intractable childhood epilepsy with generalized tonic-clonic seizures (ICEGTC)
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Genetics
- associated with defects in SCN1A
Clinical manifestations
- generalized tonic-clonic seizures beginning usually in infancy & induced by fever
- seizures are associated with subsequent mental decline & ataxia or hypotonia
- ICEGTC is similar to SMEI, except for the absence of myoclonic seizures