five patients (70%) were Engel I and one was Engel II (2%) at six months of follow-up, whereas 28 (56%) were Engel I and 32 (64%) were Engel I or II at two years of follow-up. Preoperatively, 21 (42%) presented with moderate or severe intellectual disability. Postoperative cognitive evaluations at the two-year follow-up showed 18 (36%) maintained similar deficits. The QVCE-50 showed postoperative improvement in the two-year follow-up period, but not at six months after surgery. Postoperative improvements were associated mainly with better seizure outcome. Autoperception evaluations were limited because of the clinical and cognitive severity of patients. The burden of caregivers was quoted as mild to moderate and remained unchanged postoperatively. CONCLUSIONS: Children and adolescents with surgically treated epilepsy reach a good seizure outcome, stabilize in intellectual and adaptive functions, and have an increase in QOL, from the caregiver's perspective. Nevertheless, their burden remains unchanged. Seizure outcome is the main factor for improvement in the QOL. The upgrading of structured questionnaires and QOL instruments specific to pediatric epilepsy can be helpful to assess patient- and caregiver-reported surgical outcomes, allowing for better planning of therapeutic approaches.

Left hemispherectomy in older children and adolescents: outcome of cognitive abilities.

PURPOSE: Hemispherectomy is an effective treatment option for pharmacoresistant epilepsy. Nevertheless, when high cortical functions are at risk during the presurgical evaluation, especially for older children, and for the left hemisphere, despite good seizure outcome, the anticipated decrease of cognitive functions may prevent a decision to perform surgery. The objective of this study is to report the cognitive outcome, based on verbal and performance intelligence skills, in a series of older children and adolescents who underwent left hemispherectomy, analyzing the risks (residual cognitive deficit) and benefits (seizure reduction) of surgery. METHODS: We retrospectively analyzed pre- and postoperative clinical and neuropsychological data from our patients who underwent left hemispherectomy, aged between 6 and 18 years. RESULTS: We included 15 patients, with a mean follow-up of 3.1 years, 12 patients (80%) were Engel I, and the other three were classified as Engel II, III, and IV. Nine patients were tested by Wechsler Scales of Intelligence; postsurgically all but one kept the same intellectual levels; verbal intelligence quotient (VIQ) remained unchanged in 13 and improved in one, whereas performance intelligence quotient (PIQ) decreased in four patients. Both Total Vineland and communication scores of Vineland Adaptive Behavior Scales were obtained in six patients: in all, scores were classified as deficient adaptive functioning pre- and postoperatively, remaining unchanged. CONCLUSION: The evaluation of the remaining intellectual abilities after left hemispherectomy in older children and adolescents is useful to discuss the risks and benefits of this surgery, enabling better and safer decisions regarding surgical indications and timing.

Defecation reflex seizures: a case report with long-term VEEG monitoring, neuroimaging and comprehensive epilepsy evaluation.

Reflex seizures are consistently elicited by a specific afferent sensory stimulus or an activity undertaken by the patient. Among many known stimuli, defecation has rarely been reported. We describe the case of a child with reflex seizures triggered by defecation, considering the diagnostic challenge, epilepsy evaluation with video-EEG monitoring, as well as impact on neuropsychology, behaviour and quality of life. The child was a 10-year-old boy with seizure onset at age four with epilepsy diagnosis established one and a half years later. Seizures were focal with impaired awareness triggered by defecation. Video-EEG and structural and functional neuroimaging were performed and all pointed to the left temporal region. The patient became seizure-free with carbamazepine and valproic acid. Neuropsychological and quality of life assessments suggested global impairment, both before and after seizure control. This is the third case of epilepsy induced by defecation reported in the literature. The rarity of this entity may be a diagnostic challenge and postpone specific treatment. Reporting of cases of defecation reflex epilepsy may provide a better understanding of its physiopathology and optimize effective treatment, avoiding cognitive, behavioural and poor social consequences. [Published with video sequence].

Refractory epilepsy in children with brain tumors. The urgency of neurosurgery.

In order to verify indications for surgery, 27 patients with refractory epileptic seizures and brain tumor, aged up to 19 years at the time of surgery, were studied between 1996 and 2013 and followed up for at least one year. The mean interval between the onset of seizures and the diagnosis of the tumor was 3.6 years, and from diagnosis to the surgery, 18 months. The location of the tumor was in the temporal lobe in 16, with ganglioglioma and dysembryoplastic neuroepithelial tumors being the most frequent. Among the patients, 92.5% and 90.4% were seizure-free in the first and fifth year after surgery, respectively. Twelve of 16 children were successful in becoming drug-free, with complete withdrawal by 3.2 years. Surgery proved to be potentially curative and safe in these cases, suggesting that the tumor diagnosis and surgery cannot be postponed.

