Pediatric Focal Epilepsy Syndromes

Fernández, Iván Sánchez; Loddenkemper, Tobias

doi:10.1097/wnp.0b013e31826bd943

Cited by 22 publications

(20 citation statements)

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“…Neurocognitive dysfunction, if present, is mild. The individual description of each particular syndrome is beyond the scope of this review and can be found elsewhere [101, 102]. Because of their main features, “benign” pediatric focal epileptic syndromes may be considered as part of the electroclinical spectrum of CSWS [101, 102].…”

Section: Related Conditionsmentioning

confidence: 99%

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

Fernández

Chapman

Peters

et al. 2013

Epilepsy Research and Treatment

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Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized in most patients by (1) difficult to control seizures, (2) interictal epileptiform activity that becomes prominent during sleep leading to an electroencephalogram (EEG) pattern of electrical status epilepticus in sleep (ESES), and (3) neurocognitive regression. In this paper, we will summarize current epidemiological, clinical, and EEG knowledge on CSWS and will provide suggestions for treatment. CSWS typically presents with seizures around 2–4 years of age. Neurocognitive regression occurs around 5-6 years of age, and it is accompanied by subacute worsening of EEG abnormalities and seizures. At approximately 6–9 years of age, there is a gradual resolution of seizures and EEG abnormalities, but the neurocognitive deficits persist in most patients. The cause of CSWS is unknown, but early developmental lesions play a major role in approximately half of the patients, and genetic associations have recently been described. High-dose benzodiazepines and corticosteroids have been successfully used to treat clinical and electroencephalographic features. Corticosteroids are often reserved for refractory disease because of adverse events. Valproate, ethosuximide, levetiracetam, sulthiame, and lamotrigine have been also used with some success. Epilepsy surgery may be considered in a few selected patients.

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Section: Related Conditionsmentioning

confidence: 99%

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

Fernández

Chapman

Peters

et al. 2013

Epilepsy Research and Treatment

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“…Patients were diagnosed as being affected by ICOEs based on the following inclusion criteria 16 : (1) onset during developmental ages (between 3 and 15 years); (2) uneventful medical history, normal psychomotor development and neurologic examination, normal brain MRI; (3) EEG showing normal background activity with spikes or spike-wave discharges over the occipital lobe(s) activated by eye closure, drowsiness, and sleep; and (4) ictal manifestations including visual symptoms, eye and head deviation, ictal vomiting, autonomic seizures, postictal headache and impairment of consciousness. 6 The coexistence of rolandic spikes during scalp EEG was not an exclusion criterion. The recruited patients were administered a general intelligence evaluation (The Wechsler Intelligence Scale for Children-Fourth Edition [WISC-IV]) 17 1-6 months prior to fMRI recordings (mean AE SD=4.1 AE 1.8 months).…”

Section: Patientsmentioning

confidence: 99%

“…2 The pathophysiology underlying this seizure predisposition is unknown, but it is hypothesized that one or several genetic defects or early developmental lesions may disrupt the normal maturation of neuronal networks, leading to a diminished seizure threshold during development. 2,6 It has been proposed that the clinical expression of BCSSS reflects the main location of the hyperexcitable network, which is represented by the lower rolandic cortex in BECTS and by the posterior cortex in ICOEs. 30,31 The results of the present work appear to support this hypothesis.…”

Section: Overlap Of Icoes With Other Idiopathic Epilepsy Syndromesmentioning

confidence: 99%

“…5 ICOEs are therefore hypothesized to represent a neurobiologic spectrum with overlapping electroclinical features and outcomes. 2,5,6 Seizures in ICOEs are thought to be the expression of cortical, mainly occipital, hyperexcitability. Dysfunction of parietooccipital and temporooccipital regions is generally considered to be the basis for the electroclinical and neuropsychological features of ICOEs.…”

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confidence: 99%

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Extrastriate visual cortex in idiopathic occipital epilepsies: The contribution of retinotopic areas to spike generation

et al. 2016

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SUMMARYObjectives: To provide insight into the pathophysiology of idiopathic childhood occipital epilepsies (ICOEs), by mapping the contribution of retinotopic visual areas to the generation and sustainment of epileptic activity. Methods: Thirteen patients affected by ICOEs (mean age = 10.9 years) underwent a video electroencephalography-functional magnetic resonance imaging (EEGfMRI) study. A flexible-related fMRI analysis was applied to estimate the shape of the blood oxygen level-dependent (BOLD) response in each patient. Secondlevel analysis was performed using the interictal EEG discharge (IED)-specific response shape for the ICOE group. The resulting fMRI t-maps were warped to the Population-Average, Landmark-and Surface-based (PALS)-B12 atlas in Caret. For localization purposes, functional results were plotted and compared against 19 retinotopic areas for each hemisphere. A correlation analysis was performed between the hemodynamic maps and electroclinical variables. Results: The shape of the group-averaged hemodynamic response in ICOE patients showed an earlier time-to-peak and a more pronounced undershoot than the canonical hemodynamic response function (HRF). The random-effect analysis showed positive hemodynamic changes in the bilateral temporooccipital network. With regard to the retinotopic subdivision of the visual cortex, the primary visual area was consistently spared. Conversely, an extensive involvement of the occipitotemporal cortex, including the fusiform gyrus, and the occipitoparietal areas was observed. Moreover, a linear relationship was detected between the occipital spike-density and BOLD increases at the postcentral gyrus and temporooccipital cortex. Significance: Our data indicate that both the ventral and dorsal visual pathways are involved in spike generation in ICOEs, to extents that vary between patients, and reinforce the concept of benign childhood seizure susceptibility syndrome as a substrate for ICOEs. Finally, these results underscore the need for appropriate neuropsychological testing in these children, aimed at revealing selective impairments in functions subserved by both visual pathways.

