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Diagnostic criteria for myoclonic epilepsy in infancy

Diagnostic criteria for myoclonic epilepsy in infancy
  Mandatory Alerts Exclusionary
Seizures Myoclonic seizures
  • Afebrile generalized tonic-clonic seizure or generalized clonic at time of epilepsy onset
  • Any of the following seizure types:
    • Absence seizures
    • Atonic seizures
    • Epileptic spasms
    • Focal impaired awareness seizures
    • Focal clonic (hemiclonic) seizures
    • Myoclonic-absence seizures
    • Tonic seizures
EEG Normal background
  • Interictal: Lack of generalized spike-wave discharge on sleep recording
  • PPR at low frequency photic stimulation (suggest CLN2 disease)
  • Ictal: Recorded myoclonic event without EEG correlate
  • Interictal:
    • Hypsarhythmia
    • Generalized slow spike-wave (<2.5 Hz)
Age at onset    
  • Age at onset of myoclonic seizures ≤4 months or >3 years
Development at onset  
  • Speech delay at time of diagnosis
  • Moderate to profound ID
  
Neurologic examination  
  • Significant neurologic examination abnormalities, excluding incidental findings
  • Dysmorphism or other congenital anomalies (suggests chromosomal disorder)
Imaging    
  • Significant neuroimaging abnormalities
Other testing (eg, genetics)    
  • Low CSF glucose or pathogenic SLC2A1 variants (Glut1DS)
Course of illness    
  • Neurocognitive regression
Are MRI or ictal EEG required for diagnosis?
  • A nonlesional MRI is required for diagnosis
  • An ictal EEG is not required for diagnosis but should be strongly considered if the interictal sleep recording does not show generalized spike-wave to confirm that myoclonus is epileptic
Syndrome without laboratory confirmation: In resource-limited regions, at a minimum, a sleep EEG showing generalized spike-wave is required to make this diagnosis
EEG: electroencephalography; PPR: photoparoxysmal response; ID: intellectual disability; CSF: cerebrospinal fluid.
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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