Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report
Rett syndrome (RTT), a severe neurodevelopmental disorder caused by MECP2 gene abnormalities, is characterized by atypical EEG activity, and its detailed examination is lacking. We combined the comparison of one-time eyes open EEG resting state activity from 32 girls with RTT and their 41 typically...
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MDPI AG
2022-11-01
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| Series: | Journal of Personalized Medicine |
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| Online Access: | https://www.mdpi.com/2075-4426/12/12/1973 |
| _version_ | 1797456896180879360 |
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| author | Galina Portnova Anastasia Neklyudova Victoria Voinova Olga Sysoeva |
| author_facet | Galina Portnova Anastasia Neklyudova Victoria Voinova Olga Sysoeva |
| author_sort | Galina Portnova |
| collection | DOAJ |
| description | Rett syndrome (RTT), a severe neurodevelopmental disorder caused by MECP2 gene abnormalities, is characterized by atypical EEG activity, and its detailed examination is lacking. We combined the comparison of one-time eyes open EEG resting state activity from 32 girls with RTT and their 41 typically developing peers (age 2–16 years old) with longitudinal following of one girl with RTT to reveal EEG parameters which correspond to the RTT progression. Traditional measures, such as epileptiform abnormalities, generalized background activity, beta activity and the sensorimotor rhythm, were supplemented by a new frequency rate index measured as the ratio between high- and low-frequency power of sensorimotor rhythm. Almost all studied EEG parameters differentiated the groups; however, only the elevated generalized background slowing and decrease in our newly introduced frequency rate index which reflects attenuation in the proportion of the upper band of sensorimotor rhythm in RTT showed significant relation with RTT progression both in longitudinal case and group analysis. Moreover, only this novel index was linked to the breathing irregularities RTT symptom. The percentage of epileptiform activity was unrelated to RTT severity, confirming previous studies. Thus, resting EEG can provide information about the pathophysiological changes caused by MECP2 abnormalities and disease progression. |
| first_indexed | 2024-03-09T16:13:25Z |
| format | Article |
| id | doaj.art-f1e601b5cba549568417e27baf408462 |
| institution | Directory Open Access Journal |
| issn | 2075-4426 |
| language | English |
| last_indexed | 2024-03-09T16:13:25Z |
| publishDate | 2022-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Personalized Medicine |
| spelling | doaj.art-f1e601b5cba549568417e27baf4084622023-11-24T16:01:25ZengMDPI AGJournal of Personalized Medicine2075-44262022-11-011212197310.3390/jpm12121973Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case ReportGalina Portnova0Anastasia Neklyudova1Victoria Voinova2Olga Sysoeva3Institute of Higher Nervous Activity and Neurophysiology, Russian Academy of Sciences, Moscow 117485, RussiaInstitute of Higher Nervous Activity and Neurophysiology, Russian Academy of Sciences, Moscow 117485, RussiaVeltischev Research and Clinical Institute for Pediatrics of the Pirogov, Russian National Research Medical University, Ministry of Health of Russian Federation, Moscow 125412, RussiaInstitute of Higher Nervous Activity and Neurophysiology, Russian Academy of Sciences, Moscow 117485, RussiaRett syndrome (RTT), a severe neurodevelopmental disorder caused by MECP2 gene abnormalities, is characterized by atypical EEG activity, and its detailed examination is lacking. We combined the comparison of one-time eyes open EEG resting state activity from 32 girls with RTT and their 41 typically developing peers (age 2–16 years old) with longitudinal following of one girl with RTT to reveal EEG parameters which correspond to the RTT progression. Traditional measures, such as epileptiform abnormalities, generalized background activity, beta activity and the sensorimotor rhythm, were supplemented by a new frequency rate index measured as the ratio between high- and low-frequency power of sensorimotor rhythm. Almost all studied EEG parameters differentiated the groups; however, only the elevated generalized background slowing and decrease in our newly introduced frequency rate index which reflects attenuation in the proportion of the upper band of sensorimotor rhythm in RTT showed significant relation with RTT progression both in longitudinal case and group analysis. Moreover, only this novel index was linked to the breathing irregularities RTT symptom. The percentage of epileptiform activity was unrelated to RTT severity, confirming previous studies. Thus, resting EEG can provide information about the pathophysiological changes caused by MECP2 abnormalities and disease progression.https://www.mdpi.com/2075-4426/12/12/1973EEGRett syndromeMECP2background slowingsensorimotor rhythm |
| spellingShingle | Galina Portnova Anastasia Neklyudova Victoria Voinova Olga Sysoeva Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report Journal of Personalized Medicine EEG Rett syndrome MECP2 background slowing sensorimotor rhythm |
| title | Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report |
| title_full | Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report |
| title_fullStr | Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report |
| title_full_unstemmed | Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report |
| title_short | Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report |
| title_sort | clinical eeg of rett syndrome group analysis supplemented with longitudinal case report |
| topic | EEG Rett syndrome MECP2 background slowing sensorimotor rhythm |
| url | https://www.mdpi.com/2075-4426/12/12/1973 |
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