Febrile Infection-Related Epilepsy Syndrome (FIRES)

National Organization for Rare Disorders, Inc.

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It is possible that the main title of the report Febrile Infection-Related Epilepsy Syndrome (FIRES) is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

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).

Supporting Organizations

American Epilepsy Society

135 South LaSalle Street
Suite 2850
Chicago, IL 60603
Tel: 312-883-3800
Email: emurray@aesnet.org
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
Tel: (877)263-5430
Email: jstone@childneurologyfoundation.org
Website: http://www.childneurologyfoundation.org

Epilepsy Foundation

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.