FIRES
MCID: FBR064
MIFTS: 34

Febrile Infection-Related Epilepsy Syndrome (FIRES)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Febrile Infection-Related Epilepsy Syndrome

MalaCards integrated aliases for Febrile Infection-Related Epilepsy Syndrome:

Name: Febrile Infection-Related Epilepsy Syndrome 53 59
Acute Encephalitis with Refractory Repetitive Partial Seizures 53 59 37
Acute Non-Herpetic Encephalitis with Severe Refractory Status Epilepticus 53 59
Fever-Induced Refractory Epileptic Encephalopathy in School-Aged Children 53 59
Severe Refractory Status Epilepticus Owing to Presumed Encephalitis 53 59
Devastating Epileptic Encephalopathy in School-Aged Children 53 59
Idiopathic Catastrophic Epileptic Encephalopathy 53 59
Desc Syndrome 53 59
Aerrps 53 59
Fires 53 59
Status Epilepticus Owing to Presumed Encephalitis 53

Characteristics:

Orphanet epidemiological data:

59
febrile infection-related epilepsy syndrome
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Germany),1-9/100000 (Europe); Age of onset: All ages; Age of death: normal life expectancy;

HPO:

32
febrile infection-related epilepsy syndrome:
Clinical modifier sudden death


Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

KEGG 37 H01829
Orphanet 59 ORPHA163703

Summaries for Febrile Infection-Related Epilepsy Syndrome

NIH Rare Diseases : 53 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. 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. 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. You can learn more about FIRES on GARD's NORSE Information Page.

MalaCards based summary : Febrile Infection-Related Epilepsy Syndrome, also known as acute encephalitis with refractory repetitive partial seizures, is related to status epilepticus and encephalopathy. An important gene associated with Febrile Infection-Related Epilepsy Syndrome is PCDH19 (Protocadherin 19). The drugs Vortioxetine and Serotonin Uptake Inhibitors have been mentioned in the context of this disorder. Affiliated tissues include brain, testes and eye, and related phenotypes are eeg abnormality and developmental regression

KEGG : 37
Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is a new epileptic syndrome mainly affecting children. AERRPS has not been perceived in Western countries, although it is widely recognized in Japan. They usually present abruptly with seizure or impaired consciousness as well as high-grade fever following antecedent infection. Seizures in AERRPS are almost exclusively of localized origin, whose semiology includes eye deviation, hemifacial twitching, hemiclonic convulsion, and autonomic manifestations. Partial seizures are brief, but repeat with increasing frequency and develop status epilepticus at nadir. It is followed by continuous transition to intractable epilepsy without a latent period. They are uniformly resistant to conventional antiepileptic drugs. In most cases, high-dose barbiturate treatment is necessary during the acute phase of the illness. Benzodiazepines, mainly midazolam, were partially effective in some patients. The pathogenesis of AERRPS is still poorly understood. It is currently suspected to represent an inflammatory disorder of the central nervous system.

Wikipedia : 75 Febrile infection-related epilepsy syndrome (FIRES) is a form of epilepsy that affects children three to... more...

Related Diseases for Febrile Infection-Related Epilepsy Syndrome

Diseases related to Febrile Infection-Related Epilepsy Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 41)
# Related Disease Score Top Affiliating Genes
1 status epilepticus 30.5 SCN1A POLG
2 encephalopathy 30.2 SCN1A PCDH19
3 epilepsy 30.0 SCN1A POLG PCDH19
4 epileptic encephalopathy, early infantile, 6 29.2 SCN1A PCDH19
5 new-onset refractory status epilepticus 11.8
6 ocular motor apraxia 10.6
7 encephalitis 10.4
8 seizure disorder 10.4
9 viral encephalitis 10.3
10 subacute delirium 10.3
11 autoimmune disease 10.3
12 visual epilepsy 10.3
13 pharyngitis 10.2
14 learning disability 10.2
15 autoimmune encephalitis 10.2
16 cerebral atrophy 10.2
17 infectious encephalitis 10.2
18 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
19 cyanosis, transient neonatal 10.2
20 dysgraphia 10.2
21 herpes simplex 10.2
22 limbic encephalitis 10.2
23 non-herpetic acute limbic encephalitis 10.2
24 cytokine deficiency 10.1
25 alacrima, achalasia, and mental retardation syndrome 10.1
26 west syndrome 10.1
27 hemophagocytic lymphohistiocytosis 10.1
28 exanthem 10.1
29 hemiplegia 10.1
30 focal epilepsy 10.1
31 brain edema 10.1
32 rasmussen encephalitis 10.1
33 febrile seizures 10.1
34 secondary hemophagocytic lymphohistiocytosis 10.1
35 rare epilepsy 10.1
36 exanthema subitum 9.6 SCN1A POLG
37 infancy electroclinical syndrome 9.6 SCN1A PCDH19
38 epileptic encephalopathy, early infantile, 9 9.5 SCN1A PCDH19
39 early infantile epileptic encephalopathy 9.3 SCN1A PCDH19
40 body mass index quantitative trait locus 11 9.2 SCN1A POLG
41 epilepsy, idiopathic generalized 9.1 SCN1A POLG PCDH19

