ABSTRACT
ABSTRACT Background: self-limited epilepsy with centrotemporal spikes, previously considered benign focal childhood epilepsy with centrotemporal spikes show clinical signs of involvement of Rolandic areas, mainly lower area, which may affect the planning and execution of motor sequences. Objective: This study aimed to evaluated oral praxis in children with self-limited epilepsy with centrotemporal spikes and compare to the age-matched control group. Methods: This was a descriptive study with 74 children with self-limited epilepsy with centrotemporal spikes, with the classical forms according to International League Against Epilepsy, and between 4 and 15 years of age, selected from the child neurology outpatient clinic of the Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, and 239 age-matched and educational level-matched (convenience sampling) control children. All children were submitted to the battery of oral volitional movements, which consisted of 44 tests for oral movement (tongue, lip, cheek, jaw, and palate) and 34 phonemes and consonant cluster tasks, with simple and sequenced oral movements. Results: The mean age and standard deviation (SD) of children with epilepsy was 9.08 years (SD 2.55) and of controls 9.61 years (SD 3.12). The results showed significant differences between the groups with a poorer performance of children with epilepsy compared to children without epilepsy in simple and particularly in sequenced movements. Conclusion: These findings can be attributed to the genetically determined immaturity of cortical structures related to motor planning in children with self-limited epilepsy with centrotemporal spikes.
RESUMO Antecedentes: Epilepsia autolimitada com descarga centrotemporal, previamente designada por epilepsia benigna focal infantil com espículas centrotemporais, mostra sinais clínicos de envolvimento de áreas rolândicas, principalmente área inferior, que podem afetar o planejamento e a execução de sequências motoras. Objetivo: Este estudo visou avaliar a práxis oral em crianças com epilepsia autolimitada com espículas centrotemporais e comparar com o grupo de controle de mesma idade e grau de escolaridade. Métodos: Tratou-se de um estudo descritivo, com 74 crianças com epilepsia autolimitada com espículas centrotemporais selecionadas no ambulatório de neurologia infantil do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brasil, e 239 crianças do grupo controle da mesma faixa etária e grau de escolaridade. Todas as crianças foram submetidas à bateria de tarefas de movimento oral volitivo, que inclui movimentos orais simples e sequenciados. Resultados: A idade média das crianças com epilepsia era de 9,08 anos (desvio padrão - DP 2,55) e dos controles 9,61 anos (DP 3,12). Os resultados mostraram diferenças significativas entre os grupos, com desempenho mais fraco das crianças com epilepsia em comparação ao das crianças saudáveis, em movimentos simples e particularmente em movimentos sequenciados. Conclusão: Esses resultados podem ser atribuídos à imaturidade geneticamente determinada das estruturas corticais relacionadas com o planejamento motor em crianças com epilepsia autolimitada com espículas centrotemporais.
Subject(s)
Humans , Child , Apraxias , Epilepsy, Rolandic , Brazil , Control Groups , ElectroencephalographyABSTRACT
Objective@#To investigate the electroclinical findings in epilepsy children with epileptic negative myoclonus (ENM) restricted to the lower limb as the first seizure type.@*Methods@#Each retrieved electroencephalogram record performed between March 2011 and March 2018 at the Department of Pediatrics of Peking University First Hospital was searched with "midline" . There were 302 records of 175 patients with "benign" or "functional" midline spikes. A retrospective review of each patient′s hospital record was performed. Thirteen patients had ENM restricted to the lower limb as the first seizure type. The clinical and electroencephalogram characteristics of them were analyzed.@*Results@#Thirteen patients manifested ENM restricted to the lower limb as the first seizure type, diagnosed as benign childhood focal epilepsy with vertex spikes (BEVS). Six patients had ENM as the first and only seizure type during the short-time follow-up. Among them, there were 1 male and 5 females. The age at seizure onset was (2.5±0.7) years. One of them had electrical status epilepticus during sleep (ESES) identified on electroencephalogram at theage of 4 years and 8 months. The last follow-up age was (3.8±1.5) years. The remaining 7 patients developed nocturnal focal motor seizures. Among them, there were 4 males and 3 females. The age at seizure onset was (3.5±0.7) years. Two of them were diagnosed as BEVS evolving into benign childhood epilepsy with centrotemporal spikes (BECTS) and 5 were diagnosed as BEVS concurring with BECTS. The age at focal seizures was (4.1±0.6) years. The interval ranged from 1 month to 1 years. Six of 7 patients had electrical ESES with the age of (5.2±1.0) years. All had developmental regression, further diagnosed as atypical benign partial epilepsy (ABPE). The median age at last follow-up was 5.9 years. Five of 13 patients had repeated electroencephalogram records at our apartment, showing that epileptiform discharges in midline regions were significantly reduced either in frequency or amplitude with the improvement of ENM restricted to the lower limb and that independent epileptiform discharges in Rolandic regions from midline regions were noticed with the onset of nocturnal focal seizures.@*Conclusions@#ENM restricted to the lower limb has a close association with vertex (midline) epileptiform discharges. ENM restricted to the lower limb as the first seizure type is a peculiar phenomenon of BEVS. Some patients could evolve into BECTS or overlap with BECTS, and further into ABPE. The age of seizure onset in BEVS with ENM restricted to the lower limb as the first symptom is a little earlier than in BECTS. Ignorance of the close association between midline spikes and ENM restricted to the lower limb may lead to misdiagnosis of these patients.
