Cognitive impact in children with “benign” childhood focal epilepsy with centrotemporal spikes

Background Cognitive alterations are associated with benign childhood focal epilepsy with centrotemporal spikes (BCECTS) including aspects of executive functions. Objectives This study presents the performance profile on attention and executive function tests of fifty-eight children (BCECTS, n = 30 and controls, n = 28) aged 8-13 years. Methods The following tools were employed: Vocabulary and Block Design subtests from the Wechsler Intelligence Scale for Children III, Stroop Test, Modified Card Sorting Test, Controlled Oral Word Association – FAS and Tower of London. Results Children with BCECTS presented average IQ measure, although their performance was statistically worse when compared to the control group. Children with BCECTS showed significantly lower performance compared to the control group in the following variables: total number of recollected words on the oral fluency test, total number of categories, categorization effect and total number of errors in MCST; and execution time for the Stroop Test Card 1. After controlling for the IQ effect, the total number of errors in the MCST did not show any significant difference between the groups. Discussion Children with BCECTS showed lower performance in attention and executive functions when compared to healthy children. The results suggest that the concept of “benign” BCECTS should be reconsidered

The Perceived Social Stigma of People with Epilepsy with regard to the Question of Employability

Objective. To evaluate the perceived social stigma of people with epilepsy with regard to the question of employability. Methods. A structured questionnaire was given to two groups of people with chronic diseases: those with epilepsy (study group) and those with heart disease (control group). The questions concerned employability. Results. Having epilepsy was more strongly associated with higher unemployment rates (p<0.0001); job layoffs (p=0.001); being unfit to work (p<0.0001); feeling shame for having the disease (p=0.014); absence of partners (p=0.026); and depression (p=0.004). The tendency to hide their disease from their employers was similar for the two groups. Conclusion. The age discrepancy between groups was an important limiting factor of this study. However, despite the limited number of participants and the age difference between the groups, there is no impediment in stating that people with epilepsy show high rates of unemployment, depression, and stigma

Sequential motor task (Luria's Fist-Edge-Palm Test) in children with benign focal epilepsy of childhood with centrotemporal spikes Tarefa motora sequencial (Teste de Lúria punho-lado-palma) em crianças com epilepsia focal benigna da infância com descarga centrotemporal

<p id="para1">This study evaluated the sequential motor manual actions in children with benign focal epilepsy of childhood with centrotemporal spikes (BECTS) and compares the results with matched control group, through the application of Luria's fist-edge-palm test. The children with BECTS underwent interictal single photon emission computed tomography (SPECT) and School Performance Test (SPT). Significant difference occurred between the study and control groups for manual motor action through three equal and three different movements. Children with lower school performance had higher error rate in the imitation of hand gestures. Another factor significantly associated with the failure was the abnormality in SPECT. Children with BECTS showed abnormalities in the test that evaluated manual motor programming/planning. This study may suggest that the functional changes related to epileptiform activity in rolandic region interfere with the executive function in children with BECTS.</p><br><p id="para2">Esse estudo avaliou a&#231;&#245;es motoras manuais sequenciais em crian&#231;as com epilepsia focal benigna da inf&#226;ncia com descarga centrotemporal (EBICT) e comparou os resultados com o grupo controle pareado, atrav&#233;s do teste de L&#250;ria (punho-lado-palma). As crian&#231;as com EBICT realizaram <italic>single photon emission computed tomography</italic> (SPECT) interictal e Teste de Desempenho Escolar (TDE). Foram encontradas diferen&#231;as significativas entre os dois grupos nas atividades motoras de tr&#234;s movimentos iguais e tr&#234;s movimentos diferentes. As crian&#231;as com piores resultados no TDE e com SPECT alterado apresentaram mais erros no teste de imita&#231;&#227;o manual. Crian&#231;as com epilepsia fracassaram nos testes de avalia&#231;&#227;o motora que envolvem programa&#231;&#227;o/planejamento. Esse estudo sugere que mudan&#231;as funcionais relacionadas &#224; atividade epileptiforme na regi&#227;o rol&#226;ndica interfere com as fun&#231;&#245;es executivas de crian&#231;as com EBICT.</p

