Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria.

BACKGROUND Phenylketonuria (PKU) is an inborn error of metabolism caused by mutations in the phenylalanine hydroxylase (PAH) gene. When untreated, PKU leads to a significant intellectual deficiency. Although early initiation of dietary therapy allows normal cognitive development, low adherence to treatment may result in neuropsychological deficits, including attention problems. This study was performed to evaluate emotional and behavioral problems in early-treated children and adolescents with PKU using the Child Behavior Checklist - CBCL/6-18 answered by parents. MATERIAL AND METHODS The study included 36 PKU patients. The mean scores of internalizing, externalizing, and total problems, syndrome scales, and DSM-IV-oriented scales of patients were compared with those of controls. An analysis to evaluate the importance of adherence to treatment and presence of intellectual disability was also performed. RESULTS There were no significant differences between patients and controls for almost all CBCL/6-18 scales, with the exception of the Attention Problem Scale - CBCL-APS. The mean (±SD) of the CBCL-APS scores of patients (7.86±5.33) was considerably higher than the mean of the controls (6.07±4.37; p=0.016), but not different from the mean of a matched control subsample (6.69±4.46; p=0.316). The difference between the mean of the scores of DSM-IV/ADHD scale of patients (6.72±4.07) and controls (5.73±3.56; p=0.102) was not significant. Non-adherence to treatment and intellectual disability had a negative impact on both CBCL-APS and DSM-IV/ADHD scale scores. CONCLUSIONS Our findings indicate a significant prevalence of parents' complaints of attention problems and hyperactivity in non-adherent to treatment and intellectually low performing patients with PKU.

Sialorrhea in children with cerebral palsy / Sialorreia em crianças com paralisia cerebral

Abstract Objective: To review the literature on sialorrhea in children with cerebral palsy. Source of data: Non-systematic review using the keywords "sialorrhea" and "child" carried out in the PubMed®, LILACS®, and SciELO® databases during July 2015. A total of 458 articles were obtained, of which 158 were analyzed as they were associated with sialorrhea in children; 70 had content related to sialorrhea in cerebral palsy or the assessment and treatment of sialorrhea in other neurological disorders, which were also assessed. Data synthesis: The prevalence of sialorrhea is between 10% and 58% in cerebral palsy and has clinical and social consequences. It is caused by oral motor dysfunction, dysphagia, and intraoral sensitivity disorder. The severity and impact of sialorrhea are assessed through objective or subjective methods. Several types of therapeutic management are described: training of sensory awareness and oral motor skills, drug therapy, botulinum toxin injection, and surgical treatment. Conclusions: The most effective treatment that addresses the cause of sialorrhea in children with cerebral palsy is training of sensory awareness and oral motor skills, performed by a speech therapist. Botulinum toxin injection and the use of anticholinergics have a transient effect and are adjuvant to speech therapy; they should be considered in cases of moderate to severe sialorrhea or respiratory complications. Atropine sulfate is inexpensive and appears to have good clinical response combined with good safety profile. The use of trihexyphenidyl for the treatment of sialorrhea can be considered in dyskinetic forms of cerebral palsy or in selected cases.

Resumo Objetivo: Revisar a literatura referente à sialorreia em crianças com paralisia cerebral. Fonte de dados: Revisão não sistemática com as palavras-chave "sialorreia"; e "criança" feita nas bases de dados Pubmed®, Lilacs® e Scielo® em julho de 2015. Foram recuperados 458 artigos, 158 foram analisados por terem relação com sialorreia em crianças, foram aproveitados 70 com conteúdo relativo à sialorreia na paralisia cerebral ou à avaliação e ao tratamento da sialorreia em outros distúrbios neurológicos. Síntese dos dados: A sialorreia tem prevalência entre 10% e 58% na paralisia cerebral e implica consequências clínicas e sociais. É causada por disfunção motora oral, disfagia e distúrbio da sensibilidade intraoral. A gravidade e o impacto da sialorreia são avaliados por meio de métodos objetivos ou subjetivos. Estão descritas diversas formas de manejo terapêutico: treino para consciência sensorial e habilidades motoras orais, terapia farmacológica, injeção de toxina botulínica e tratamento cirúrgico. Conclusões: O tratamento mais eficaz e que aborda a causa da sialorreia nas crianças com paralisia cerebral é o treino para consciência sensorial e habilidades motoras orais, feito por um fonoaudiólogo. Injeção de toxina botulínica e o uso de anticolinérgicos têm efeito transitório e são auxiliares ao tratamento fonoaudiológico ou devem ser considerados nos casos de sialorreia moderada a grave ou com complicações respiratórias. O sulfato de atropina tem baixo custo e parece ter boa resposta clínica com bom perfil de segurança. O uso de triexifenidil para o tratamento da sialorreia pode ser considerado nas formas discinéticas de paralisia cerebral ou em casos selecionados.

