Febrile Seizures Plus

 

Also called Genetic epilepsy with febrile seizures plus (GEFS+)

 

Presentation

Unlike other epilepsy syndromes, this is a diagnosis is applied to a child with atypical febrile seizures where these have early onset, multiple episodes, focal seizures or continuing beyond 6y age sometimes till puberty; with or without focal or generalized afebrile seizures.

 

Previously called ‘Generalised epilepsy with febrile seizures plus’, but more appropriate to call Febrile Seizures Plus as focal or myoclonic seizures are also seen.

 

Development and cognition are not affected.

 

There is f/h/o of different epilepsy types in many family members. There may be family member with epileptic encephalopathies like Dravet or Doose syndrome where developmental impairment is seen.

 

Investigation

Detailed history of seizure types and family history helps in making the diagnosis.

 

Autosomal dominant inheritance is seen; some individuals have mutations in SCN1A or GABA receptor gene, though other are being explored.

 

Interictal EEG may be normal or show generalized spike-and-waves, especially if sleep deprived.

 

Management

Treatment may not be needed for infrequent febrile seizures.

Provide rescue medication (e.g. buccal Midazolam) for those who experience prolonged seizures.

Those with afebrile seizures may require regular AEDs like Valproate, Levetiracetam, Lamotrigine or Clobazam; or their combination.

 

Ensure diagnosis is correct; and differentiate from epileptic encephalopathies such as Dravet or Doose syndrome that may require input from tertiary neurology.