Seizures | - Recurrent focal clonic (hemiclonic) febrile and afebrile seizures (which often alternate sides from seizure to seizure), focal to bilateral tonic-clonic, and/or generalized clonic seizures
| - No history of prolonged seizures (>10 min)
- Lack of fever sensitivity as a seizure trigger
| - Epileptic spasms
- Early infantile SCN1A DEE
|
EEG | | - Normal EEG background without interictal discharges after age 2 years
| |
Development at onset | | - Developmental delay at seizure onset
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Imaging | | | - MRI showing a causal focal lesion
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Course of illness | - Drug-resistant epilepsy
- Intellectual disability
| - Good efficacy with prophylactic sodium-channel agents including carbamazepine, oxcarbazepine, and phenytoin
| |
Possible evolving syndrome: In a child <12 months who presents with a prolonged hemiclonic or bilateral tonic-clonic seizure with fever, and no other underlying cause, the possibility of Dravet syndrome should be considered. Further convulsive seizures (often with fever, and if prolonged or hemiclonic) would allow more definitive diagnosis of Dravet syndrome. A diagnosis would be further supported by the finding of a pathogenic SCN1A variant. |