Febrile infection-related epilepsy syndrome

Febrile infection-related epilepsy syndrome (FIRES) is a form of epilepsy that affects children three to fifteen years old. A healthy child that may have been ill in the last few days or with a lingering fever goes into a state of continuous seizures. The seizures are resistant to seizure medications and treatments, though barbiturates may be administered.^[1] Medical diagnostic tests may initially return no clear diagnosis and may not detect any obvious swelling on the brain. The syndrome is very rare: it may only affect 1 in 1,000,000 children.^[2]

Signs and symptoms

FIRES seizures are non-focal - there is no specified starting or stopping point - making brain surgery impossible. These seizures damage cognitive abilities of the brain such as memory or sensory abilities. This can result in learning disabilities,^[1] behavioral disorders, memory issues, sensory changes, inability to move, and death. Children continue to have seizures throughout their lives.

Cause

The cause of FIRES is not known.^[3] It does not happen twice in the same family, but the medical community does not know if it is genetic. It happens in boys more than girls. After the initial status, life expectancy is not affected directly. Issues such as overdose of medications or infections at a food tube site are examples of things that would be secondary to the status.

Treatment

• Ketogenic diet has been used
• Vagus nerve stimulation helps control seizure activity after recovery from status.
• Intravenous immunoglobulin treatment is being explored as an option to treat this form of epilepsy.^[4]
• Barbiturates have been shown to be effective in treating status epilepsy.^[5]

History

FIRES was named in 2008 by Dr. Andreas van Baalen and colleagues.^[6] Previous names include AERRPS, DESC (Devastating Epileptic encephalopathy in School-aged Children),^[7] and NORSE (New-Onset Refractory Status Epilepticus).^[7]

References

1. Kramer, U; Chi, CS; Lin, KL; Specchio, N; Sahin, M; Olson, H; Nabbout, R; Kluger, G; Lin, JJ; van Baalen, A (November 2011). "Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children.". Epilepsia 52 (11): 1956–65. doi:10.1111/j.1528-1167.2011.03250.x. PMID 21883180.
2. van Baalen, A; Häusler, M; Plecko-Startinig, B; Strautmanis, J; Vlaho, S; Gebhardt, B; Rohr, A; Abicht, A; Kluger, G; Stephani, U; Probst, C; Vincent, A; Bien, CG (August 2012). "Febrile infection-related epilepsy syndrome without detectable autoantibodies and response to immunotherapy: a case series and discussion of epileptogenesis in FIRES.". Neuropediatrics 43 (4): 209–16. doi:10.1055/s-0032-1323848. PMID 22911482.
3. "FEBRILE INFECTION RELATED EPILEPSY". www.epilepsydiagnosis.org. Retrieved 7 October 2014.
4. Gall, C.R.E., Jumma, O. & Mohanraj, R. 2013, "Five cases of new onset refractory status epilepticus (NORSE) syndrome: Outcomes with early immunotherapy", Seizure, vol. 22, no. 3, pp. 217-220
5. Pranzatelli, M.R. & Nadi, N.S. 1995, "Mechanism of action of antiepileptic and antimyoclonic drugs.", Advances in Neurology, vol. 67, pp. 329-360.
6. van Baalen, A; Häusler, M; Boor, R; Rohr, A; Sperner, J; Kurlemann, G; Panzer, A; Stephani, U; Kluger, G (July 2010). "Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood.". Epilepsia 51 (7): 1323–8. doi:10.1111/j.1528-1167.2010.02535.x. PMID 20345937.
7. Simon Shorvon and Monica Ferlisi (2011-09-13). "The treatment of super-refractory status epilepticus: a critical review of available therapies and a clinical treatment protocol". Brain.oxfordjournals.org. doi:10.1093/brain/awr215. Retrieved 2014-04-04.