INFANTILE EPILEPTIC SPASMS SYNDROME (IESS)

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
Encephalopathy
Epilepsy imitators

INFANTILE EPILEPTIC SPASMS SYNDROME (IESS)

Background/Interictal EEG

The background EEG may show focal or diffuse slowing slowing.

CAUTION The EEG background can be normal early after onset of epileptic spasms or abnormality may only be seen in sleep or on wakening.

Interictal

The background and interictal EEG may be highly disorganized with high voltage irregular slow waves intermixed with multifocal spikes and polyspikes, this is termed 'hypsarrhythmia'. Some patients may have focal or multifocal discharges without hypsarrhythmia.

eeg
Example of hypsarrhythmia

Activation

EEG abnormality is enhanced in drowsiness, in sleep and on wakening.

Ictal

Epileptic spasms are most commonly accompanied by a high voltage triphasic sharp or slow wave followed by low amplitude fast activity and voltage attenuation.