Mandatory | Alerts | Exclusionary | |
Seizures | Flexor, extensor or mixed epileptic spasms that often occur in clusters | ||
EEG | Interictal:
| Interictal:
| Ictal:
|
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?
| |||
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. |
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