Sequential motor task (Luria's Fist-Edge-Palm Test) in children with benign focal epilepsy of childhood with centrotemporal spikes

This study evaluated the sequential motor manual actions in children with benign focal epilepsy of childhood with centrotemporal spikes (BECTS) and compares the results with matched control group, through the application of Luria's fist-edge-palm test. The children with BECTS underwent interictal single photon emission computed tomography (SPECT) and School Performance Test (SPT). Significant difference occurred between the study and control groups for manual motor action through three equal and three different movements. Children with lower school performance had higher error rate in the imitation of hand gestures. Another factor significantly associated with the failure was the abnormality in SPECT. Children with BECTS showed abnormalities in the test that evaluated manual motor programming/planning. This study may suggest that the functional changes related to epileptiform activity in rolandic region interfere with the executive function in children with BECTS

Impact of benign childhood epilepsy with centrotemporal spikes (BECTS) on school performance

BECTS represents the vast majority of childhood focal epilepsy. Owing to the age peculiarity of children who suffer from this disease, i.e., school-going age of between 6 and 9 years, the condition is often referred to as a school disorder by parents and teachers. Objective: The aim of this study was to evaluate the academic performance of children with BED'S, according to the clinical and electroencephalographic ILAE criteria, and compare the results of neuropsychological tests of language and attention to the frequency of epileptic discharges. Methods: The performances of 40 school children with BED'S were evaluated by applying a school performance test (SBT), neuropsychological tests (WISC and Trail-Making), and language tests (Illinois Test Psycholinguistic Abilities - ITPA - and Staggered Spondaic Word - SSW). The same tests were applied in the control group. Results: Children with BED'S, when compared to those in the control group, showed lower scores in academic performance (SPT), digits and similarities subtests of WISC, auditory processing subtest of SSW, and ITPA - representational and automatic level. The study showed that epileptic discharges did not influence the results. Conclusion: Children with BED'S scored significantly lower scores in tests on academic performance, when compared with those in the control group probably due to executive dysfunction. (C) 2011 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.FAPESP (Fundo de Amparo a Pesquisa do Estado de So Paulo - ClnAPCe (Cooperacao Interinstitucional de Apoio a Pesquisas sobre o Cerebro)FAPESP (Fundo de Amparo a Pesquisa do Estado de So Paulo ClnAPCe (Cooperacao Interinstitucional de Apoio a Pesquisas sobre o Cerebro

Clinical management and diagnosis of CLN2 disease: consensus of the Brazilian experts group

Neuronal ceroid lipofuscinosis type 2 (CLN2) is a rare neurodegenerative genetic disease that affects children in early life. Its classic form is rapidly progressive, leading to death within the first 10 years. The urge for earlier diagnosis increases with the availability of enzyme replacement therapy. A panel of nine Brazilian child neurologists combined their expertise in CLN2 with evidence from the medical literature to establish a consensus to manage this disease in Brazil. They voted 92 questions including diagnosis, clinical manifestations, and treatment of the disease, considering the access to healthcare in this country. Clinicians should suspect CLN2 disease in any child, from 2 to 4 years old, with language delay and epilepsy. Even though the classic form is the most prevalent, atypical cases with different phenotypes can be found. Electroencephalogram, magnetic resonance imaging, molecular and biochemical testing are the main tools to investigate and confirm the diagnosis. However, we have limited access to molecular testing in Brazil, and rely on the support from the pharmaceutical industry. The management of CLN2 should involve a multidisciplinary team and focus on the quality of life of patients and on family support. Enzyme replacement therapy with Cerliponase α is an innovative treatment approved in Brazil since 2018; it delays functional decline and provides quality of life. Given the difficulties for the diagnosis and treatment of rare diseases in our public health system, the early diagnosis of CLN2 needs improvement as enzyme replacement therapy is available and modifies the prognosis of patients