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Objective: We describe a multicenter experience with vagus nerve stimulator implantation in pediatric patients with drug-resistant epilepsy. Our goal was to assess vagus nerve stimulation efficacy and identify potential predictors of favorable outcome. Methods: This is a retrospective study. Inclusion criteria: ≤18 years at time of vagus nerve stimulator implantation, at least 1 year of follow-up. All patients were previously found to be unsuitable for an excisional procedure. Favorable clinical outcome and effective vagus nerve stimulation therapy were defined as seizure reduction >50%. Outcome data were reviewed at 1, 2, 3, and 5 years after vagus nerve stimulator implantation. Fisher exact test and multiple logistic regression analysis were employed. Results: Eighty-nine patients met inclusion criteria. Responder rate (seizure frequency reduction >50%) at 1-year follow-up was 25.8% (4.5% seizure-free). At last follow-up, 31.5% had a favorable outcome and 5.2% were seizure free. The only factor significantly predicting favorable outcome was time to vagus nerve stimulator implantation, with the best outcome achieved when vagus nerve stimulator implantation was performed within 3 years of seizure onset. Implantation between 3 and 5 years after epilepsy onset correlated with better long-term seizure freedom (13.3% at T5). Overall, 65.2% of patients evidenced improved quality of life at last follow-up. However, 12.4% had adverse events, but most were mild and disappeared after 3-4 months. Conclusions: Early vagus nerve stimulator implantation within 5 years of seizure onset was the only predictor of favorable clinical outcome in pediatric patients. Improved quality of life and a low incidence of significant adverse events were observed.

Background Epilepsy is a main feature of Mowat Wilson Syndrome (MWS), a congenital malformation syndrome caused by ZEB2 variants. The aim of this study was to investigate the long-term evolution of the electroclinical phenotype of MWS in a large population. Methods Forty-individuals with a genetically confirmed diagnosis were enrolled. Three age groups were identified (t1 = 0–4; t2 = 5–12; t3 = >13 years); clinical data and EEG records were collected, analyzed, and compared for age group. Video-EEG recorded seizures were reviewed. Results Thirty-six of 40 individuals had epilepsy, of whom 35/35 aged >5 years. Almost all (35/36) presented focal seizures at onset (mean age at onset 3.4 ± 2.3 SD) that persisted, reduced in frequency, in 7/22 individuals after the age of 13. Absences occurred in 22/36 (mean age at onset 7.2 ± 0.9 SD); no one had absences before 6 and over 16 years old. Paroxysmal interictal abnormalities in sleep also followed an age-dependent evolution with a significant increase in frequency at school age (p = 0.002) and a reduction during adolescence (p = 0.008). Electrical Status Epilepticus during Sleep occurred in 14/36 (13/14 aged 5–13 years old at onset). Seven focal seizure ictal video-EEGs were collected: all were long-lasting and more visible clinical signs were often preceded by prolonged electrical and/or subtle (erratic head and eye orientation) seizures. Valproic acid was confirmed as the most widely used and effective drug, followed by levetiracetam. Conclusions Epilepsy is a major sign of MWS with a characteristic, age-dependent, electroclinical pattern. Improvement with adolescence/adulthood is usually observed. Our data strengthen the hypothesis of a GABAergic transmission imbalance underlying ZEB2-related epilepsy.

Objective: We describe a multicenter experience with VNS implantation in pediatric patients with epileptic encephalopathy. Our goal was to assess VNS efficacy and identify potential predictors of favorable outcome. Methods: This was a retrospective study. Inclusion criteria were: ≤18 years at the time of VNS implantation and at least one year of follow-up. All patients were non-candidates for excisional procedures. Favorable clinical outcome and effective VNS therapy were defined as seizure reduction >50%. Outcome data were reviewed at one, two, three and five years after VNS implantation. Fisher's exact test, Kaplan-Meier and multiple logistic regression analysis were employed. Results: Twenty-seven patients met inclusion criteria. Responder rate (seizure frequency reduction ≥ 50%) at one-year follow-up was 25.9%, and 15.3% at last follow-up visit. The only variable significantly predicting favorable outcome was time to VNS implantation, with the best outcome achieved when VNS implantation was performed within five years of seizure onset (overall response rate of 83.3% at one year of follow-up and 100% at five years). In total, 63% of patients evidenced improved QOL at last follow-up visit. Only one patient exited the study due to an adverse event at two years from implantation. Significance: Early VNS implantation within five years of seizure onset was the only predictor of favorable clinical outcome in pediatric patients with epileptic encephalopathy. Improved QOL and a very low incidence of adverse events were observed.

