National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Febrile infection-related epilepsy syndrome


Other Names:
FIRES; Acute encephalitis with refractory repetitive partial seizures; AERRPS; FIRES; Acute encephalitis with refractory repetitive partial seizures; AERRPS; Acute non-herpetic encephalitis with severe refractory status epilepticus; Devastating epileptic encephalopathy in school-aged children; DESC syndrome; Fever-induced refractory epileptic encephalopathy in school-aged children; Idiopathic catastrophic epileptic encephalopathy; Severe refractory status epilepticus owing to presumed encephalitis; Status epilepticus owing to presumed encephalitis; Febrile infection-related epilepsy syndrome See More
Categories:
This disease is grouped under:
FIRES (Febrile Infection-Related Epilepsy Syndrome) is a sub-type of cryptogenic new-onset refractory status epilepticus (NORSE). NORSE describes a condition in which a healthy person who has not had seizures before, begins having seizures. Over a few days, the seizures increase in frequency and length and evolve into status epilepticus (SE). SE is a prolonged seizure or cluster of seizures during which a person does not regain consciousness between seizures.[1][2] The seizure activity is considered refractory because it cannot be controlled with standard anti-seizure medications. Cryptogenic means all possible known causes have been ruled out. People with the sub-type FIRES have a fever a day to two weeks before the beginning of the seizures. The fever may or may not be present when SE begins, but no infection can be found to be causing SE. SE can last for days, weeks, or even months.[1][2]

Of note, FIRES previously was considered a separate condition that occurred only in children, while NORSE was described only in adults. However, there is now consensus that FIRES and NORSE without a prior fever can both be described in people of any age.[1][3]

You can learn more about FIRES on GARD's NORSE Information Page.
Last updated: 8/15/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Developmental regression
Loss of developmental milestones
Mental deterioration in childhood
[ more ] 		0002376
EEG abnormality 0002353
Focal-onset seizure
Seizure affecting one half of brain
0007359
Lethargy 0001254
30%-79% of people have these symptoms
Behavioral abnormality
Behavioral changes
Behavioral disorders
Behavioral disturbances
Behavioral problems
Behavioral/psychiatric abnormalities
Behavioural/Psychiatric abnormality
Psychiatric disorders
Psychiatric disturbances
[ more ] 		0000708
Cough
Coughing
0012735
Fever 0001945
Headache
Headaches
0002315
Myalgia
Muscle ache
Muscle pain
[ more ] 		0003326
Sinusitis
Sinus inflammation
0000246
5%-29% of people have these symptoms
Autoimmunity
Autoimmune disease
Autoimmune disorder
[ more ] 		0002960
Sudden death 0001699
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Last updated: 7/1/2020

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

  • The Critical Care EEG Monitoring Research Consortium provides a list of members that treat NORSE. The list does not represent a complete list of doctors and facilities that can effectively treat NORSE, but is one place to start a search.

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include Dravet syndrome, Alpers syndrome, female restricted epilepsy with intellectual disability (due to PCDH19 mutations), infectious or limbic encephalitis (e.g., with VGKC antibodies or NMDA receptor antibodies) and metabolic diseases (e.g., biotin-responsive basal ganglia disease, citrullinemia) (see these terms).
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 
  • The NORSE Institute provides information about ongoing and future research on NORSE and FIRES.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Febrile infection-related epilepsy syndrome. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Gaspard N. New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES). National Organization for Rare Disorders (NORD). 2018; https://rarediseases.org/rare-diseases/new-onset-refractory-status-epilepticus-norse/.
  2. Gaspard N, Hirsch LJ, Sculier C. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. Epilepsia. April, 2018; 59(4):745-752. https://www.ncbi.nlm.nih.gov/pubmed/29476535.
  3. Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. April 2018; 59(4):739-744. https://www.ncbi.nlm.nih.gov/pubmed/29399791.