What are DEEs?
Developmental and epileptic encephalopathies, or DEEs, are types of rare seizure disorders. They most often begin in infancy and are associated with seizures of long duration and major developmental delays.1 The term DEE refers to when brain functions are influenced by seizure activity.2
Developmental Encephalopathy (DE)
This is one type of DEE. It refers to those with non-progressive diseases with cognitive impairment and co-existing epilepsy. In these cases, the seizure activity has minimal or no effect on function.2
Epileptic Encephalopathy (EE)
This term is for patients whose seizure activity has a direct impact on thought processes. Most can revert back to their normal or near normal baseline cognitive state with effective treatment.2
DEEs are rare and also challenging to diagnose and treat. In fact, most are resistant to initial treatment and other treatments are often required. DEEs include, but are not limited to the conditions below:1
- Lennox-Gastaut Syndrome
- Dravet Syndrome
- Tuberous Sclerosis Complex
- Epilepsy of Infancy with Migrating Focal Seizures (EIMFS)
- Infantile Spasms Syndrome (ISS)
- Gelastic Seizures with Hypothalamic Hamartoma
- Myoclonic-Atonic Epilepsy
- Lafora Disease
- Several Syndromes Involving Specific Genes (eg, KCNQ2, CDKL5, PCDH19, etc)
References
- Epilepsy Foundation. Developmental and Epileptic Encephalopathy (DEE). March 1, 2021. Accessed August 20, 2024. https://www.epilepsy.com/what-is-epilepsy/syndromes/developmental-and-epileptic-encephalopathy
- Raga S, Specchio N, Rheims S, Wilmshurst JM. Developmental and epileptic encephalopathies: Recognition and approaches to care. Epileptic Disord. 2021;23:40-52. doi:10.1684/epd.2021.1244