Journal of neurology, neurosurgery, and psychiatry 92(10), 1044-1052 (2021). doi:10.1136/jnnp-2020-325932, Published by BMJ Publishing Group, London

Purpose: The aim of this study is to describe demographic data, semiology and etiology in a pediatric population with status epilepticus (SE) and refractory SE (RSE). Method: We retrospectively reviewed patients with the following inclusion criteria: i) age between two months and eighteen years; ii) SE diagnosis; iii) admission from January 2001 to December 2016; iv) available clinical data. Results: We enrolled 124 patients. Mean and median age was 4.6 ± 4.2 years and 3.3 [1.2-7.5] years respectively. SE had a “de novo” onset in 66.9%. Focal convulsive-SE was the most common semiology (50.8%) whilst generalised (32.3%) and nonconvulsive-SE (NCSE) (16.9%) were less represented. Some etiologies showed a different age distribution: febrile in youngest age (p = 0.002, phi 0.3) and idiopathic-cryptogenic in older children (p = 0.016, phi 0.2). A statistical significance correlation was detected between semiology and etiology (p < 0.001, Cramer's V 0.4), chemotherapy and NCSE (n = 6/21 vs 3/103, p < 0.001) as well as PRES and NCSE (n = 7/21 vs 5/103, p < 0.001). Only 17.7% had a RSE. No correlation was found in demographic and clinical data, but NCSE, acute and idiopathic-cryptogenic etiologies were more frequently associated to RSE. Encephalitis was the most common diagnosis in acute etiologies whereas unknown epilepsy in idiopathic-cryptogenic group. Conclusion: Most of our findings were previously described however we found a significant role of non-antiepileptic treatments (chemotherapy-dialysis) and comorbidity (PRES) determining acute etiology and NCSE. Acute (mostly encephalitis), idiopathic-cryptogenic (mainly unknown-epilepsy) and NCSE were frequently detected in RSE. In the above mentioned conditions a high level of suspicion was recommended.

The building sector has a significant environmental impact and environmental rating systems could to indicate some guidelines for achieving sustainable developments. There are several systems, but the certifications in general are adjusted to peculiar conditions of developer country. The Brazilian bank Caixa Economica Federal created and is responsible to assign the Selo Casa Azul (“Blue House Seal”). This certification apparently is the most applicable to Brazilian conditions. However, there are few studies about the procedures for analysis of housing projects. The aim of this study is to analyse the adequacy of typical housing developments with respect to Selo Casa Azul criteria by verifying existing facilities and considering the limitations of real projects. This research analyses 13 developments certified by the bank and seven new projects in Caxias do Sul, a city in southern Brazil. As a result, we found that the Selo Casa Azul constitutes a viable tool, demonstrating the relative ease of application. We verify that some criteria were not present in any of these projects and that alignment with Selo's criteria depends on a company's strategy, no matter the economic standard of its buildings. Lastly, we observe that most actions needed to reach unsatisfied criteria may be solved in the design stage, and these modifications do not require large investments. This study aims to contribute to the discussion about sustainable construction in Brazil.

O setor da construcao civil se caracteriza como uma atividade de reconhecida importância economica, mas que, no entanto, gera impactos significativos. Neste contexto, a incorporacao de estrategias as quais proporcionem o desenvolvimento de empreendimentos mais sustentaveis e essencial para a minimizacao destes impactos e para que se propicie uma maior qualidade de vida as comunidades envolvidas. As certificacoes ambientais indicam diretrizes que podem garantir a realizacao de um empreendimento mais sustentavel. O Selo Casa Azul, concedido pela Caixa Economica Federal desde 2011, se trata de um sistema de classificacao desenvolvido para a realidade brasileira em seus aspectos regionais diversos, e no qual se consideram, inclusive, aspectos relacionados ao desempenho termico de edificacoes. Atraves do presente estudo se buscou analisar empreendimentos habitacionais verticais tipicos executados em Caxias do Sul, RS, subsidiados por diferentes fontes de recursos, quanto ao atendimento a estes criterios e se haveria relacao entre o seu padrao economico com a facilidade ou limitacao de atendimento. Como resultado da avaliacao se verificou certa dificuldade em se atender aos criterios e que as opcoes das empresas para cada um dos empreendimentos foram mais determinantes para se atender ou nao um criterio do que o proprio padrao economico.

A 45-year-old male noticed progressive weakness of the right lower limb with gait disturbance. Over the following months, motor deficits worsened, spreading to the right upper limb. Electromyography showed active denervation in the upper and lower limb muscles. A diagnosis of amyotrophic lateral sclerosis (ALS) was made. About 2 years after symptom onset, gradual improvement occurred. Cerebrospinal fluid analysis performed about 3 years after the beginning of symptoms identified Cronobacter sakazakii. Since no other possible causes were identified, we suggest that an almost completely reversible ALS-like syndrome had been triggered by Cronobacter infection in our immunocompetent patient.

