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Epilepsy syndromes of childhood with sleep activation: Insights from functional imaging
1Department of Pediatric Neurology, CHU d'Angers and Laboratoire Angevin de Recherche en Ingénierie des Systèmes (LARIS), Université d'Angers, France.
Insights
Functional neuroimaging reveals altered brain activity in childhood epilepsy syndromes with sleep activation. These changes, linked to interictal epileptiform discharges, impact cognition but require further study regarding timing and treatment effects.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Epileptology
Background:
- Childhood epilepsy syndromes with sleep activation, ranging from benign childhood epilepsy with centrotemporal spikes (BECTS) to epileptic encephalopathy (EE) with continuous spike and waves during slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS), exhibit complex functional changes.
- Existing functional imaging studies present inconsistent findings due to varying neuroimaging techniques and clinical phenotype variability.
Purpose of the Study:
- To review and synthesize findings from functional imaging studies in childhood epilepsy syndromes with sleep activation.
- To elucidate the relationship between interictal epileptiform discharges (IEDs), functional brain disturbances, and cognitive impact.
Main Methods:
- Systematic review of functional imaging studies (e.g., PET, fMRI) in pediatric epilepsy syndromes with sleep activation.
- Analysis of regional glucose metabolism, brain activation patterns, and functional connectivity.
Main Results:
- Consistent findings include increased metabolism in epileptogenic zones and decreased metabolism or connectivity in default mode network regions.
- Functional changes can be transient, correlating with IEDs, or permanent.
- More severe phenotypes, such as EE with CSWS, show more pronounced functional disturbances.
Conclusions:
- Functional imaging supports the concept that IEDs negatively impact cognition in these epilepsy syndromes.
- Further research is needed to clarify the temporal relationship between IEDs and functional changes, neuropsychological correlates, and the effects of anti-epileptic treatments.
Abstract:
In epilepsy syndromes of childhood with sleep activation, defined as the spectrum of epileptic conditions going from classical benign childhood epilepsy with centrotemporal spikes (BECTS) to epileptic encephalopathy (EE) with continuous spike and waves during slow-wave sleep (CSWS) including Landau-Kleffner syndrome (LKS), a lot of functional imaging studies have been performed so far, leading to results that are not always consistent, related to the technique of neuroimaging performed and to the variability of the clinical phenotype. Most consistent findings are, depending of the method used, activations or increased regional glucose metabolism in the epileptogenic regions, and deactivations, hypometabolism or decreased functional connectivity in cortical regions that belong to the default mode network. Functional changes are either transitory, temporally related to the occurrence of interictal epileptiform discharges (IED), or permanent, persisting across IED-free periods. Some studies have shown that the more severe phenotype, i.e. EE with CSWS, displays the more profound functional disturbances. Taken together, functional imaging studies support the concept that IED impact cognition in epilepsy syndromes of childhood with sleep activation. However, the precise chronology between the occurrence of IED and the functional disturbances, the neuropsychological correlates of the functional disturbances, and the effects of the anti-epileptic treatments on IED, functional disturbances and cognition need to be further studied.
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