Sialorrhea in children with cerebral palsy.

OBJECTIVE: To review the literature on sialorrhea in children with cerebral palsy. SOURCE OF DATA: Non-systematic review using the keywords "sialorrhea" and "child" carried out in the PubMed®, LILACS®, and SciELO® databases during July 2015. A total of 458 articles were obtained, of which 158 were analyzed as they were associated with sialorrhea in children; 70 had content related to sialorrhea in cerebral palsy or the assessment and treatment of sialorrhea in other neurological disorders, which were also assessed. DATA SYNTHESIS: The prevalence of sialorrhea is between 10% and 58% in cerebral palsy and has clinical and social consequences. It is caused by oral motor dysfunction, dysphagia, and intraoral sensitivity disorder. The severity and impact of sialorrhea are assessed through objective or subjective methods. Several types of therapeutic management are described: training of sensory awareness and oral motor skills, drug therapy, botulinum toxin injection, and surgical treatment. CONCLUSIONS: The most effective treatment that addresses the cause of sialorrhea in children with cerebral palsy is training of sensory awareness and oral motor skills, performed by a speech therapist. Botulinum toxin injection and the use of anticholinergics have a transient effect and are adjuvant to speech therapy; they should be considered in cases of moderate to severe sialorrhea or respiratory complications. Atropine sulfate is inexpensive and appears to have good clinical response combined with good safety profile. The use of trihexyphenidyl for the treatment of sialorrhea can be considered in dyskinetic forms of cerebral palsy or in selected cases.

Clinical and neuropsychological assessment of attention and ADHD comorbidity in a sample of children and adolescents with idiopathic epilepsy.

UNLABELLED: Children with epilepsy present significant problems concerning attention and comorbidity with attention deficit hyperactivity disorder (ADHD). OBJECTIVE: To determine the prevalence of attention complaints, ADHD diagnosis and attention profile in a sample of children and adolescents with idiopathic epilepsy. METHOD: 36 children and adolescents with idiopathic epilepsy and 37 genre and age matched healthy controls underwent several procedures to diagnose their neuropsychological profile and comorbidity with ADHD. RESULTS: The prevalence of ADHD was higher in patients with epilepsy [χ2= 4.1, p = 0.043, 6 (16.7%) vs 1 (2.7%)], with worse results in attention related WISC items and factors in patients with epilepsy comparing to the controls, but not between patients with and without ADHD. Clinical characteristics did not influence those results. CONCLUSION: This study found a greater prevalence of problems wih attention in pediatric patients with idiopathic epilepsy, but not a distinct profile between those with or without ADHD.

Clinical and neuropsychological assessment of attention and ADHD comorbidity in a sample of children and adolescents with idiopathic epilepsy / Avaliação clínica e neuropsicológica da atenção e comorbidade com TDAH em crianças e adolescentes com epilepsia idiopática

Children with epilepsy present significant problems concerning attention and comorbidity with attention deficit hyperactivity disorder (ADHD). Objective To determine the prevalence of attention complaints, ADHD diagnosis and attention profile in a sample of children and adolescents with idiopathic epilepsy. Method 36 children and adolescents with idiopathic epilepsy and 37 genre and age matched healthy controls underwent several procedures to diagnose their neuropsychological profile and comorbidity with ADHD. Results The prevalence of ADHD was higher in patients with epilepsy [χ2= 4.1, p = 0.043, 6 (16.7%) vs 1 (2.7%)], with worse results in attention related WISC items and factors in patients with epilepsy comparing to the controls, but not between patients with and without ADHD. Clinical characteristics did not influence those results. Conclusion This study found a greater prevalence of problems wih attention in pediatric patients with idiopathic epilepsy, but not a distinct profile between those with or without ADHD. .

