Ictal and interictal electroencephalographic findings can contribute to early diagnosis and prompt treatment in KCNQ2-associated epileptic encephalopathy

Lee, Inn‐Chi; Chang, Ming-Yuh; Liang, Jao‐Shwann; Chang, Ting-Wei

doi:10.1016/j.jfma.2020.08.014

Cited by 14 publications

(13 citation statements)

References 36 publications

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“…Ictal EEG outcome associated with various etiologies are rarely reported. The detection of the same pattern on aEEG and EEG monitoring suggests that this is a distinct aEEG seizure pattern in specific etiology like KCNQ2 ( 13 , 14 ). that can facilitate early treatment, including oxcarbazepine and phenytoin.…”

Section: Introductionmentioning

confidence: 79%

Amplitude Integrated Electroencephalography and Continuous Electroencephalography Monitoring Is Crucial in High-Risk Infants and Their Findings Correlate With Neurodevelopmental Outcomes

et al. 2021

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Background: To evaluate seizure diagnosis in sick infants in the neonatal intensive care unit (NICU) based on electroencephalography (EEG) monitoring combined with amplitude integrated electroencephalography (aEEG).Methods: We retrospectively reviewed EEG and aEEG findings and determined their correlations with neurodevelopmental outcomes at the age of >1 year in 65 patients with diagnosed seizures, encephalopathy, or both.Results: Seizure identification rate was 43.1%. The rate in nonstructural groups (hypocalcemic, hypoglycemic, and genetic seizures) was 71.4%, which was higher (p < 0.05) than the rate of 35.3% of structural brain lesion group [hypoxic–ischemic encephalopathy (HIE) and congenital brain structural malformation]. The aEEG background correlating with neurodevelopmental outcomes had 70.0% positive prediction value (PPV), 65.5%% negative prediction value (NPV), 67.7% specificity, and 67.9% sensitivity (p < 0.005). The aEEG background strongly (PPV, 93.8%; p < 0.005) correlated with the outcomes in HIE. For genetic seizures, the detected rate was high. The ictal recordings for the nonstructural seizures revealed downflected on the aEEG background initially, which differed from the structural lesion.Conclusions: EEG monitoring combined with aEEG can detect seizures, facilitating early treatment. EEG changes during seizures could exhibit delta-theta waves with or without clinical seizures in patients with brain lesions. In non-structural etiologies (hypocalcemic and KCNQ2 seizures), aEEG initially exhibited lower background during seizures that could aid in differentiating these EEG changes from those of other etiologies. The aEEG background was correlated with neurodevelopmental outcome and exhibited high PPV but not NPV in neonatal HIE.

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Section: Introductionmentioning

confidence: 79%

Amplitude Integrated Electroencephalography and Continuous Electroencephalography Monitoring Is Crucial in High-Risk Infants and Their Findings Correlate With Neurodevelopmental Outcomes

et al. 2021

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“…Epileptogenic areas were identified by a conventional EEG in an 11‐year‐old non‐critically ill patient with frontal lobe epilepsy and then monitored with aEEG for days, allowing clinicians to detect frequent seizures and titrate treatment 26 . In a series of patients with genetic epilepsy, the seizure pattern showed a specific triphasic shape that differed from the typical seizure patterns usually observed in an aEEG 30 …”

Section: Resultsmentioning

confidence: 99%

“…26 In a series of patients with genetic epilepsy, the seizure pattern showed a specific triphasic shape that differed from the typical seizure patterns usually observed in an aEEG. 30 Being able to display the long-term trend of cerebral activity makes it possible to identify seizures in aEEG tracings. In a retrospective analysis in our PICU, aEEG helped staff to detect epileptic states more frequently than clinical observation on its own or intermittent conventional EEG.…”

Section: Seizuresmentioning

confidence: 99%

aEEG as a useful tool for neuromonitoring in critically ill children – Current evidence and knowledge gaps