Graphical network of the top 20 diseases related to Febrile Infection-Related Epilepsy Syndrome:



Diseases related to Febrile Infection-Related Epilepsy Syndrome

Symptoms & Phenotypes for Febrile Infection-Related Epilepsy Syndrome

Human phenotypes related to Febrile Infection-Related Epilepsy Syndrome:

59 32 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 eeg abnormality 59 32 hallmark (90%) Very frequent (99-80%) HP:0002353
2 developmental regression 59 32 hallmark (90%) Very frequent (99-80%) HP:0002376
3 focal-onset seizure 59 32 hallmark (90%) Very frequent (99-80%) HP:0007359
4 lethargy 59 32 hallmark (90%) Very frequent (99-80%) HP:0001254
5 behavioral abnormality 59 32 frequent (33%) Frequent (79-30%) HP:0000708
6 fever 59 32 frequent (33%) Frequent (79-30%) HP:0001945
7 sinusitis 59 32 frequent (33%) Frequent (79-30%) HP:0000246
8 myalgia 59 32 frequent (33%) Frequent (79-30%) HP:0003326
9 cough 59 32 frequent (33%) Frequent (79-30%) HP:0012735
10 headache 59 32 frequent (33%) Frequent (79-30%) HP:0002315
11 autoimmunity 59 32 occasional (7.5%) Occasional (29-5%) HP:0002960
12 sudden death 59 Occasional (29-5%)

Drugs & Therapeutics for Febrile Infection-Related Epilepsy Syndrome

Drugs for Febrile Infection-Related Epilepsy Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 69)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vortioxetine Approved, Investigational Phase 3 508233-74-7 9966051
2 Serotonin Uptake Inhibitors Phase 3
3 Serotonin Receptor Agonists Phase 3
4 Tranquilizing Agents Phase 3
5 Anti-Anxiety Agents Phase 3
6 Antidepressive Agents Phase 3
7 Serotonin 5-HT1 Receptor Agonists Phase 3
8 Neurotransmitter Uptake Inhibitors Phase 3
9 Serotonin Antagonists Phase 3
10 Serotonin 5-HT3 Receptor Antagonists Phase 3
11 Serotonin Agents Phase 3
12 Psychotropic Drugs Phase 3
13
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
14
Valproic acid Approved, Investigational Phase 2 99-66-1 3121
15
Phenytoin Approved, Vet_approved Phase 2 57-41-0 1775
16
Guaifenesin Approved, Investigational, Vet_approved Phase 2 93-14-1 3516
17
Dextromethorphan Approved Phase 2 125-71-3 5360696 5362449
18
Tocopherol Approved, Investigational Phase 2 1406-66-2, 54-28-4 14986
19
Pyridoxine Approved, Investigational, Nutraceutical, Vet_approved Phase 2 65-23-6 1054
20
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
21
Vitamin E Approved, Nutraceutical, Vet_approved Phase 2 59-02-9 14985
22 Tocotrienol Investigational Phase 2 6829-55-6
23 Sodium Channel Blockers Phase 2
24 GABA Agents Phase 2
25 Diuretics, Potassium Sparing Phase 2
26 Cytochrome P-450 Enzyme Inducers Phase 2
27 Antimanic Agents Phase 2
28 Vitamin B9 Phase 2
29 Vitamin B Complex Phase 2
30 Folate Phase 2
31 Vitamin B 6 Phase 2
32 Anticonvulsants Phase 2
33 Respiratory System Agents Phase 2
34 Chlorpheniramine, phenylpropanolamine drug combination Phase 2
35 Excitatory Amino Acid Antagonists Phase 2
36 Excitatory Amino Acids Phase 2
37 Antitussive Agents Phase 2
38 Micronutrients Phase 2
39 Trace Elements Phase 2
40 Antioxidants Phase 2
41 Tocotrienols Phase 2
42 Tocopherols Phase 2
43 Vitamins Phase 2
44 Protective Agents Phase 2
45 Nutrients Phase 2
46
Pyridoxal Experimental, Nutraceutical Phase 2 66-72-8 1050
47
Nitroprusside Approved, Investigational 15078-28-1 11963622
48
Acetylcholine Approved, Investigational 51-84-3 187
49
Verapamil Approved 52-53-9 2520
50
Nitrous oxide Approved, Vet_approved 10024-97-2 948