Crianças com epilepsia podem apresentar problemas de atenção e comorbidade com transtorno de atenção e hiperatividade (TDAH). Objetivo Determinar a prevalência de queixas de atenção, diagnóstico de TDAH e perfil atentivo em uma amostra de crianças e adolescentes com epilepsia idiopática. Método 36 crianças e adolescentes com epilepsia idiopática e 37 controles saudáveis foram submetidos a vários procedimentos para diagnosticar perfil neuropsicológico e comorbidade com TDAH. Resultados A prevalência de TDAH foi maior em pacientes com epilepsia [χ2 = 4,1, p = 0,043, 6 ( 16,7%) vs 1 (2,7%)] , que também apresentaram piores resultados em itens e fatores dependentes de atenção do WISC. Não foram observadas diferenças entre pacientes com e sem TDAH. As características clínicas não influenciaram resultados. Conclusão Este estudo encontrou uma maior prevalência de problemas com atenção em pacientes pediátricos com epilepsia idiopática , mas não um perfil distinto entre aqueles com ou sem TDAH. .

Mental health of children and adolescents with epilepsy: analysis of clinical and neuropsichological aspects / Saúde mental de crianças e adolescentes com epilepsia: análise de fatores clínicos e neuropsicológicos

Epilepsy compromises the development of cognitive and social skills and represents a risk of psychiatric comorbidity. Objective: To compare psychopathological symptoms in children with epilepsy and in a healthy group, and to correlate the results with neuropsychological and clinical variables. Method: Forty five children with idiopathic epilepsy and sixty five healthy controls underwent neuropsychological evaluation and their caregivers replied to a psychopathology questionnaire (Child Behavior Checklist – CBCL). Results: There were significant differences in CBCL, with poorer results showed mainly by patients with epilepsy. There was no significant association between any psychopathological symptom and disease duration or amount of antiepileptic drugs used. There was positive correlation between intelligence quocient and CBCL on items such as sluggish cognitive tempo, aggressive behavior, attention problems and activities and a negative relation between academic achievement, conduct and rule-breaking behavior. Conclusion: Children with epilepsy had the worse results in the psychopathology evaluation. Certain psychopathological variables are related to the cognitive profile, with no relation to clinical variables. .

A epilepsia compromete o desenvolvimento de habilidades cognitivas e sociais e representa um risco para comorbidade psiquiátrica. Objetivo: Comparar sintomas psicopatológicos em crianças com epilepsia e um grupo saudável, e correlacionar os resultados com variáveis neuropsicológicas e clínicas. Método: Quarenta e cinco crianças com epilepsia idiopática e sessenta e cinco controles saudáveis foram submetidos à avaliação neuropsicológica e seus cuidadores responderam a um questionário de psicopatologia (Child Behavior Checklist – CBCL). Resultados: Houve diferenças significativas no CBCL, com piores resultados principalmente para pacientes com epilepsia. Não houve associação significativa entre os resultados e duração da doença ou número de drogas antiepilépticas. Houve relação positiva entre quociente de inteligência e CBCL em itens como tempo cognitivo lento, comportamento agressivo, problemas de atenção e atividades e uma relação negativa entre o desempenho acadêmico e conduta e comportamento de quebras de regra. Conclusão: Crianças com epilepsia apresentaram piores resultados em psicopatologia, com certas variáveis psicopatológicas relacionadas ao perfil cognitivo, mas sem relação com variáveis clínicas. .

Mental health of children and adolescents with epilepsy: analysis of clinical and neuropsichological aspects.

UNLABELLED: Epilepsy compromises the development of cognitive and social skills and represents a risk of psychiatric comorbidity. OBJECTIVE: To compare psychopathological symptoms in children with epilepsy and in a healthy group, and to correlate the results with neuropsychological and clinical variables. METHOD: Forty five children with idiopathic epilepsy and sixty five healthy controls underwent neuropsychological evaluation and their caregivers replied to a psychopathology questionnaire (Child Behavior Checklist - CBCL). RESULTS: There were significant differences in CBCL, with poorer results showed mainly by patients with epilepsy. There was no significant association between any psychopathological symptom and disease duration or amount of antiepileptic drugs used. There was positive correlation between intelligence quocient and CBCL on items such as sluggish cognitive tempo, aggressive behavior, attention problems and activities and a negative relation between academic achievement, conduct and rule-breaking behavior. CONCLUSION: Children with epilepsy had the worse results in the psychopathology evaluation. Certain psychopathological variables are related to the cognitive profile, with no relation to clinical variables.