As nocoes de sustentabilidade, quando aplicadas juntamente ao espaco urbano, podem ser relacionadas a capacidade das estruturas existentes em atender satisfatoriamente as demandas sociais geradas pelo crescimento populacional, dado muitas vezes atraves de processos de ocupacao desordenada e onde se faz necessaria a adocao de novos modelos de planejamento. A partir de conceitos de sustentabilidade urbana em aspectos fisicos e sociais, atribuidos pela existencia de infraestrutura local, o presente trabalho se propoe a avaliar dois bairros da cidade de Caxias do Sul/RS no que se refere ao atendimento das comunidades em algumas necessidades primeiras. Os Bairros Santa Catarina e Santa Fe foram selecionados para este estudo por se tratarem dos dois bairros mais populosos da cidade, mas que, contudo, sao divergentes em suas demais caracteristicas. A pesquisa foi realizada durante o desenvolvimento de atividade pratica supervisionada do curso de graduacao em Engenharia Civil, atraves do levantamento e analise de dados censitarios e de outros obtidos junto ao poder publico municipal, podendo ser ampliada e aplicada aos demais bairros com vistas a definicao de estrategias que possam atribuir um carater mais sustentavel a estes locais. Como resultado, foi possivel se estabelecer um comparativo entre as duas comunidades, que mesmo apresentando numeros aproximados em termos populacionais e de infraestrutura revelaram-se diferentes em niveis de sustentabilidade em razao das demandas sociais diversas que competem a uma populacao que em geral expoe um menor nivel socioeconomico. Deste modo, se pode viabilizar o estabelecimento de politicas publicas que proporcionem a melhoria do nivel de sustentabilidade, de forma a satisfazer as carencias diagnosticadas.

Submitted by Fabricia Fialho Reginato (fabriciar) on 2015-06-25T22:55:10Z No. of bitstreams: 1 DanielaFastofski.pdf: 3816296 bytes, checksum: c32c92056ea3373431fc7b52e7d514aa (MD5) Made available in DSpace on 2015-06-25T22:55:10Z (GMT). No. of bitstreams: 1 DanielaFastofski.pdf: 3816296 bytes, checksum: c32c92056ea3373431fc7b52e7d514aa (MD5) Previous issue date: 2014-11-21 Nenhuma A indústria da construção civil apresenta reconhecida importância para a economia global, ao mesmo tempo em que se caracteriza como uma atividade geradora de impactos ambientais e sociais significativos. A incorporação de estratégias que proporcionem empreendimentos mais sustentáveis é essencial para que se possa contribuir com a minimização dos impactos e para a maior qualidade de vida das comunidades envolvidas. As certificações ambientais indicam diretrizes que podem garantir a realização de um empreendimento mais sustentável. O Selo Casa Azul, concedido pela Caixa Econômica Federal, se trata de um sistema de classificação desenvolvido para a realidade brasileira, considerando os aspectos regionais. Através do presente estudo se buscou verificar a viabilidade de aplicação desta metodologia. Os objetivos específicos são avaliar empreendimentos habitacionais verticais quanto a sua adequação ao Selo Casa Azul, identificar limitações existentes para atender aos requisitos e as relações com diferentes padrões construtivos e quais destes poderiam vir a ser mais facilmente atendidos a partir de alterações simples. A investigação está embasada em estudo de casos de empreendimentos habitacionais típicos de Caxias do Sul, RS subsidiados por diferentes fontes de recursos. Como resultado, verificou-se que o Selo Casa Azul demonstra uma relativa facilidade de aplicação sendo, portanto, uma ferramenta viável, apesar de terem sido necessários esclarecimentos junto à Caixa. No que concerne ao padrão construtivo, alguns critérios podem ser mais facilmente apreendidos quando não se tratam de recursos limitados, tendo sido observado, contudo, que parte da dificuldade de adequação deve-se às práticas das empresas e da própria construção civil. A partir dos resultados verificou-se ainda que nenhum dos empreendimentos avaliados atenderia ao Selo já que todos deixam de satisfazer a algum critério obrigatório, bem como nenhum atingiu ao menos 50% do total de critérios propostos. Dos 53 critérios, cerca de 25% não foram atendidos por todos os empreendimentos. The construction industry has great importance to the global economy, while it is characterized as an activity that generates significant environmental and social impacts. Incorporating strategies that provide more sustainable buildings is essential so that one can help to minimize these impacts and greater quality of life to the communities involved. Environmental certifications indicates guidelines that can guarantee the achievement of a more sustainable development. The Selo Casa Azul (Blue House Seal), awarded by the Brazilian federal bank Caixa, it’s a classification system developed for the brazilian reality considering regional aspects. This study was oriented to verify the feasibility of applying this certification. The specific objectives are analyze vertical housing developments as its suitability for the Selo Casa Azul, identify existing limitations to meet the requirements and their correlation to different construction standards and which of these could become more easily met from simple changes. The research is based on case studies in typical housing developments in Caxias do Sul, Brazil subsidized by different resources. As a result, it was found that the Selo Casa Azul demonstrates the relative ease of application, therefore, a viable tool, although necessary clarifications from bank’ staff. Concerning the construction standards some criteria may be more easily seized when the resources are not limited, however, it was observed that part of difficulty of fitness is due to the practices of companies and the construction itself. From the results it was also verified that none of the analyzed developments attend the Seal, since all fail to satisfy a mandatory criterion, and none has reached at least 50 % of total proposed criteria. Of the 53 criteria, about 25 % were not attended by all of the developments.