EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patterns
Angelman syndrome (AS) is a genetic disorder caused by a mutation in the maternal copy of the UBE3A gene and characterized by typical clinical manifestations (such as mental retardation, difficulty walking, and laughter) and specific changes on the electroencephalogram (EEG).The aim of this study wa...
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| Format: | Article |
| Language: | Russian |
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ABV-press
2021-07-01
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| Series: | Русский журнал детской неврологии |
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| Online Access: | https://rjdn.abvpress.ru/jour/article/view/361 |
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| author | G. V. Kuzmich M. Yu. Bobylova K. Yu. Mukhin O. A. Pylaeva L. Yu. Glukhova A. S. Bagdasaryan A. Yu. Zakharova |
| author_facet | G. V. Kuzmich M. Yu. Bobylova K. Yu. Mukhin O. A. Pylaeva L. Yu. Glukhova A. S. Bagdasaryan A. Yu. Zakharova |
| author_sort | G. V. Kuzmich |
| collection | DOAJ |
| description | Angelman syndrome (AS) is a genetic disorder caused by a mutation in the maternal copy of the UBE3A gene and characterized by typical clinical manifestations (such as mental retardation, difficulty walking, and laughter) and specific changes on the electroencephalogram (EEG).The aim of this study was to analyze age-specific characteristics of the main EEG patterns, including high-amplitude frontal delta activity with spikes, slow-wave delta-theta activity with spikes in the posterior regions, and diffuse continuous rhythmic theta activity. In addition to that, we assessed the frequency of a rare and highly specific for AS EEG pattern: notched slow waves.We have identified and described additional criteria for EEG during sleep: high index of pathological slow-wave activity and the ratio of pathological slow-wave activity index to epileptiform activity index during sleep. We also analyzed all EEG patterns at the age most significant for the detection of this syndrome (up to 3 years) and their age-specific dynamics.We covered the frequency and characteristics of EEG patterns rare in AS patients, such as three-phase bifrontal delta waves, reactive pathological activity in the posterior areas, EEG patterns of focal seizures originating from the posterior areas, benign epileptiform discharges of childhood, and migrating continuous slow-wave activity.We analyzed the differences between main EEG patterns in AS and frontal and occipital intermittent rhythmic delta activity (fIRDA and OIRDA patterns). |
| first_indexed | 2024-04-10T01:31:14Z |
| format | Article |
| id | doaj.art-27b9cc1a55904a288015b636930d07e7 |
| institution | Directory Open Access Journal |
| issn | 2073-8803 2412-9178 |
| language | Russian |
| last_indexed | 2024-04-10T01:31:14Z |
| publishDate | 2021-07-01 |
| publisher | ABV-press |
| record_format | Article |
| series | Русский журнал детской неврологии |
| spelling | doaj.art-27b9cc1a55904a288015b636930d07e72023-03-13T09:30:12ZrusABV-pressРусский журнал детской неврологии2073-88032412-91782021-07-01161-2425710.17650/2073-8803-2021-16-1-2-42-57241EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patternsG. V. Kuzmich0M. Yu. Bobylova1K. Yu. Mukhin2O. A. Pylaeva3L. Yu. Glukhova4A. S. Bagdasaryan5A. Yu. Zakharova6ООО «Институт детской неврологии и эпилепсии им. Свт. Луки»; ГБУЗ «Научно-практический центр психического здоровья детей и подростков им. Г.Е. Сухаревой» ДЗМООО «Институт детской неврологии и эпилепсии им. Свт. Луки»; ООО «Институт детской и взрослой неврологии и эпилепсии им. Свт. Луки»ООО «Институт детской неврологии и эпилепсии им. Свт. Луки»; ООО «Институт детской и взрослой неврологии и эпилепсии им. Свт. Луки»ООО «Институт детской неврологии и эпилепсии им. Свт. Луки»; ООО «Институт детской и взрослой неврологии и эпилепсии им. Свт. Луки»ООО «Институт детской неврологии и эпилепсии им. Свт. Луки»ФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова»ФГБУ «Национальный научно-практический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева»Angelman syndrome (AS) is a genetic disorder caused by a mutation in the maternal copy of the UBE3A gene and characterized by typical clinical manifestations (such as mental retardation, difficulty walking, and laughter) and specific changes on the electroencephalogram (EEG).The aim of this study was to analyze age-specific characteristics of the main EEG patterns, including high-amplitude frontal delta activity with spikes, slow-wave delta-theta activity with spikes in the posterior regions, and diffuse continuous rhythmic theta activity. In addition to that, we assessed the frequency of a rare and highly specific for AS EEG pattern: notched slow waves.We have identified and described additional criteria for EEG during sleep: high index of pathological slow-wave activity and the ratio of pathological slow-wave activity index to epileptiform activity index during sleep. We also analyzed all EEG patterns at the age most significant for the detection of this syndrome (up to 3 years) and their age-specific dynamics.We covered the frequency and characteristics of EEG patterns rare in AS patients, such as three-phase bifrontal delta waves, reactive pathological activity in the posterior areas, EEG patterns of focal seizures originating from the posterior areas, benign epileptiform discharges of childhood, and migrating continuous slow-wave activity.We analyzed the differences between main EEG patterns in AS and frontal and occipital intermittent rhythmic delta activity (fIRDA and OIRDA patterns).https://rjdn.abvpress.ru/jour/article/view/361синдром ангельманаген ube3aэпилепсияэлектроэнцефалографиявидео-ээг-мониторингзубчатая дельта-активностьпаттерн дельта-тета-ортдоброкачественные эпилептиформные паттерны детства |
| spellingShingle | G. V. Kuzmich M. Yu. Bobylova K. Yu. Mukhin O. A. Pylaeva L. Yu. Glukhova A. S. Bagdasaryan A. Yu. Zakharova EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patterns Русский журнал детской неврологии синдром ангельмана ген ube3a эпилепсия электроэнцефалография видео-ээг-мониторинг зубчатая дельта-активность паттерн дельта-тета-орт доброкачественные эпилептиформные паттерны детства |
| title | EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patterns |
| title_full | EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patterns |
| title_fullStr | EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patterns |
| title_full_unstemmed | EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patterns |
| title_short | EEG findings in patients with angelman syndrome. Notched slow waves and age-specific characteristics of the main EEG patterns |
| title_sort | eeg findings in patients with angelman syndrome notched slow waves and age specific characteristics of the main eeg patterns |
| topic | синдром ангельмана ген ube3a эпилепсия электроэнцефалография видео-ээг-мониторинг зубчатая дельта-активность паттерн дельта-тета-орт доброкачественные эпилептиформные паттерны детства |
| url | https://rjdn.abvpress.ru/jour/article/view/361 |
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