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

Mandatory seizures

Eyelid myoclonias occur, these are brief, repetitive, often rhythmic, fast (4-6 Hz) myoclonic jerks of the eyelids with simultaneous upward deviation of the eyeballs and extension of the head. Seizures are brief (<6 seconds) but frequent (multiple times a day) and resistant to treatment. Eyelid myoclonias are induced by eye closure. All patients are photosensitive, resulting in light induction of seizures in daily life e.g. on going into sunlight. Eyelid myoclonic status epilepticus may occur (seen in up to one fifth of patients, repetitive and discontinuous episodes of eyelid myoclonias with mild impairment of awareness and responsiveness).

Eyelid myoclonias may or may not be associated with impairment in awareness, absences with eyelid myoclonia. If absences with eyelid myoclonias occur, the impairment in awareness is usually mild.

May have

Febrile seizures (seen in 10%)
Generalized tonic-clonic seizures - these are infrequent and seen in older patients (often provoked by sleep deprivation, alcohol and visual stimulation)
Myoclonic seizures - uncommon, infrequent (some authors consider myoclonic seizures exclusionary)
Typical Absence Seizures - uncommon, infrequent