Paediatric-tailored modified IC-CoDE approach in non-lesional D/EE-SWAS.

Area of Science:

• Pediatric Neurology
• Neuropsychology
• Epileptology

Background:

• Developmental and/or Epileptic Encephalopathy with Spike-Wave Activation during Slow Sleep (D/EE-SWAS) is a condition affecting children.
• Characterizing cognitive and behavioral profiles is crucial for understanding D/EE-SWAS.
• A multidomain approach is needed to comprehensively assess neurodevelopmental impact.

Purpose of the Study:

• To characterize cognitive and behavioral profiles in children with non-lesional D/EE-SWAS.
• To explore associations between these profiles and clinical features/outcomes.
• To define cognitive-behavioral phenotypes using a modified International Classification of Cognitive Disorders in Epilepsy (IC-CoDE) model.

Main Methods:

• Retrospective analysis of 28 pediatric patients with D/EE-SWAS.
• Standardized neuropsychological battery assessing Language, Visuospatial, Attention-Executive, Behaviour, and Motor domains.
• Exploratory cluster analysis based on a four-graded severity score of neuropsychological impairment.

Main Results:

• Most patients (75%) exhibited attention-executive deficits, followed by language deficits (61%).
• Multidomain impairments were frequent, with 61% showing generalized impairment.
• Three distinct profiles emerged: severe generalized impairments (Cluster 1), predominant executive-behavioral deficits (Cluster 2), and selective cognitive impairments (Cluster 3).
• Cluster 1 had significantly worse long-term functioning and earlier SWAS onset compared to other clusters.

Conclusions:

• A structured, multidomain neuropsychological approach is clinically relevant for D/EE-SWAS.
• Identified cognitive-behavioral phenotypes have prognostic value.
• Earlier D/EE-SWAS onset may correlate with more severe neurodevelopmental disruption.