DEVELOPMENTAL AND/OR EPILEPTIC ENCEPHALOPATHY WITH SPIKE-WAVE ACTIVATION IN SLEEP (DEE-SWAS, EE-SWAS)

Overview
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Seizure Classification
Generalized onset seizure
- Motor Onset
- Non-Motor Onset
Focal Onset Seizure
- By Onset Feature
- Focal to Bilateral Tonic-Clonic
- Hemispheric lateralization
- Lobar localization
  - Frontal
  - Temporal
  - Parietal
  - Occipital
Unknown Onset Seizure
Neonatal Seizure
Epilepsy Classification
Generalized Epilepsy
Focal Epilepsy
Generalized and Focal Epilepsy
Unknown Epilepsy
Epilepsy Syndromes
Neonate/Infant
- Self-limited
- With developmental and epileptic encephalopathy
Childhood
- Self-limited focal epilepsy syndromes
  - Remission expected in all cases by adolescence
    - Self-limited epilepsy with centrotemporal spikes
    - Self-limited epilepsy with autonomic seizures
  - Remission expected in most cases by adolescence
    - Childhood occipital visual epilepsy
    - Photosensitive occipital lobe epilepsy
- Genetic generalised epilepsy syndromes
  - Epilepsy with eyelid myoclonia
  - Epilepsy with myoclonic absence
- Idiopathic generalised epilepsy syndrome
  - Childhood absence epilepsy
- With developmental and/or epileptic encephalopathy
Variable Age
- Idiopathic generalised epilepsy syndromes
- Focal epilepsy syndromes
- Combined generalised and focal epilepsy syndrome
  - Epilepsy with reading-induced seizures
- With developmental and/or epileptic encephalopathy or progressive neurological deterioration
  - Progressive myoclonus epilepsies
  - Rasmussen syndrome
Epilepsy Etiologies
Genetic Etiologies
- Chromosomal abnormalities
- Gene abnormalities
Structural Etiologies
- Malformations of cortical development
- Vascular malformations
- Hippocampal sclerosis
- Hypoxic-Ischemic
- Traumatic brain injury
- Tumors
- Porencephalic Cyst
Metabolic Etiologies
Immune Etiologies
Infectious Etiologies
Unknown Etiologies
Encephalopathy
Epilepsy imitators

DEVELOPMENTAL AND/OR EPILEPTIC ENCEPHALOPATHY WITH SPIKE-WAVE ACTIVATION IN SLEEP (DEE-SWAS, EE-SWAS)

GENETICS

PATTERN OF INHERITANCE

Inheritance can be monogenic or complex/polygenic.

KNOWN GENES

The major monogenic cause is GRIN2A. Affected individuals have a characteristic speech pattern that may persist into adult life.

FAMILY HISTORY OF SEIZURES/EPILEPSY

A family history of seizures is seen in up to 50% of patients with this syndrome.

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