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Early identification of epileptic encephalopathy with continuous spikes-and-waves during sleep: A case-control study
Camille Desprairies1, Blandine Dozières-Puyravel2, Adina Ilea2
1AP-HP, Hôpital Robert Debré, Service de Neurologie Pédiatrique, 75019, Paris, France; Université Paris Diderot, Sorbonne Paris Cité, INSERM UMR1141, 75019, Paris, France.
Insights
Early recognition of epileptic encephalopathy with continuous spikes-and-waves during sleep (EE-CSWS) is crucial. However, no specific clinical or EEG signs predict EE-CSWS after the first seizure.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neurophysiology
Background:
- Epileptic encephalopathy with continuous spikes-and-waves during sleep (EE-CSWS) is a rare childhood epilepsy.
- EE-CSWS presents with cognitive, behavioral, and psychiatric decline, seizures, and characteristic EEG patterns.
- Early diagnosis and treatment are vital for improving outcomes in EE-CSWS.
Purpose of the Study:
- To investigate early clinical and electroencephalographic (EEG) indicators of EE-CSWS following the initial seizure.
- To determine if predictive signs exist for EE-CSWS development after a first seizure.
Main Methods:
- A retrospective case-control study was conducted.
- Ten EE-CSWS patients with initial EEG recordings were matched with ten control patients.
- EEG recordings from the first seizure were analyzed for predictive features.
Main Results:
- No specific clinical or EEG features at the time of the first seizure were found to predict the later development of EE-CSWS.
- Cases showed a higher frequency of multiple seizure types and seizure worsening during follow-up compared to controls.
Conclusions:
- Predictive clinical or EEG markers for EE-CSWS are not identifiable at the time of the first seizure.
- Multiple seizure types and worsening seizures may serve as clinical red flags for identifying potential EE-CSWS cases.
Abstract:
Epileptic encephalopathy with continuous spikes-and-waves during sleep (EE-CSWS) is a rare childhood epilepsy syndrome characterized by a regression in cognitive, behavioral and psychiatric functioning, seizures and a specific electroencephalographic pattern. An early recognition and an appropriate treatment might play a key role in the outcome of this epileptic encephalopathy. We conducted a case-control study to evaluate if there is any clinical or electroencephalographic sign suggestive of EE-CSWS after the first seizure. We retrospectively identified 10 EE-CSWS patients with available EEG recordings at time of the first seizure. We matched them with 10 controls from our first seizure clinics. All EEG recording were analyzed for the study. We did not find any clinical or EEG features that would suggest later development of EE-CSWS. As reported by others, the occurrence of multiple seizures types and a seizure worsening during the follow-up is more frequent in the cases than in the controls. These clinical criteria might be used as a red flag in clinical practice to identify the very few patients with EE-CSWS among the frequent patients with BECTS.
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