ABSTRACT
La epilepsia benigna atípica de la infancia con paroxismos centrotemporales es una forma poco frecuente de epilepsia en la que se combinan crisis focales similares a las observadas en la epilepsia benigna de la infancia con paroxismos centrotemporales y crisis generalizadas que además presenta un patrón electroencefalográfico característico de punta-onda continua durante el sueño NREM. Se discute la importancia de la correlación electroclínica en el correcto diagnóstico de la entidad en cuestión así como en su adecuado manejo y evaluación pronóstica.
Atypical benign childhood epilepsy with centro-temporal spikes is a rare form of epilepsy that combine partial seizures similar to those seen in benign partial epilepsy of childhood with centro-temporal paroxysms and generalized seizures, also presenting a characteristic EEG pattern of continuous spike-wave during NREM sleep. We discuss the importance of electro-clinical correlation in the correct diagnosis of the disease as well as proper treatment and prognostic assessment.
Subject(s)
Humans , Sleep , Epilepsy, Rolandic , EpilepsyABSTRACT
Objective To study the level and structure of intelligence in children with benign epilepsy of childhood with centrotemporal spikes(BECT)and investigate factors affecting their intelligence.Methods Congitive skills such as intelligence quotient(IQ),phonological test and morphological test,were evaluated in 47 children with BECT,and the results were compared to a control group of 30 children matched for gender,age,level of education and family background.The effects of age of onset,disease course,seizure frequency,seizure type,location of spike,and spike and wave index(SWI)on the level and structure of intelligence were also analyzed.Results The total IQ score was slightly lower in children with BECT(104.20±12.34)when compared to the control group of normal children(109.45±15.01),but the difference was not significant.There was no difference in performance IQ scores between BECT and normal children.BECT children had a lower verbal IQ score(90.67±18.40)when compared to the control group(98.17±13.18,t=3.431.P<0.05).Analyses of verbal subtests revealed significant differences between BECT and normal children in vocabulary and similarities(5.97±2.95 vs 8.51±3.67 and 4.85±3.02 vs 6.95±3.07,respectively,t value were 2.365 and 2.096,both P<0.05).The differences between BECT and normal children were also significant in phonological test and morphological test (12.56±2.3 vs 16.78±3.72 and 22.35±3.25 vs 24.15±5.28,respectively,t=2.478 and 2.770,both P<0.05).SWI was negatively correlated with verbal IQ,vocabulary,similarities,phonological test and morphological test(r=-0.305--0.838,P<0.05).Age of onset,disease course and seizure frequency were not correlated with verbal IQ,phonological test and morphological test.The level of intelligence was not different among children with left hemispheric foci,fight hemispheric foci or bilateral foci.The level of intelligence Was similar between children with partial seizures or secondarily generalized seizures.Conclusions Children with BECT have normal level of intelligence,but the structure of intelligence is abnormal with retardation of their language ability.SWI affects the language ability in children with BECT,but age of onset,seizure type and location of spike do not influence their language ability.