Febrile Infection-Related Epilepsy Syndrome (FIRES)
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Febrile Infection-Related Epilepsy Syndrome (FIRES) is not the name you expected.
Febrile infection-related epilepsy syndrome (FIRES) is a rare epilepsy syndrome of unclear etiology in which children, usually of school age, suddenly develop very frequent seizures (up to hundreds per day) within two weeks after a mild febrile illness. Seizures are very difficult to treat with conventional anti-seizure medications and often require deep sedation with anesthetics. It is possible that the syndrome is caused by an inflammatory or autoimmune mechanism but this remains to be confirmed. Immune treatment and the ketogenic diet may help. Outcome is poor with most children left with significant cognitive disability and refractory epilepsy.
Status epilepticus (SE) is defined as a continuous seizure or a flurry of very frequent seizures. In children, the most common causes of SE include congenital and genetic disorders, missed medication in the setting of chronic epilepsy, and acute brain injury, including infections. SE in young children (<6 year old) can also be the consequence of fever, a condition known as febrile convulsions or febrile SE, but it is usually self-limited. However, in rare cases, a severe form of SE that does not respond to anticonvulsant medications, a situation called refractory SE, can follow a minor febrile illness. Lyon and colleagues made the first description of a syndrome of catastrophic epilepsy in children in 1961. The syndrome was subsequently reported under different names and acronyms by other groups. The current suggested acronym is FIRES (febrile infection-related epilepsy syndrome).
American Epilepsy Society
- 135 South LaSalle Street
- Suite 2850
- Chicago, IL 60603
- Tel: 312-883-3800
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- Website: http://www.aesnet.org
CURE: Citizens United for Research in Epilepsy
- 430 W. Erie
- Suite Suite 210
- Chicago, IL 60654
- Tel: (312)765-7118
- Fax: (312)255-1801
- Tel: (800)765-7118
- Email: info@CUREepilepsy.org
- Website: http://www.CUREepilepsy.org
Child Neurology Foundation
- 201 Chicago Ave, #200
- Minneapolis, MN 55415
- Tel: (952)641-6100
- Fax: (952)881-6276
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- 8301 Professional Place
- Landover, MD 20785-7223
- Tel: (866)330-2718
- Fax: (877)687-4878
- Tel: (800)332-1000
- Email: ContactUs@efa.org
- Website: http://www.epilepsyfoundation.org
Genetic and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Tel: (301)251-4925
- Fax: (301)251-4911
- Tel: (888)205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
Intractable Childhood Epilepsy Alliance (ICE)
- PO Box 365
- 250 Lewisville-Vienna Road
- Lewisville, NC 27023
- Tel: (336)918-9440
- Fax: (336)946-1197
- Website: http://www.icepilepsy.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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Last Updated: 1/5/2016
Copyright 2015 National Organization for Rare Disorders, Inc.
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