Beliefs and Attitudes about Childhood Epilepsy among School Teachers in Two Cities of Southeast Brazil.

Childhood epilepsy is a chronic neurological disorder associated with profound psychosocial limitations epileptic children's routine. Lack of information and inappropriate beliefs are still the factors that most contribute to the stigma and discrimination. This study aimed at characterizing teacher's beliefs and attitudes at regular and special schools in two cities of southeastern Brazil where students with epilepsy studied. Fifty-six teachers of public regular schools and specialized educational institutions for children with disabilities from two cities of Southeast Brazil who had epileptic children in their classroom completed the Brazilian version of The Epilepsy Beliefs and Attitudes Scale: Adult Version and answered a data sheet about sociodemographic characteristics. The results showed that no significant differences (P ≤ 0.05) have been found between the beliefs and attitudes of teachers in mainstream and special schools but both schoolteachers had more inappropriate beliefs and attitudes than appropriate ones against childhood epilepsy. These findings raise an important issue, providing us with the knowledge that epilepsy is still a condition which is surrounded by wrong beliefs. Also, educational programs could help reduce the gaps in knowledge about how such disease has been perceived worldwide.

Impacto da epilepsia no processo de escolarização de crianças e adolescentes / Impact of epilepsy on the schooling of children and adolescent

O objetivo deste trabalho foi identificar o número de crianças com epilepsia matriculadas em escolas públicas regulares e especiais de duas cidades brasileiras e verificar se variáveis associadas à epilepsia estão correlacionadas ao tipo de escola, que as crianças frequentavam. Participaram do estudo 56 crianças e adolescentes com idade entre 7 e 14 anos e utilizaram-se para a coleta de dados dois instrumentos: Formulário de identificação da criança e Classificação de Crises de Engel. Os dados obtidos foram analisados descritiva e comparativamente, utilizando-se o Software Minitab - Versão 12.1, por meio dos testes t de Student, Mann-Whitney, qui-quadrado, além de regressão logística. Os resultados indicaram que as crianças das escolas especiais quando comparadas às crianças das escolas regulares apresentavam gravidade da doença e frequência de crises mais elevadas, início mais precoce e duração da epilepsia mais longa, além de utilizarem maior número de medicamentos. Conclui-se que variáveis orgânicas da epilepsia podem interferir no processo de escolarização de crianças epilépticas. Entretanto, são necessários novos estudos visando investigar dificuldades que poderiam interferir no rendimento acadêmico causando alterações no aprendizado de crianças com epilepsia.

The objective of this paper was to identify the number of children with epilepsy enrolled in regular and special public schools in two medium size Brazilian cities and verify which disease variables are correlated to the type of school the children attended to. Study participants were 56 children and adolescents aged between 7 and 14 years; two instruments were used: forms of identification of children and Engel's seizure classification. Obtained data were analyzed, using the Minitab Software - Version 12.1, by means of the Student t, Mann-Whitney and chi-square tests and logistic regression. The results indicate that children from special schools presented cases that were more sever, with greater seizure frequency, earlier epilepsy onset, and longer episodes of epilepsy; they also used a greater number of anticonvulsants. The conclusion indicated that organic epilepsy variables can affect the schooling process for children with epilepsy. However, further studies are needed to investigate difficulties that might interfere with academic performance and that may affect learning in children with epilepsy.

Epileptic events in the XIX century as reported by the Brazilian Royal Family.

UNLABELLED: Members of the Brazilian Royal Family carry a rich medical history of epileptic seizures and alike. OBJECTIVE: To present the medical knowledge about epilepsy by the time of the Brazilian Empire, as reported by the royal family. METHOD: Narrative review of historical facts about D. Pedro I's family health. RESULTS: The Royal Family, since D. João VI's generation is full of members with epilepsy or acute symptomatic seizures of different etiologies. CONCLUSION: The reported cases suggest that Dom Pedro I's family presented epilepsy with tonic-clonic generalized seizures, besides psychogenic, organic non epileptic events and acute symptomatic seizures. As a whole, this familial epilepsy could fit the diagnosis of generalized epilepsy with febrile convulsion plus.

