Diagnostic criteria for early infantile developmental and epileptic encephalopathy

	Mandatory	Alerts	Exclusionary
Seizures	Tonic and/or myoclonic seizures
EEG	Interictal: Either burst suppression or multifocal discharges Diffuse slowing
Age at onset	Birth to 3 months (adjusted for prematurity)
Development at onset		Normal development at onset, although it is acknowledged that this can be challenging to accurately assess historically
Neurologic examination at onset		Normal neurologic examination, although it is acknowledged that this can be challenging to assess historically or in an infant who has had very frequent seizures and/or received ASMs that may alter their examination
Early comorbidities	Developmental impairment is present prior to or shortly after seizure onset
Course of illness	Abnormal neurodevelopment including intellectual disability
Are MRI or ictal EEG required for diagnosis? An MRI is not required for diagnosis but is strongly recommended to exclude structural causes An ictal EEG is not required in an infant with characteristic clinical features where the interictal EEG shows burst-suppression, multifocal discharges with diffuse slowing
Syndrome without laboratory confirmation: In resource-limited regions, this syndrome cannot be diagnosed without an interictal EEG

EEG: electroencephalography; ASM: antiseizure medication; MRI: magnetic resonance imaging.

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 November 11, 2022). Reproduced under the terms of the Creative Commons Attribution License 4.0.

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Diagnostic criteria for early infantile developmental and epileptic encephalopathy