International Journal of Epilepsy 2015; 02(02): 090-093
DOI: 10.1016/j.ijep.2015.07.001
Case Report
Thieme Medical and Scientific Publishers Private Ltd.

Refractory status epilepticus: Febrile Illness Related Epileptic Syndrome (FIRES)

Varun Kumar Sharma
1   Jaslok Hospital and Research Centre, Mumbai, India
,
Anaita Udwadia Hegde
1   Jaslok Hospital and Research Centre, Mumbai, India
,
Fazal Nabi
1   Jaslok Hospital and Research Centre, Mumbai, India
› Institutsangaben

Verantwortlicher Herausgeber dieser Rubrik:
Weitere Informationen

Publikationsverlauf

Received: 26. November 2014

Accepted: 20. Juli 2015

Publikationsdatum:
06. Mai 2018 (online)

Abstract

In recent literature, a new entity has emerged, which focuses on a possible non-encephalitic epileptic encephalopathy precipitated by fever in a previously normal child. We report a typical case of Febrile illness related epileptic syndrome (FIRES) from Indian subcontinent. The index case presented with fever and multiple seizures, which progressed to status epilepticus and encephalopathy. All infectious, metabolic and autoimmune markers were negative. Convulsions were refractory to all possible treatment except thiopentone which achieved burst suppression pattern. Breakthrough seizures were prevented by using lacosamide and ketogenic diet along with multiple anticonvulsants. At 1-year follow-up, patient had a relatively good neurological outcome, however has persistent refractory epilepsy.

 
  • References

  • 1 Karmarkar SA, Aneja S, Khare S, Saini A, Seth A, Chauhan BK. A study of acute febrile encephalopathy with special reference to viral etiology. Indian J Pediatr 75 2008; 801-805
  • 2 Ismail FY, Kossoff EH. AERRPS, DESC, NORSE, FIRES: multi-labeling or distinct epileptic entities?. Epilepsia 52 2011; e185-e189
  • 3 Nabbout R. FIRES and IHHE: Delineation of the syndromes. Epilepsia 54 Suppl 2013; 54-56
  • 4 Baxter P, Clarke A, Cross H. et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure 12 2003; 379-387
  • 5 Wilder-Smith EP, Lim EC, Teoh HL. et al. The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity. Ann Acad Med Singapore 34 2005; 417-420
  • 6 Mikaeloff Y, Jambaqué I, Hertz-Pannier L. et al. Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis. Epilepsy Res 69 2006; 67-79
  • 7 Sakuma H, Awaya Y, Shiomi M. et al. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand 121 2010; 251-256
  • 8 Van Baalen A, Häusler M, Boor R. et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia 51 2010; 1323-1328
  • 9 Kramer U, Chi CS, Lin KL. et al. Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia 52 2011; 1956-1965
  • 10 Nabbout R, Vezzani A, Dulac O, Chiron C. Acute encephalopathy with inflammation mediated status epilepticus. Lancet Neurol 10 2011; 99-108
  • 11 Kim JS, Kim H, Lim BC. et al. Lacosamide as an adjunctive therapy in pediatric patients with refractory focal epilepsy. Brain Dev 36 2014; 510-515
  • 12 Yorns Jr WR, Khurana DS, Carvalho KS, Hardison HH, Legido A, Valencia I. Efficacy of lacosamide as adjunctive therapy in children with refractory epilepsy. J Child Neurol 29 2014; 23-27
  • 13 Grosso S, Parisi P, Spalice A, Verrotti A, Balestri P. Efficacy and safety of lacosamide in infants and young children with refractory focal epilepsy. Eur J Paediatr Neurol 18 2014; 55-59
  • 14 Nabbout R, Mazzuca M, Hubert P. et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES). Epilepsia 51 2010; 2033-2037