Epileptic events in the XIX century as reported by the Brazilian Royal Family / Eventos epilépticos no século XIX segundo relatos da Família Real Brasileira

Members of the Brazilian Royal Family carry a rich medical history of epileptic seizures and alike. OBJECTIVE: To present the medical knowledge about epilepsy by the time of the Brazilian Empire, as reported by the royal family. METHOD: Narrative review of historical facts about D. Pedro I's family health. RESULTS: The Royal Family, since D. João VI's generation is full of members with epilepsy or acute symptomatic seizures of different etiologies. CONCLUSION: The reported cases suggest that Dom Pedro I's family presented epilepsy with tonic-clonic generalized seizures, besides psychogenic, organic non epileptic events and acute symptomatic seizures. As a whole, this familial epilepsy could fit the diagnosis of generalized epilepsy with febrile convulsion plus.

Há uma história médica rica de crises epilépticas e similares nos membros da família real brasileira. OBJETIVO: Apresentar o conhecimento médico sobre epilepsia à época do Império no Brasil, conforme informado pelos acometidos da família real. MÉTODO: Revisão narrativa de fatos históricos sobre a saúde da família de D. Pedro I. RESULTADOS: A genealogia da família real, desde a geração de D. João VI é repleta de pessoas acometidas por epilepsia ou crises sintomáticas agudas de diversas etiologias. CONCLUSÃO: O relato de casos sugere que Dom Pedro I e sua família apresentavam epilepsia de incidência familiar predominantemente com crises tônico-clonicas generalizadas, além de eventos sintomáticos agudos, psicogênicos e orgânicos não epilépticos. Como um todo, a epilepsia dessa família poderia ser enquadrada no diagnóstico de epilepsia generalizada com convulsão febril plus.

Epileptic seizures in a descendant of Dom Pedro I.

UNLABELLED: Intrauterine seizure is a rare event. Genetic predisposition and trauma are possible risk factors. OBJECTIVE: To review and comment on the historical description of intrauterine events of a bastard daughter of Dom Pedro I (Maria Isabel Alcântara Brasileira - 1830-1896). METHOD: Review of historical facts about the health of Dom Pedro I's daughter according to primary and secondary historical data. RESULTS: According to historical accounts, Dom Pedro I's daughter suffered trauma during the intrauterine period that provoked intrauterine seizures. At the age of eight years, she developed self-limited and benign generalized epilepsy. Like her father, she had mood problems and also learning difficulties. CONCLUSION: Dona Maria Isabel's own report does not shown sufficient evidence to support the diagnosis of post-traumatic intrauterine seizures. Nevertheless, her family history suggests a genetic basis for her epilepsy.

Epileptic seizures in a descendant of Dom Pedro I / Crises epilépticas em uma descendente de Dom Pedro I

Intrauterine seizure is a rare event. Genetic predisposition and trauma are possible risk factors. OBJECTIVE: To review and comment on the historical description of intrauterine events of a bastard daughter of Dom Pedro I (Maria Isabel Alcântara Brasileira - 1830-1896). METHOD: Review of historical facts about the health of Dom Pedro I's daughter according to primary and secondary historical data. RESULTS: According to historical accounts, Dom Pedro I's daughter suffered trauma during the intrauterine period that provoked intrauterine seizures. At the age of eight years, she developed self-limited and benign generalized epilepsy. Like her father, she had mood problems and also learning difficulties. CONCLUSION: Dona Maria Isabel's own report does not shown sufficient evidence to support the diagnosis of post-traumatic intrauterine seizures. Nevertheless, her family history suggests a genetic basis for her epilepsy.