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“…Os estudos epidemiológicos apontam para taxas de incidência que variam de 4,7 a 21 casos em 100.000 indivíduos (Cavazzuti, 1980;Chahine & Mikati, 2006;Eriksson & Koivikko, 1997;Berg, Shinnar, Levy, & Testa, 1999;Larsson & Eeg-Olofsson, 2006;Heijbel & Bohman, 1975;Sidenvall, Forsgren, & Heijbel, 1996;Sánchez Fernández & Loddenkemper, 2012).…”

Section: A Epilepsia Benigna Da Infância Com Paroxismos Centrotemporaisunclassified

Epilepsia Benigna da Infância com Paroxismos Centrotemporais - delineamento do perfil executivo

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Aos meus pais, Cida e Antonio, pelo amor incondicional e pelo apoio em todos os momentos da minha vida. Essa dissertação foi construída por vocês e para vocês. Por me ensinarem que, em tudo o que se fizer, tem que ter coração, garra, persistência e dedicação. Por me ensinarem (e me mostrarem!) o valor do amor e do trabalho. A vocês toda a minha gratidão e o meu amor, eternos.Aos meus irmãos, Marco, Andréa e Luciana, e aos meus primos, Renato, Patrícia e Vicenzo, pelo carinho e pelo cuidado.À minha irmã-amiga, Luciana, pelo amor e por se fazer presente sempre em minha vida, mesmo com a distância geográfica. Pelo modelo de mulher, força, garra e psicóloga. Por ser meu porto seguro, junto com o papai e com a mamãe.À minha amiga-irmã, Renata, pela parceria completa, pela presença em meus dias, por compartilhar tantos momentos comigo, profissionais e pessoais. Por ser minha irmã de alma e de vida. Pelos ensinamentos. Pela sintonia e reciprocidade.Ao meu namorado, Victor, pelo companheirismo e pelo cuidado. Pela presença tão confortante e pelo carinho. Por ter entendido nos momentos em que não pude estar presente, para realizar esse trabalho. Você é incrível ("e nunca deixe que digam o contrário!").Ao meu amado Fofinho, pela companhia incansável nas noites (e dias, e madrugadas...) de trabalho. Por me acompanhar há 13 anos em minhas jornadas de estudo, de trabalho e de vida, sempre pertinho de mim.Aos meus amigos, Mariana e Lucas, pelo apoio tão carinhoso e pelas conversas, que fizeram parte do meu crescimento. Pela doçura e pela compreensão, tão raros nesse mundo. À mestre, professora e amiga, Camila, pelos ensinamentos, desde os meus 17 anos, com tanta paixão e generosidade que transbordam -e transbordaram em mim! Obrigada por ter me apresentado a Neuropsicologia, a pesquisa, a estatística e, acima de tudo, o amor a tudo isso e aos pacientes. Amo vocês! AgradecimentosA todos os meus pacientes e clientes, em especial às crianças, adolescentes e pais que participaram desse trabalho. Sem vocês, nada disso seria possível. Vocês foram (e são!) a maior motivação de todo o meu esforço e dedicação, do início ao fim. O conhecimento vem, antes de tudo, do contato com vocês e das experiências que compartilhamos.À minha orientadora, Profa. Dra. Kette Dualibi Ramos Valente, pela orientação, dedicada e disponível, e pelos ensinamentos sempre sábios e valorosos. Essa dissertação é fruto de um trabalho árduo e constante, o qual sem a sra. não teria sido possível.À minha co-orientadora, Dra. Patrícia Rzezak, pelas oportunidades, pela disponibilidade, pelo conhecimento compartilhado e pelas inúmeras contribuições inteligentes.Às Profas. Dras. Marilisa Mantovani Guerreiro e Maria Augusta Montenegro, pela generosidade em compartilhar conosco alguns de seus pacientes e pela inspiração ao longo de todo o trabalho.À Mel e à Cecília Canalle, por toda a ajuda no recrutamento dos participantes para esse estudo.A todos os colegas pós-graduandos do Laboratório de Neurofisiologia, pelas vivências em comum, pelo compartilhamento de experiências ...

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Pediatric Focal Epilepsy Syndromes

Cited by 22 publications

References 217 publications

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

Extrastriate visual cortex in idiopathic occipital epilepsies: The contribution of retinotopic areas to spike generation

Epilepsia Benigna da Infância com Paroxismos Centrotemporais - delineamento do perfil executivo

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