Refractory epilepsy in children with brain tumors. The urgency of neurosurgery / Epilepsia refratária em crianças com tumores cerebrais. A urgência de neurocirurgia

ABSTRACT In order to verify indications for surgery, 27 patients with refractory epileptic seizures and brain tumor, aged up to 19 years at the time of surgery, were studied between 1996 and 2013 and followed up for at least one year. The mean interval between the onset of seizures and the diagnosis of the tumor was 3.6 years, and from diagnosis to the surgery, 18 months. The location of the tumor was in the temporal lobe in 16, with ganglioglioma and dysembryoplastic neuroepithelial tumors being the most frequent. Among the patients, 92.5% and 90.4% were seizure-free in the first and fifth year after surgery, respectively. Twelve of 16 children were successful in becoming drug-free, with complete withdrawal by 3.2 years. Surgery proved to be potentially curative and safe in these cases, suggesting that the tumor diagnosis and surgery cannot be postponed.

RESUMO A fim de verificar os aspectos da indicação cirúrgica, vinte e sete pacientes com epilepsia refratária secundária a tumor cerebral, com idade de até 19 anos na cirurgia, operados entre 1996 e 2013 e seguidos por pelo menos um ano, foram estudados. O intervalo médio entre o início das crises e o diagnóstico do tumor foi de 3,6 anos, e deste para a cirurgia, 18 meses. A localização do tumor foi lobo temporal em 16, sendo ganglioglioma e DNET os tipos mais frequentes. Entre os pacientes, 92,5% e 90,4% estavam livres de crises no primeiro e no quinto ano após a cirurgia, respectivamente. Doze de 16 crianças obtiveram sucesso na retirada de drogas, com a média de tempo de 3,2 anos após o procedimento. A cirurgia provou ser potencialmente curativa e segura nestes casos, o que sugere que perante o diagnóstico de tumor esta não pode ser adiada.

Hemispheric surgery for refractory epilepsy in children and adolescents: outcome regarding seizures, motor skills and adaptive function.

PURPOSE: The aim of the study was to report the seizure outcome, motor skills and adaptive motor functions in a series of children and adolescents who underwent hemispheric surgery, analysing the risk-benefits of surgery. METHODS: The clinical course, seizure and motor function outcomes of 15 patients who underwent hemispheric surgery were reviewed. RESULTS: The mean age at surgery was 9.5, with 1-9 years follow-up. The underlying pathologies were Rasmussen encephalitis, vascular disorders, and hemimegalencephaly. All the patients presented with severe epilepsy and different degrees of hemiparesis, although motor functionality was preserved in 80% of the patients. At last follow-up, 67% were seizure free, and 20% rarely experienced seizures. Antiepileptic drugs were reduced in 60%, and complete withdrawal from such drugs was successful in 20% of the patients. The motor outcome following the surgery varied between the patients. Despite the motor deficit after surgery, the post-operative motor function showed unchanged for gross motor function in most (60%), while 27% improved. Similar results were obtained for the ability to handle objects in daily life activities. Sixty percent of the children were capable of handling objects, with somewhat reduced coordination and/or motor speed. CONCLUSION: Pre-surgical motor function continues to play a role in the pre-surgical evaluation process in order to provide a baseline for outcome. Hemispheric surgery, once regarded as a radical intervention and last treatment resource, may become routinely indicated for refractory hemispheric epilepsy in children and adolescents, with oftentime favourable motor outcomes.

Gastaut type idiopathic childhood occipital epilepsy.

Gastaut type idiopathic childhood occipital epilepsy is an uncommon epileptic syndrome characterised by frequent seizures, most commonly presenting as elementary visual hallucinations or blindness. Other occipital (non-visual) symptoms may also occur. Interictal EEG typically shows occipital paroxysms, often with fixation-off sensitivity. Ictal EEG is usually characterised by interruption by paroxysms and sudden appearance of low-voltage, occipital, fast rhythm and/or spikes. Despite well described clinical and EEG patterns, to our knowledge, there are very few reports in the literature with video-EEG recording of either seizure semiology or fixation-off phenomena. We present a video-EEG recording of a 12-year-old girl with Gastaut type epilepsy, illustrating the interictal and ictal aspects of this syndrome. Our aim was to demonstrate the clinical and neurophysiological pattern of a typical seizure of Gastaut type epilepsy, as well as the fixation-off phenomena, in order to further clarify the typical presentation of this syndrome. [Published with video sequences].