A convulsão intra-uterina é evento raro, sendo possíveis fatores de risco a genética e o traumatismo. OBJETIVO: Rever e comentar a descrição histórica de eventos intra-uterinas de uma filha bastarda de D. Pedro I (Maria Isabel Alcântara Brasileira - 1830-1896). MÉTODO: Revisão dos fatos históricos sobre a saúde da filha do D. Pedro I, de acordo com dados históricos primários e secundários. RESULTADOS: A filha de Dom Pedro I, de acordo com relatos históricos teria sofrido um traumatismo durante o período intra-uterino, o que provocou convulsões intra-uterinas. Na idade de oito anos a menina desenvolveu uma epilepsia generalizada limitada e benigna. Como seu pai, teve problemas do humor e, também, dificuldades de aprendizagem. CONCLUSÃO: O relato de Dona Maria Isabel não gera prova suficiente para sustentar o diagnóstico de convulsões intra-uterinas de origem traumática. Não obstante, seus antecedentes familiares sugerem uma base genética para sua epilepsia.

Adaptação transcultural para o português brasileiro do instrumento The Epilepsy Beliefs and Attitudes Scale (Ebas): adult version / Transcultural adaptation for brazilian portuguese of The Epilepsy Beliefs and Attitudes Scale (Ebas): adult version

INTRODUÇÃO: o desconhecimento acerca da epilepsia tem fomentado o interesse em desenvolver ferramentas voltadas para detecção das crenças e atitudes da comunidade em geral acerca da doença. OBJETIVO: apresentar o processo de adaptação transcultural do instrumento The Epilepsy Beliefs and Attitudes Scale (EBAS) - Adult Version e uma versão em português para uso no Brasil. MÉTODO: o processo de adaptação envolveu a equivalência conceitual, de itens, semântica e operacional. RESULTADOS: os conceitos apreendidos foram considerados pertinentes à nossa cultura e seus itens adequados quanto à sua capacidade de representar tais conceitos na população-alvo, além de apresentar boa equivalência semântica entre a versão final em português e o original. CONCLUSÃO: o instrumento mostrou-se adequado para uso na população em geral e seus resultados serão revistos em estudos futuros a luz de avaliações psicométricas.

INTRODUCTION: the lack of knowledge about epilepsy has boosted the interest to develop instruments aimed for the detection of beliefs and attitudes of the community in general about the disease. OBJECTIVE: to carry out the transcultural adaptation of the instrument The Epilepsy Beliefs and Attitudes Scale (EBAS) - Adult Version and a version in Brazilian Portuguese. METHOD: the transcultural adaptation involved the conceptual, items, operational and semantic equivalences. RESULTS: the concepts were considered relevant to our culture and the items as appropriate to their ability to represent these concepts in the target population. It was possible establish high-quality semantic equivalence between the Brazilian Portuguese-language final version and the original. CONCLUSION: the instrument proved to be suitable for use in the general population and the results will be reevaluated in the light of forthcoming psychometric analysis.

Avaliação clínica e neuropsicológica da atenção e comorbidade com TDAH nas epilepsias da infância: uma revisão sistemática / Clinical and neuropsychological evaluation of attention in children and adolescents with epilepsy: a systematic review

INTRODUÇÃO: As comorbidades psiquiátricas em epilepsia, tais como o Transtorno do Déficit de Atenção e Hiperatividade (TDAH), podem comprometer o desempenho acadêmico e o funcionamento social, ocasionando sofrimento psíquico adicional e reduzindo a qualidade de vida dos pacientes, independente do controle adequado das crises. OBJETIVO: Abordar aspectos epidemiológicos e diagnósticos do TDAH e problemas de atenção em crianças e adolescentes com epilepsia, revisando a literatura sobre o tema. METODOLOGIA: Revisão sistemática a partir de artigos obtidos do banco bibliográfico MEDLINE e LILACS. RESULTADOS: Estudos demonstram a relevância epidemiológica de TDAH em crianças e adolescentes com epilepsia, sendo sua prevalência maior do que na população normal. O problema de atenção ocorre mais frequentemente na epilepsia, independente da presença de TDAH. Há falta de uniformidade nas amostras estudadas, dificultando avaliar a natureza do déficit atentivo, dada a multicausalidade do mesmo nas epilepsias e a real prevalência do TDAH. CONCLUSÃO: Há necessidade de maior atenção ao diagnóstico de TDAH nas crianças com epilepsia para a abordagem clínica adequada.

