Epileptic spasms with terror during sleep in CDKL5 encephalopathy
Электронный научный архив УРФУ
Информация об архиве | Просмотр оригинала| Поле | Значение | |
| Заглавие |
Epileptic spasms with terror during sleep in CDKL5 encephalopathy
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| Автор |
Melikishvili, G.
Sharkov, A. Gachechiladze, T. Tomenko, T. Pivovarova, A. Volkov, I. Andrade, M. -T. Castellanos, A. Bienvenu, T. Dulac, O. Roisman, G. Gataullina, S. |
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| Тематика |
CHILD
CLONAZEPAM EEG EPILEPSY EPILEPTIC SEIZURES INFANT QUIET SLEEP SLEEP TERROR SLOW-WAVE SLEEP |
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| Описание |
Study Objectives: To describe early diagnostic clues in Cyclin-Dependent Kinase-Like 5 (CDKL5) refractory encephalopathy, to improve treatment strategies. Methods: We retrospectively studied 35 patients (25 females, 10 males) with CDKL5 gene mutations or deletion, focusing on their early seizure semiology, the electroencephalogram (EEG) pattern, the effect of treatment, and developmental outcome. Results: The first seizures were recognizable and consisted of tonic, then clonic, and spasms phases, occurring in sleep at a median age of 6 weeks. Clusters of spasms were observed in quiet sleep or slow-wave sleep (SWS), with screaming, staring, and arms' extension that mimicked sleep terror in 28 of 35 patients (80%). Programmed awakening prevented these spasms in 9 of 16 patients and small doses of clonazepam given at night improved epilepsy in 14 of 23 patients. Conclusions: Peculiar seizures with spasms starting in SWS are an early diagnostic clue in infants with CDKL5 encephalopathy. Sleep video-EEG polygraphy is an easy tool to disclose these early seizures and epileptic spasms in infants during the first months of life while polysomnography is unlikely to give a contribution at that early age. While conventional antiepileptic treatment and corticosteroids are poorly, transiently, or not efficient, therapeutic strategy used for sleep terror could help, although the mechanism of spasms generation in SWS needs to be elucidated. © 2022 The Author(s). Published by Oxford University Press on behalf of Sleep Research Society.
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| Дата |
2024-04-08T11:07:35Z
2024-04-08T11:07:35Z 2022 |
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| Тип |
Article
Journal article (info:eu-repo/semantics/article) Published version (info:eu-repo/semantics/publishedVersion) |
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| Идентификатор |
Melikishvili, G, Sharkov, A, Gachechiladze, T, Tomenko, T, Pivovarova, A, Volkov, I, Andrade, M-T, Castellanos, A, Bienvenu, T, Dulac, O, Roisman, G & Gataullina, S 2022, 'Epileptic spasms with terror during sleep in CDKL5 encephalopathy', SLEEP Advances, Том. 3, № 1, zpac010. https://doi.org/10.1093/sleepadvances/zpac010
Melikishvili, G., Sharkov, A., Gachechiladze, T., Tomenko, T., Pivovarova, A., Volkov, I., Andrade, M-T., Castellanos, A., Bienvenu, T., Dulac, O., Roisman, G., & Gataullina, S. (2022). Epileptic spasms with terror during sleep in CDKL5 encephalopathy. SLEEP Advances, 3(1), [zpac010]. https://doi.org/10.1093/sleepadvances/zpac010 2632-5012 Final All Open Access; Gold Open Access; Green Open Access https://academic.oup.com/sleepadvances/article-pdf/3/1/zpac010/43615653/zpac010.pdf https://academic.oup.com/sleepadvances/article-pdf/3/1/zpac010/43615653/zpac010.pdf http://elar.urfu.ru/handle/10995/131483 10.1093/sleepadvances/zpac010 85174019464 |
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| Язык |
en
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| Права |
Open access (info:eu-repo/semantics/openAccess)
cc-by https://creativecommons.org/licenses/by/4.0/ |
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| Формат |
application/pdf
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| Издатель |
Oxford University Press
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| Источник |
SLEEP Advances
SLEEP Advances |
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