EEG mapping in a child with Rett syndrome

Faienza, C; Capone, Cristiano; Sani, Emanuela; Villani, Daniele; Prati, Giovanni

doi:10.1016/0165-1781(89)90110-8

Cited by 3 publications

(3 citation statements)

References 3 publications

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“…While the higher-frequency activity ranging from 6 to 30 Hz was attenuated similarly in patients with RTT regardless of disease stage, the low frequency activity differentiated between the RTT stages. In contrast to widely reported increased power of low frequency activity in patients with RTT [ 137 , 142 , 143 ], girls with RTT in the active regression period were characterized by lower frontal and central theta and delta activity as compared to typically-developing peers. Decrease in frontal theta activity was so dramatic that about half of the patients had theta power values below the lowest value in the control group, clearly indicative of underlying neuropathology (Figure 3A in [ 139 ]).…”

Section: Resultscontrasting

confidence: 79%

“…Quantitative studies of spontaneous EEG recorded under eyes-open or eyes-closed conditions show the attenuation of alpha and beta band power with concomitant generalized increase of theta and delta activity in RTT [ 137 , 139 , 142 , 143 ]. Moreover, abnormally increased delta activity in girls with RTT was reported also during slow wave sleep (SWS), suggesting that predominance of low-frequency activity might characterize EEG in RTT regardless of the functional state [ 141 ].…”

Section: Resultsmentioning

confidence: 99%

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Reviewing Evidence for the Relationship of EEG Abnormalities and RTT Phenotype Paralleled by Insights from Animal Studies

Smirnov

Stroganova²,

Molholm

et al. 2021

IJMS

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Rett syndrome (RTT) is a rare neurodevelopmental disorder that is usually caused by mutations of the MECP2 gene. Patients with RTT suffer from severe deficits in motor, perceptual and cognitive domains. Electroencephalogram (EEG) has provided useful information to clinicians and scientists, from the very first descriptions of RTT, and yet no reliable neurophysiological biomarkers related to the pathophysiology of the disorder or symptom severity have been identified to date. To identify consistently observed and potentially informative EEG characteristics of RTT pathophysiology, and ascertain areas most worthy of further systematic investigation, here we review the literature for EEG abnormalities reported in patients with RTT and in its disease models. While pointing to some promising potential EEG biomarkers of RTT, our review identify areas of need to realize the potential of EEG including (1) quantitative investigation of promising clinical-EEG observations in RTT, e.g., shift of mu rhythm frequency and EEG during sleep; (2) closer alignment of approaches between patients with RTT and its animal models to strengthen the translational significance of the work (e.g., EEG measurements and behavioral states); (3) establishment of large-scale consortium research, to provide adequate Ns to investigate age and genotype effects.

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Section: Resultscontrasting

confidence: 79%

Section: Resultsmentioning

confidence: 99%

Reviewing Evidence for the Relationship of EEG Abnormalities and RTT Phenotype Paralleled by Insights from Animal Studies

Smirnov

Stroganova²,

Molholm

et al. 2021

IJMS

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“…Na síndrome de Rett, o MC tem demonstrado intenso alentecimento com presença de ondas theta e delta de alta amplitude, quando comparado com grupo controle (Faienza et al, 1989 Na SIDA (Síndrome de Imunodeficiência Adquirida), o MC também tem demonstrado sua importância nas encefalopatias precoces, como no caso de um paciente de 69 anos que estava sendo tratado de depressão. 0 MC conduziu este paciente para uma investigação mais profunda, ao mostrar ondas lentas na regido posterior direita (Duffy, 1989).…”

Section: Outras Doenças Neurológicasunclassified

Mapeamento Cerebral

et al. 1995

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O mapeamento cerebral é uma técnica digital que gera mapas topográficos coloridos da atividade eletrencefalográfica captada sobre o escalpo. O eletrencefalograma após passar por um microcomputador, que realiza a análise quantitativa em várias faixas de freqüência, pode ser visualizado em um monitor colorido (EEG digital). Posteriormente, as épocas do EEG digital são selecionadas para a realização dos mapas, impressão final e arquivo em disquetes. Este método tem demonstrado ser ON em várias doenças neurológicas e psiquiátricas e deve ser realizado sempre em conjunto com o EEG digital. Achamos que dentro de alguns anos os aparelhos de EEG digital/mapeamento cerebral substituam as máquinas que realizam o EEG convencional.

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Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report

Portnova

Neklyudova

Воинова

et al. 2022

JPM

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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.

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EEG mapping in a child with Rett syndrome

Cited by 3 publications

References 3 publications

Reviewing Evidence for the Relationship of EEG Abnormalities and RTT Phenotype Paralleled by Insights from Animal Studies

Reviewing Evidence for the Relationship of EEG Abnormalities and RTT Phenotype Paralleled by Insights from Animal Studies

Mapeamento Cerebral

Clinical EEG of Rett Syndrome: Group Analysis Supplemented with Longitudinal Case Report

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