INTRODUCTION: Psychiatric comorbidities in epilepsy, such as Attention Deficit and Hyperactivity Disorder (ADHD) may compromise one child academic achievement and social functioning, increasing psychic suffering and reducing quality of life, in spite of adequate seizure control. OBJECTIVE: To analyze epidemiological and diagnostic aspects of ADHD and attention problems in children and adolescents with epilepsy (literature review). METHODOLOGY: Systematic literature review (MEDLINE and LILACS). RESULTS: Studies demonstrate the epidemiological relevance of ADHD in the pediatric epilepsy population as it's more prevalent than among normal children and adolescents. Attention problems can occur in spite of an ADHD diagnosis. There is no homogeneity among the studied samples, what turns difficult the evaluation of the real nature of the attention deficits in children with epilepsy, which are multicausal, as its real prevalence. CONCLUSIONS: It's important to pay attention to ADHD diagnosis in children and adolescents with epilepsy.

Validation of a Portuguese version of a generic health quality-of-life questionnaire for children with neurological and neuropsychiatric disorders, including epilepsy.

PURPOSE: To validate a Portuguese version of a generic HRQL instrument already used for children with epilepsy. To analyze differences of scores among children with epilepsy and co-morbidities in contrast to other neurological and neuropsychiatric diagnosis, especially Attention Deficit and Hyperactivity Disorder (ADHD). METHODS: A total of 194 children (64 with epilepsy, 94 with ADHD and 38 with other neurological or neuropsychiatric diagnosis) were consecutively seen in a neuropaediatric ambulatory unit. Parents or caregivers responded to a Portuguese version of ICIS (Impact of Childhood Illness Scale). The questionnaire was analyzed for internal consistency and face validity. Clinical and socio-demographic variables were also analyzed. RESULTS: Internal consistency, analyzed by Cronbach's alpha showed good results for total and combined scores and all sections of ICIS, except for the section "impact of illness and its treatment". The diagnostic categories differed in several ICIS sections, with the worst scores for children with epilepsy and co-morbidities. Children who only had epilepsy had a worse score on the section about treatment impact compared with children with ADHD, but better scores in relation to disease impact in development and parents/family. In the epilepsy group, HRQL scores were worst for symptomatic epilepsy, whilst seizure control and socioeconomic factors affected each section of the instrument in different ways. CONCLUSIONS: ICIS Portuguese version has good psychometric properties. It is a useful tool for analyzing HRQL in children with epilepsy and other neurodevelopmental and psychiatric disorders. Epilepsy and psychiatric co-morbidity seems to have an additive impact on a child's HRQL. This result is clinically important since epilepsy management must include not only seizure control but also mental health support.

Epilepsia e saúde mental na infância / Epilepsy and child mental health

OBJETIVO: Revisar critica e sistematicamente a literatura atual sobre as comorbidades neuropsiquiátricas em crianças e adolescentes com epilepsia e seu impacto na qualidade de vida destes pacientes. METODOLOGIA: Revisão da literatura em revistas indexadas através do MEDLINE, período de janeiro de 2000 a dezembro de 2005. Foram selecionados, a partir dos resumos, os estudos originais (epidemiológicos, diagnósticos e terapêuticos), as meta-análises, revisões sistemáticas e revisões em língua portuguesa e inglesa. RESULTADOS: As taxas de transtornos mentais nos pacientes estudados são maiores do que em crianças normais ou mesmo com outras doenças crônicas. Uma vez que a epilepsia é uma síndrome heterogênea, múltiplos fatores podem determinar o desenvolvimento de psicopatologia. O artigo revisa as relações entre epilepsia e retardo mental, transtornos abrangentes, transtornos da linguagem, aprendizagem e TDAH, transtornos ansiosos e do humor, psicoses, além de aspectos diagnósticos e terapêuticos e prognóstico. CONCLUSÕES: O tratamento moderno da criança com epilepsia ultrapassa o simples controle das crises epilépticas, devendo ser abrangidos o adequado diagnóstico e tratamento das comorbidades psiquiátricas a fim de melhorar a sua qualidade de vida.

