Abstract
CSWS is an epileptic encephalopathy with multiple clinical manifestations (various seizure types in association with cognitive, motor, and behavioral disturbances) related to a distinct electroencephalography (EEG) pattern characterized by a spike (sharp wave) and (slow) wave activity significantly activated during slow sleep. There are two fundamental aspects that should be considered in the diagnosis of the syndrome: clinical evaluation that should be related to cognitive, motor, behavioral, and language deficits in a previously cognitively normal or delayed child; and an EEG component of usually generalized (near)–continuous spike and wave complexes during a significant proportion of Non-REM sleep. The aim of this study was to examine the changes of neuropsychological functions over a year, at two distinct moments—T1 (2017) and T2 (2018), in an 8-year-old female child diagnosed with CSWS. The patient underwent EEGs to control her condition in which it was possible to verify patterns consistent with CSWS. Within a year, the patient showed a decline in her performance in most of the neuropsychological tests and tasks. The assessed functions were IQ, language, memory, attention, executive functions, and also visuomotor and visuospatial skills.
Resumo
O mal epiléptico do sono é uma encefalopatia epiléptica com múltiplas manifestações clínicas (vários tipos de crises associadas a distúrbios cognitivos, motores e comportamentais) relacionadas a um padrão distinto de eletroencefalografia (EEG) caracterizado por um pico (onda aguda) e atividade de onda (lenta) significativamente ativada durante sono lento. Há dois aspectos fundamentais que devem ser considerados no diagnóstico da síndrome: avaliação clínica que deve estar relacionada a déficits cognitivos, motores, comportamentais e de linguagem em uma criança previamente cognitivamente normal ou com atraso; e um componente de EEG de complexos de pico e onda geralmente generalizados e contínuos durante uma proporção significativa de sono não-REM. O objetivo deste estudo foi examinar as alterações das funções neuropsicológicas ao longo de um ano, em dois momentos separados—T1 (2017) e T2 (2018), em uma criança do sexo feminino de oito anos com diagnóstico de mal epiléptico do sono. A paciente foi submetida a EEGs para controlar sua condição, onde foi possível verificar padrões consistentes com o diagnóstico. No intervalo de um ano, a paciente apresentou um declínio no desempenho na maioria dos testes e tarefas neuropsicológicas. As funções avaliadas foram QI, linguagem, memória, atenção, funções executivas e também habilidades visuomotoras e visuoespaciais.
Resumen
El trastorno del sueño epiléptico es una encefalopatía epiléptica con múltiples manifestaciones clínicas (varios tipos de convulsiones asociadas con trastornos cognitivos, motores y de comportamiento) relacionadas con un patrón distinto de electroencefalografía (EEG) caracterizado por un pico (onda aguda) y actividad de las ondas (lento) activado significativamente durante el sueño lento. Hay dos aspectos fundamentales que deben considerarse en el diagnóstico del síndrome: la evaluación clínica que debe estar relacionada con los déficits cognitivos, motores, conductuales y del lenguaje en un niño cognitivamente normal o retrasado; y un componente EEG de complejos de pico y onda generalmente generalizados y continuos durante una proporción significativa de sueño no REM. El objetivo de este estudio fue examinar los cambios en las funciones neuropsicológicas a lo largo de un año, en dos momentos separados: T1 (2017) y T2 (2018), en una niña de ocho años diagnosticada con trastorno del sueño epiléptico. La paciente se sometió a EEG para controlar su condición, donde fue posible verificar patrones consistentes con el diagnóstico. Dentro de un año, el paciente experimentó una disminución en el rendimiento en la mayoría de las pruebas y tareas neuropsicológicas. Las funciones evaluadas fueron IQ, lenguaje, memoria, atención, funciones ejecutivas y también habilidades visuomotoras y visoespaciales.
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The study was reviewed and approved by the Ethics Committee in Research from the Hospital da Criança Santo Antônio, Brazil (report no. 2.971.248).
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Trivilin, T., Scornavacca, F. & Wagner, G.P. Neuropsychological Assessment of a Continuous Spikes and Waves During Sleep (CSWS) Patient: a Case Study/Description in Childhood. Trends in Psychol. 30, 242–260 (2022). https://doi.org/10.1007/s43076-021-00100-3
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DOI: https://doi.org/10.1007/s43076-021-00100-3
Keywords
- CSWS
- Continuous spikes and waves during sleep
- ESES
- Electrical status epilepticus during sleep
- Childhood
- Neuropsychology
- Cognitive functions