Seizures | - Focal seizures occur with behavioral arrest, impaired awareness, automatisms, head/eye version, and clonic movements (often alternating from one side to the other and progressing to a hemiclonic or focal to bilateral tonic-clonic seizure). Seizures are usually brief (<3 min).
| - Prolonged or focal clonic (hemiclonic) seizures (>10 min)
| - Epileptic spasms
- Myoclonic seizures
- Sequential seizures
- Tonic seizures
|
EEG | | - Interictal: Mild background slowing
| - Interictal:
- Persistent focal slowing or moderate or greater background slowing not limited to the postictal period
- Hypsarhythmia
|
Development at onset | | | - Moderate to profound delay
- Neurocognitive regression
|
Imaging | | | - Causal lesion on brain MRI
|
Course of illness | | - Lack of remission by late childhood
| - Neurocognitive regression with myoclonic seizures, ataxia, spasticity
|
Syndrome without laboratory confirmation: In resource-limited regions, SeLIE can be diagnosed without EEG and MRI in an infant with a family history suggestive of familial SeLIE who meets all other mandatory and exclusionary clinical criteria and has no alerts. However, the clinical history of affected family members should be consistent with the expected course for SeLIE, and careful follow-up of the patient is required to ensure their course is also consistent with this syndrome. |