EPILEPSY WITH MYOCLONIC-ATONIC SEIZURES

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
Epilepsy Classification
Generalized Epilepsy
Focal Epilepsy
Generalized and Focal Epilepsy
Unknown Epilepsy
Epilepsy Syndromes
Neonatal/Infantile
- Self-limited neonatal seizures and Self-limited familial neonatal epilepsy
- Self limited familial and non-familial infantile epilepsy
- Early myoclonic encephalopathy
- Ohtahara syndrome
- West syndrome
- Dravet syndrome
- Myoclonic epilepsy in infancy
- Epilepsy of infancy with migrating focal seizures
- Myoclonic encephalopathy in non-progressive disorders
- Febrile seizures plus, genetic epilepsy with febrile seizures plus
Childhood
- Epilepsy with myoclonic-atonic seizures
- Epilepsy with eyelid myoclonias
- Lennox-Gastaut syndrome
- Childhood absence epilepsy
- Epilepsy with myoclonic absences
- Panayiotopoulos syndrome
- Childhood occipital epilepsy (Gastaut type)
- Photosensitive occipital lobe epilepsy
- Childhood epilepsy with centrotemporal spikes
- Atypical childhood epilepsy with centrotemporal spikes
- Epileptic encephalopathy with continuous spike-and-wave during sleep
- Landau-Kleffner syndrome
- Autosomal dominant nocturnal frontal lobe epilepsy
Adolescent/Adult
- Juvenile absence epilepsy
- Juvenile myoclonic epilepsy
- Epilepsy with generalized tonic-clonic seizures alone
- Autosomal dominant epilepsy with auditory features
- Other familial temporal lobe epilepsies
Any Age
- Familial focal epilepsy with variable foci
- Reflex epilepsies
- Progressive myoclonus epilepsies
Epilepsy Etiologies
Genetic Etiology
- Chromosomal abnormalities
- Gene abnormalities
Structural Etiology
- Malformations of cortical development
- Vascular malformations
- Hippocampal sclerosis
- Hypoxic-Ischemic
- Traumatic brain injury
- Tumors
- Porencephalic Cyst
Metabolic Etiology
Immune Etiology
- Rasmussen syndrome
- Antibody mediated
Infectious Etiology
Unknown Etiology
- Febrile infection related epilepsy syndrome
Epilepsy imitators

EPILEPSY WITH MYOCLONIC-ATONIC SEIZURES

Background

The background may be normal or show generalized slowing. Background bi-parietal theta is usually seen.

eeg
Example of bi-parietal theta.

CAUTION Focal slowing consistently over one area is not seen right arrow consider structural brain abnormality.

Interictal

Generalized spike-and-wave and polyspike-and-wave occur.

CAUTION Focal discharges are not seen right arrow consider structural brain abnormality.

Activation

Intermittent photic stimulation may trigger generalized spike-and-wave and polyspike-and-wave, and myoclonic-atonic seizures.

EEG abnormality is enhanced by sleep deprivation and by sleep. Generalized spike-and-wave often becomes fragmented with sleep deprivation or in sleep. Fragmented generalized spike-and-wave can appear focal or multi-focal but usually is not consistently seen in one area. The morphology of the focal spike-and-wave typically appears similar to the generalized spike-and-wave.

Ictal

The myoclonic component is associated with a generalized spike or polyspike. The atonic component is associated with the aftergoing high voltage slow wave.