Epileptic Disorders


Developmental and epileptic encephalopathies: recognition and approaches to care Volume 23, numéro 1, February 2021


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1 Paediatric Neurology Division, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, Cape Town, South Africa
2 Neurosciences Institute, University of Cape Town, South Africa
3 Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesu’ Children's Hospital, IRCCS, Rome, Italy
4 Member of the European Reference Network EpiCARE
5 Department of Functional Neurology and Epileptology, Hospices Civils de Lyon and University of Lyon, Lyon, France “Member of the European Reference Network EpiCARE”
6 Lyon's Neurosciences Research Center (INSERM U1028/CNRS UMR5292), Lyon, France
* Correspondence: Jo Wilmshurst Paediatric Neurology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, Cape Town, South Africa

The term “developmental and epileptic encephalopathy” (DEE) refers to when cognitive functions are influenced by both seizure and interictal epileptiform activity and the neurobiological process behind the epilepsy. Many DEEs are related to gene variants and the onset is typically during early childhood. In this setting, neurocognition, whilst not improved by seizure control, may benefit from some precision therapies. In patients with non-progressive diseases with cognitive impairment and co-existing epilepsy, in whom the epileptiform activity does not affect or has minimal effect on function, the term “developmental encephalopathy” (DE) can be used. In contrast, for those patients with direct impact on cognition due to epileptic or epileptiform activity, the term “epileptic encephalopathy” (EE) is preferred, as most can revert to their normal or near normal baseline cognitive state with appropriate intervention. These children need aggressive treatment. Clinicians must tailor care towards individual needs and realistic expectations for each affected person; those with DE are unlikely to gain from aggressive antiseizure medication whilst those with EE will gain. Patients with DEE might benefit from a precision medicine approach in order to reduce the overall burden of epilepsy.

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