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]

Febrile infection-related epilepsy syndrome
Other namesAcute non-herpetic encephalitis with severe refractory status epilepticus, Fever-induced refractory epileptic encephalopathy in school-aged children

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 is unknown if the disease is genetic in origin. It is more common in boys more than girls.

Diagnosis

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. Brain.oxfordjournals.org. 134 (Pt 10): 2802–2818. doi:10.1093/brain/awr215. PMID 21914716.
Classification
  • ICD-10: G40.5
External resources
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