2020

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Aim Amplitude‐integrated electroencephalography (aEEG) is used in children beyond neonatal age, but systematic investigations have been lacking. This mini‐review summarised aEEG studies on children aged one month to 18 years, evaluated the usefulness of aEEG and identified knowledge gaps or limitations. Methods We searched the PubMed database for articles published in English up to September 2020, and 23 papers were identified. Results aEEG was frequently used to compensate for the absence of continuous full‐channel EEG monitoring, particularly for detecting seizures. Interpreting background patterns was based on neonatal classifications, as reference values for older infants and children are lacking. It is possible that aEEG could predict outcomes after paediatric cardiac arrests and other conditions. Gaps in our knowledge exist with regard to normal values in healthy children and the effects of sedation on aEEG background patterns in children. Conclusion The main application of aEEG was detecting and treating paediatric seizures. Further research should determine reference values and investigate the potential to predict outcome after critical events or in acute neurological disease. It is likely that aEEG will play a role in paediatric critical care in the future.

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“…Interictal VEEG is useful for predicting long-term prognosis in our study. An article also revealed the ictal and interictal amplitude-integrated electroencephalogram (AEEG) and EEG play a certain role in the diagnosis of KCNQ2-related disorder early, and showed diversity characteristics of EEG at different variant locations [19] .…”

Section: Discussionmentioning

confidence: 99%

“…Limited by number of cases, few studies focus on the relationship between clinical features, treatment and prognosis of KCNQ2-related disorder [18][19] . We are seeking for some factors in neonatal period that can predict the long-term outcome, such as: interictal video-electro-encephalography (VEEG), date of onset, variant location, and so on.…”

Section: Introductionmentioning

confidence: 99%

Early Initial Video-Electro-Encephalography Combined With Variant Location Predict Prognosis of KCNQ2-Related Disorder

Dou

Chen

et al. 2021

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Background: The clinical features of KCNQ2-related disorders range from benign familial neonatal seizures 1 to early infantile epileptic encephalopathy 7. The genotype-phenotypic association is difficult to establish. Objective: To explore potential factors in neonatal period that can predict the prognosis of neonates with KCNQ2-related disorder. Methods: Neonates with KCNQ2-related disorder were retrospectively enrolled in our study in Children’s Hospital of Fudan University in China from Jan 2015 to Mar 2020. All patients were older than age of 12 months at time of follow-up, and assessed by Bayley Scales of Infant and Toddler Development-Third Edition (BSID-III) or Wechsler preschool and primary scale of intelligence-fourth edition (WPPSI-Ⅳ), then divided into three groups based on scores of BSID-III or WPPSI-Ⅳ: normal group, mild impairment group, encephalopathy group. We collected demographic variables, clinical characteristics, neuroimaging data. Considered variables include gender, gestational age, birth weight, age of the initial seizures, early interictal VEEG, variant location, delivery type. Variables predicting prognosis were identified using multivariate ordinal logistic regression analysis. Results: A total of 52 neonates were selected in this study. Early interictal video-electro-encephalography (VEEG) (OR=2.77; 95%CI: 1.20～4.34, P=0.001), and variant location(OR=2.77, 95%CI: 0.03～5.50, P=0.048) were independent risk factors for prognosis (p<0.05). Conclusions: The integration of early initial VEEG and variant location can predict prognosis. An individual whose KCNQ2 variant located in voltage sensor, the pore domain, with worse early initial VEEG background, often had an adverse outcome.

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Ictal and interictal electroencephalographic findings can contribute to early diagnosis and prompt treatment in KCNQ2-associated epileptic encephalopathy

Cited by 14 publications

References 36 publications

Amplitude Integrated Electroencephalography and Continuous Electroencephalography Monitoring Is Crucial in High-Risk Infants and Their Findings Correlate With Neurodevelopmental Outcomes

Amplitude Integrated Electroencephalography and Continuous Electroencephalography Monitoring Is Crucial in High-Risk Infants and Their Findings Correlate With Neurodevelopmental Outcomes

aEEG as a useful tool for neuromonitoring in critically ill children – Current evidence and knowledge gaps

Early Initial Video-Electro-Encephalography Combined With Variant Location Predict Prognosis of KCNQ2-Related Disorder

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