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We report a male infant with adenylosuccinase deficiency who developed epileptic seizures on the second day of life. Growth was normal and seizures were well controlled with anti-epileptic drugs. Despite axial hypotonia associated with peripheral hypertonicity he presented some development until seven months of age, when he developed high fever and died within a few hours. Although clinical heterogeneity in this disorder of purine synthesis and interconversion is well-known, in 14 out of 17 cases who experienced epilepsy seizures started after the first year of life. The early presentation in our index patient followed by his sudden death at the age of 7 months has not been described before. A search for disorders of purine metabolism should be included in the screening programme for every child with severe neonatal convulsions.

ADHD is three to five times more common in people with epilepsy than in the normal population 1,2 . It occurs in 14-31% of children with epilepsy 3,4 . It seems that the severity of epilepsy enhances the predisposition for having ADHD, since comorbidity is described in both benign and refractory childhood epilepsy 1,[5][6][7] . Inattentive ADHD is appointed as the most common subtype in epileptic patients 4,[6][7][8] .Etiology, duration of epilepsy, seizure frequency, seizure control, and the use of antiepileptic drugs are important varia bles related to neuropsychological and behavioural problems in patients with epilepsy and ADHD. It seems that patients with idiopathic epilepsy presenting with few seizures or who are seizure-free with monotherapy have a low frequency of neuropsychological and behavioral problems [8][9][10][11][12] . Epileptiform discharges, particularly during the active phase of the epilepsy, are also an important variable when neuropsychology and behavior are concerned 6,8,11 .The correlation between the variables of epilepsy and ADHD has not yet been fully elucidated. This study aims to verify the occurrence of ADHD symptoms in a group of patients with idiopathic epilepsy and the relationship between clinical variables, electroencephalogram (EEG), treatment, and psychosocial and behavioral changes. MetHoDsThis is a cross-sectional study. We interviewed 60 patients who were diagnosed with idiopathic epilepsy according to the ILAE criteria 13 . Patients were followed at the Childhood AbstrActOur aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD) with epilepsy and behavior problems. This was a crosssectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners' Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%), seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.Keywords: epilepsy, ADHD, childhood, psychosocial aspects, behavior. resuMo Nosso objetivo foi clarificar a correlação entre transtorno do déficit de atenção (TDAH) com epilepsia e problemas comportamentais. Este foi um estudo transversal. Sessenta crianças com epilepsia idiopática foram entrevistadas com a Escala para Pais e Professores MTA-SNAP IV, Escala de Comportamento Adaptativo Vineland e Escala Conners. Utilizamos o teste do qui-quadrado para analisar a correlação das variá-veis de epilepsia em pacientes com e sem TDAH com um nível de significâ...

RESUMO -Abordaram-se parâmetros quantitativos do eletrencefalograma em crianças com epilepsia benigna da infância com pontas centrotemporais (EBICT). Foram estudadas 27 crianças com diagnósticos de EBICT. Foi realizado o eletrecenfalograma durante vigília, em repouso, e selecionadas cerca de 20 janelas com 2,56 s. Foram calculados os valores de potência absoluta e relativa nas faixas delta, teta, alfa e beta. Os resultados foram comparados aos de 27 crianças sadias pareadas quanto a idade e escolaridade materna. A potência absoluta foi significativamente maior no grupo EBICT nas nas bandas delta e teta para a quase totalidade dos eletrodos e para alguns eletrodos nas faixas alfa e beta. A potência relativa teta foi também maior no grupo EBICT na maioria dos eletrodos. Esses achados sugerem que na EBICT, embora a atividade epileptiforme seja focal. Ocorrem modificações funcionais difusas que incluem alterações do perfil da distribuição das faixas de frequência, com maior potência relativa teta.PALAVRAS-CHAVE: eletrencefalograma, eletencefalograma quantitativo, epilepsia rolândica.Quantitative electroencephalography in children with bening childhood epilepsy with centrotemporal spikes: analysis of band power ABSTRACT -Quantitative EEG aspects are studied in children with benign childhood epilepsy with centrotempral spikes (BCET). A total of 27 children, from 7 to 11 years neurologically and intellectually normal was studied and compared to a control group of normal children. They were submitted to anamnesis, neurological examination, Raven test, digital electroencephalogram and quantitative eletroencephalogram analysis.There was a higher delta, theta, alpha and beta absolute power in most of the electrodes and of alpha and beta for some electrodes in the BCET group. Relative theta power was also higher for the BECT group in most of the electrodes. These findings suggest that in BECT there are diffuse differences form age-matched normal children including a difference in relative spectrum of electrical cerebral activity and that this may be related to a functional immaturity.KEY WORDS: electroencephalogram, quantitative electroencephalogram, EEG maturation, frequency analysis, rolandic epilepsy.As descrições da atividade de base do eletrencefalograma (EEG) em crianças com epilepsia benigna da infância com pontas centrotemporais (EBICT) se referem fundamentalmente à análise visual do traçado. A atividade elétrica cerebral de base é usualmente normal 1-3 ; esta característica é inclusive um dos critérios diagnósticos da EBICT.Braga et al. 4 ao realizarem a análise quantitativa da atividade de base em crianças com descargas centrotemporais, com ou sem crises epilépticas e com ou sem sinais lesionais cerebrais, observaram, na comparação com indivíduos normais, maior potência absoluta em várias faixas de freqüência, de modo difuso. Doze dessas crianças tinham o diagnóstico de EBICT. Os achados apontariam para alterações difusas e não apenas focais como é a atividade epileptiforme centrotemporal.Este estudo tem por objet...