OBJECTIVE: To review the current literature about neuropsychiatry co-morbidities of children and adolescents with epilepsy and the impact of these problems in the quality of life of these patients. METHODOLOGY: Literature review in journals indexed through MEDLINE, from January 2000 to December 2005. We selected from the abstracts, original studies (epidemiology, diagnoses and therapeutics), meta-analyses, and reviews in Portuguese and English. RESULTS: The rates of mental disorders in the patients studied are higher than in normal children or even in children with other chronic diseases. Once the epilepsy is a heterogeneous syndrome, multiple factors can determine the development of psychopathology. This paper reviews the relationships among epilepsy and mental retardation, pervasive disorder, language and learning disorders and attention deficit disorder, anxiety and mood disorders, psychoses, as well as diagnoses, therapeutic and prognostic aspects. CONCLUSIONS: The modern treatment of children with epilepsy goes beyond the simple control of the epileptic seizures and should include the appropriate diagnosis and treatment of the psychiatric co-morbidities in order to improve their life quality.

[Development and validation of a health related quality of life questionnaire for Brazilian children with epilepsy: preliminary findings].

PURPOSE: To construct a multidimensional questionnaire that analyses the epileptic child quality of life from the parental point of view. METHOD: The pilot questionnaire was composed of 157 questions distributed in several dimensions. Fifty-one epileptic children's parents answered the questionnaire. The instrument was tested in its diverse properties: frequency of endorsement, homogeneity (Cronbach alpha), criterion and face validity, and later it was reduced. RESULTS: Endorsement frequency excluded 65 questions that did not attain a minimum of 5% response per item. Cronbach alpha was as follows: physical (0.93), psychological (0.91), social (0.91), familiar (0.70), cognitive (0.92), medical (0.30) and economical (0.37). Patient groups, in relation to seizure control, significantly differed only in physical domain and total score, although there was a trend to differences in other domains. The final questionnaire (QVCE50) has 50 items, with good homogeneity in the physical, psychological and cognitive domains. CONCLUSION: QVCE-50 is a promissing Brazilian HRQL questionnaire for children with epilepsy. It needs to be applied in a larger population to confirm its psychometric properties.

Development and validation of a health related quality of life questionnaire for Brazilian children with epilepsy: preliminary findings

OBJETIVO: Construir um questionário multidimensional analisando a qualidade de vida da criança com epilepsia do ponto de vista dos responsáveis. MÉTODO: O questionário piloto foi composto de 157 itens distribuídos em várias dimensões. Cinqüenta e um responsáveis de crianças com epilepsia responderam ao questionário. O instrumento foi testado em suas diversas propriedades freqüência de endosso, homogeneidade (alfa de Cronbach), validade de critério e aparência, sendo posteriormente reduzido. RESULTADOS: A freqüência de endosso excluiu 65 questões que não atingiram um mínimo de 5% de respostas por item. O alfa de Cronbach foi: domínio físico (0,93), psicológico (0,91), social (0,91), familiar (0,70), cognitivo (0,92), médico (0,30) e econômico (0,37). Os grupos de pacientes (de acordo com o controle de crises) diferiram significativamente apenas no domínio físico e no escore total, embora seja percebida uma tendência à diferença nos demais domínios. O questionário final (QVCE50) tem 50 itens, com boa homogeneidade nos domínios físico, psicológico e cognitivo. CONCLUSÃO: O QVCE-50 é um questionário de qualidade de vida relacionada á saúde promissor. Há necessidade de aplicá-los a uma população maior a fim de testar suas características psicométricas.

Anti-epileptic drug intake adherence: the value of the blood drug level measurement and the clinical approach

It was evaluated the patient antiepileptic drug (AED) intake adherence in a pilot cross-sectional study carried out at a neurologic out-patient clinic of a university hospital. Ninety-three AED blood concentration (phenobarbital, phenytoin, carbamazepine) were analyzed from 24 patients. The variability of the AED blood level was measured (in the steady state period by means of the variation coefficient) and compared with the self-reported antiepileptic medication non-adherence. AED blood level according to the range (therapeutic or not), and the seizure control. It was not observed any strong correlation between the higher value of variability and the other three parameters of no adherence. The highest correlation was with the blood drug level (therapeutic or not). The evaluation of blood drug measurement alone, except in cases of extreme low adherence and variability of drug intake, is not enough for the recognition of incorrect drug intake, but the clinical markers and the self-reported adherence have to be also considered for this sort of evaluation.