User:Mr. Ibrahem/Febrile infection-related epilepsy syndrome
| Febrile infection-related epilepsy syndrome | |
|---|---|
| Other names | Acute encephalitis with refractory repetitive partial seizures (AERRPS); devastating epileptic encephalopathy in school aged children (DESC); fever induced refractory epilepsy in school-aged children |
| Medical specialty | Neurology |
| Symptoms | Severe seizures within two weeks of fever[1] |
| Complications | Intellectual disability, behavioral problems, ongoing seizures[2][1] |
| Usual onset | 2-17 yrs[1] |
| Causes | Unknown[1] |
| Differential diagnosis | Viral encephalitis, hypoxic-ischemic brain injury, mitochondrial disorders, autoimmune encephalitis[3][2] |
| Treatment | Benzodiazepines, barbiturates, ketogenic diet[2] |
| Prognosis | Poor[1] |
| Frequency | 1 in 1,000,000/yr (children)[2] |
| Deaths | 12% risk of death[2] |
Febrile infection-related epilepsy syndrome (FIRES) is onset of severe seizures (status epilepticus) following a febrile illness in someone who was previously healthy.[1] The seizures may initially be focal; however, often become tonic-clonic.[4] Complications often include intellectual disability, behavioral problems, and ongoing seizures.[1][2]
The underlying cause is unclear.[1] Often there is an upper respiratory tract or gastroenteritis one day to two weeks before onset.[1] Diagnosis involves extensive testing to rule out other possible causes.[2][5] It is a type of new-onset refractory status epilepticus (NORSE).[5]
The seizures are often resistant to treatment.[2] High doses of benzodiazepines or barbiturates are often used, with care taking place in the intensive care unit.[2] A ketogenic diet may help in some cases.[1] The medications anakinra or tocilizumab have been tried.[2] The risk of death, despite treatment is about 12%.[2]
The condition newly affects about one in a million children per year.[2] Onset is generally in children between the ages of 2 and 17.[1] Males appear to be more commonly affected than females.[2] Cases consistent with the condition were first described in 1961, with the current name coming into use in 2010.[5]
References
[change | change source]- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 "Febrile infection related epilepsy". www.epilepsydiagnosis.org. ILAE. Archived from the original on 2 August 2023. Retrieved 9 September 2023.
- ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 Nabbout, Rima. "Orphanet: Febrile infection related epilepsy syndrome". www.orpha.net. Archived from the original on 31 March 2023. Retrieved 9 September 2023.
- ↑ "Febrile infection related epilepsy". www.epilepsydiagnosis.org. Archived from the original on 11 August 2022. Retrieved 9 September 2023.
- ↑ "FEBRILE INFECTION RELATED EPILEPSY". www.epilepsydiagnosis.org. Archived from the original on 11 August 2022. Retrieved 9 September 2023.
- ↑ 5.0 5.1 5.2 Specchio, Nicola; Pietrafusa, Nicola (August 2020). "New‐onset refractory status epilepticus and febrile infection‐related epilepsy syndrome". Developmental Medicine & Child Neurology. 62 (8): 897–905. doi:10.1111/dmcn.14553.