developmental and epileptic encephalopathy
A condition where both seizures and underlying pathology contribute to cognitive and behavioral impairment beyond what is expected from seizures alone.
Full Definition
Developmental and epileptic encephalopathy (DEE) represents a spectrum of conditions where epileptic activity and the underlying pathological process both contribute to progressive cognitive and behavioral deterioration. This classification acknowledges that the developmental impairment results not only from frequent seizures but also from the same underlying brain dysfunction that causes the epilepsy. DEE encompasses various syndromes including Dravet syndrome, Lennox-Gastaut syndrome, and infantile epileptic spasms syndrome. Early recognition and treatment are crucial as the condition can lead to significant developmental regression if not properly managed.
Usage
Usage note: Use 'and' rather than 'with' between 'developmental' and 'epileptic' to reflect current ILAE terminology.
In Context
- "The patient's regression in language skills suggested a developmental and epileptic encephalopathy rather than benign childhood epilepsy." — Clinical assessment
- "Genetic testing revealed a mutation consistent with early-onset developmental and epileptic encephalopathy." — Laboratory report