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Diagnostic criteria for infantile epileptic spasms syndrome

Diagnostic criteria for infantile epileptic spasms syndrome
  Mandatory Alerts Exclusionary
Seizures Flexor, extensor or mixed epileptic spasms that often occur in clusters    
EEG Interictal:
  • Hypsarhythmia, multifocal or focal epileptiform discharges (that might be seen quickly after the spasms onset)
 Interictal:
  • Normal EEG
  • Suppression-burst pattern
 Ictal:
  • Normal EEG during recorded clinical events of suspected spasms
Age at onset 1 to 24 months (while epileptic spasms may begin later, this would not be IESS) Age at onset 1 to 2 months  
Comorbidities Developmental slowing after spasms onset but may be absent early in the course (difficult to determine in a child with existing significant developmental disorders)    
Is MRI or ictal EEG required for diagnosis?
  • An MRI is not required for diagnosis but is highly recommended to evaluate for underlying cause.
  • An ictal EEG is not required for diagnosis provided the interictal study shows hypsarhythmia or epileptiform abnormalities or developmental delay. In the absence of hypsarhythmia or epileptiform anomalies, an ictal recording is required.
Possible evolving syndrome: Infants with preceding brain injury, developmental brain malformations, or specific genetic conditions, including early-infantile DEE, who show significant interictal EEG abnormalities (high amplitude, background slowing, and/or multifocal discharges) should be watched carefully for the development of clinical epileptic spasms. However, the syndrome of IESS cannot be diagnosed prior to onset of the mandatory seizure type.
Syndrome without laboratory confirmation: In resource-limited regions, an interictal EEG is highly recommended. However, if EEG is unavailable, if clear clusters of typical epileptic spasms are witnessed by an experienced clinician (in person or on video recording), with the other clinical mandatory and exclusionary criteria, IESS can be diagnosed.
EEG: electroencephalogram; IESS: infantile epileptic spasms syndrome; MRI: magnetic resonance imaging; DEE: developmental and epileptic encephalopathy.
From: Zuberi SM, Wirrell E, Yozawitz E, et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63:1349. Copyright © 2022 The Authors. Available at: https://onlinelibrary.wiley.com/doi/10.1111/epi.17239 (Accessed on March 22, 2023). Reproduced under the terms of the Creative Commons Attribution License 4.0.
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