RESUMO -Foram selecionadas 20 crianças com diagnóstico de epilepsia benigna da infância com pontas centrotemporais. Realizou-se anamnese, exame neurológico, aplicação da Escala de Maturidade Mental" Columbia" (1993) e eletrencefalograma digital. (EEG) O EEG digital foi gravado com eletrodos de acordo com o sistema internacional 10-20, acrescido de um eletrodo suplementar entre o eletrodos C3 eT3 ou C4 e T4 no lado onde ocorreu o maior número de pontas. Foi realizada a promediação dessas descargas para análise do local de máxima negatividade. A ocorrência de crises envolvendo um membro superior foi proporcionalmente maior nos sujeitos com pontas de máxima negatividade em C3, C4 do que em C5, C6, T3 ou T4. A atividade epileptiforme não centrotemporal associada foi mais frequente nos sujeitos com descargas centrotemporais de máxima negatividade em C3 e C4. Esse estudo demonstra a existência de diferenças clínicas e eletrencefalográficas de acordo com a localização das descargas centrotemporais.PALAVRAS-CHAVE: epilepsia, infância, eletrencefalograma digital, epilepsia benigna da infância com pontas centrotemporais, foco central alto e baixo. Benign childhood epilepsy with centrotemporal spikes: high and low central focusABSTRACT -Twenty children with benign childhood epilepsy with centrotemporal spikes were studied. Anamnesis, neurological exam, "Columbia" Scale (1993) application and digital electroencephalogram were carried out. The digital electroencephalogram was recorded with electrodes according to 10-20 international system, and a supplementary electrode, between C3 and T3 or C4 and T4 -C5 or C6, respectively, at the side with a higher number of spikes. The averaging of the spikes was carried. Localization and the laterality of the spikes were analyzed. Seizures involving a superior limb occurred in a higher proportion of cases with spikes of maximum negativity in C3, C4. Independent epileptiform activity, different from centrotemporal, was more frequent in the cases were the maximum negativity of the spikes occurred in C3 or C4 regions. There are clinical and electroencephalographic differences according to the localization of the discharges KEY WORDS: epilepsia, children, electroencephalogram, benign childhood epilepsy with centrotemporal spikes, high and low central spikes. A epilepsia benigna da infância com pontas centrotemporais (EBICT) 1 caracteriza-se por acometer crianças de desenvolvimento normal e sem lesões cerebrais, com idade entre 2 e 13 anos. As crises epilépti-cas são geralmente focais, envolvendo a face, a região orofaríngea e, com menor frequência, um dos membros superiores. Apresentam curta duração e ocorrem usualmente durante o sono. O eletrencefalograma (EEG) dos pacientes com EBICT mostra atividade elétrica cerebral de fundo normal e, pontas frequentemente unilaterais, difásicas, de alta voltagem, agrupadas, recorrentes em curtos intervalos, e às vezes, acompanhadas de ondas lentas, localizadas nas regiões centrais e/ou temporais médias. O prognóstico é bom, com o desaparecimento das cr...

xii Attention deficit and hyperactivity disorder (ADHD) occurs in 0.2 to 27% of all children with epilepsy. The frequency of the ADHD in patients with epilepsy, and the correlation with psychosocial aspects and behavior problems are not completely clear. The aim of this study was to verify psychosocial aspects and behavior problems in a group of patients with epilepsy and ADHD. This was a transversal study. One hundred patients were interviewed and eighty five patients (ages ranging from six to 16 years old) were selected and interviewed with a structured questionnaire. Sixty patients were diagnosed with epilepsy with presumably genetic etiology without epileptic encephalopathy (group I) and 25 patients were diagnosed with epilepsy with structural/metabolic etiology and unknown cause (group II). After clinical and EEG characterization, we used the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and the Conner's Rating Scales. ADHD occurred in 8/60 patients of group I (13%) and in 12/25 patients of group II (48%). ADHD was significantly more frequent in patients of group II than patients of group I (p = 0.02). Patients with ADHD in group II had significantly more seizures (p = 0.01), not well controlled (p = 0.02), used politherapy (p = 0.01) and lower scores (p = 0.04) in Vineland Scales (communication domain) than patients in group I. The comorbidity epilepsy-ADHD occurred in 23,5% of patients. Sporadic or well-controlled seizures and patients on monotherapy were important variables to predict lower rates of ADHD and behavioural abnormalities. xiii xv ____________________________________________________LISTA DE ANEXOS Pag. Anexo 1 Termo de consentimento livre e esclarecido 131 Anexo 2 Anamnese 135 Anexo 3 Questionário auxiliar com os critérios diagnósticos para o TDAH (DSM IV-TR, 2002) 143 Anexo 4 Escala de pontuação para pais e professores MTA-SNAP-IV 145 Anexo 5 Distribuição dos pacientes quanto ao sexo, idade, idade de início e tipo das crises epilépticas, tempo de epilepsia e classificação da epilepsia 146 Anexo 6 Distribuição dos pacientes quanto à frequência e período de controle das crises epilépticas, o eletrencefalograma atual, a terapia medicamentosa e a ressonância magnética de encéfalo 148 Anexo 7 Escala de Conners de avaliação dos pais -versão longa e revisada 150 Anexo 8 Escala de comportamento adaptativo Vineland 152

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