EPILEPSY WITH EYELID MYOCLONIAS

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 EYELID MYOCLONIAS

Background

The EEG background is normal.

CAUTION Background generalized slowing is not seen right arrow consider other syndromes.

CAUTION Focal slowing seen consistently over one area right arrow consider structural brain abnormality.

Interictal

Brief bursts of fast (3-6 Hz) generalized polyspike-and-wave.

CAUTION Focal spikes seen consistently in one area right arrow consider structural brain abnormality.

CAUTION A normal EEG is not expected in this syndrome, even when seizures are controlled.

Activation

Eye closure (fixation off sensitivity) and intermittent photic stimulation activates the EEG and often elicits eyelid myoclonias with/without absences. Response to intermittent photic stimulation may be less in older patients and those on medication. EEG abnormality is also facilitated by hyperventilation.

Generalized spike-and-wave often becomes briefer and 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

Eyelid myoclonia is accompanied by high amplitude generalized spike-and-wave or polyspike-and-wave at a frequency of 3-6 Hz. Ictal EEG and clinical events are terminated with complete elimination of light.