Interventional clinical trials:

(show all 25)
# Name Status NCT ID Phase Drugs
1 A Randomized, Double-Blinded, Placebo Controlled Trial Using Single Dose Dexmedetomidine In The Treatment Of Pain In Patients Undergoing Cleft Palate Repair Withdrawn NCT02915042 Phase 4 Dexmedetomidine;Placebo
2 Vortioxetine for the Treatment of Hoarding Disorder- an Open Label Study Not yet recruiting NCT04035850 Phase 3 Vortioxetine Tablets
3 Treatment of Nodding Syndrome - A Randomized Blinded Placebo-Controlled Crossover Trial of Oral Pyridoxine and Conventional Anti-Epileptic Therapy, in Northern Uganda — 2012 Unknown status NCT01730313 Phase 2 Pyridoxine;Sodium Valproate;Phenytoin;Placebo
4 Parent Training in Child Safety Practices Completed NCT02934633 Phase 2
5 Trial of Naltrexone and Dextromethorphan for Gulf War Veterans Illnesses Completed NCT02206490 Phase 2 naltrexone;Dextromethorphan
6 Field Evaluation of Malaria Vector Control Using Traditional Plant-based Anti-mosquito Measures in Yunnan Province, P.R. China Completed NCT00116766 Phase 2
7 A Phase II Randomized, Double Blinded, Placebo-controlled Study of Gamma Tocopherol-enriched Supplement on Lower Airway Responses to Inhaled Wood Smoke in Healthy Adults Recruiting NCT03444298 Phase 2 Gamma Tocopherol;Placebo
8 A Double-Blind, Placebo-Controlled Study of Naltrexone in Pyromania Withdrawn NCT00467454 Phase 2 Naltrexone;Placebo
9 Effect of Woodsmoke on Vascular Function in Healthy Firefighters Unknown status NCT01495325
10 European Survey: Risk of Cyanide Poisoning in Smoke Inhalation, Symptoms, Key Treatment and Outcome (RISK) Completed NCT01386788
11 Evaluation of the Effectiveness and Safety of Transcorneal Electrical Stimulation to Improve Visual Function After Ocular Trauma Completed NCT02019927
12 Effect of Omentectomy on Metabolic Syndrome, Acute Phase Reactants & Inflammatory Mediators in Patients Undergoing LRYGBP: A Randomized Trial Completed NCT00923260
13 Keeping Children Safe at Home: Cluster Randomised Controlled Trial of the Implementation of an Injury Prevention Briefing in Children's Centres for the Prevention of Fire-related Injuries Completed NCT01452191
14 Anesthetic Gas Leakage in Children During Tonsillectomy: a Comparison of Cuffed and Uncuffed Tracheal Tubes Completed NCT02725164 oxygen concentration;nitrous oxide concentration;carbon dioxide concentration;sevoflurane concentration
15 Use of Biomass Briquettes: Its Effect on Indoor Air Pollution and on Pneumococcal Nasopharyngeal Carriage. A Randomized Clinical Trial Completed NCT01660659
16 Make Safe Happen App Evaluation Study Completed NCT02751203
17 Early Auditory Referral in Primary Care (EAR-PC) Completed NCT03004937
18 A Longitudinal Study of Exhaled Nitric Oxide in Children Completed NCT00991874
19 Using Rapid Cycle Randomized Controlled Trials to Optimize the Influenza Clinical Decision Support Tool Recruiting NCT03415425
20 Using Rapid Cycle Randomized Controlled Trials to Study a Tobacco Cessation Best Practice Advisory. Recruiting NCT03714191
21 A Worldwide Post-Market Surveillance Registry to Assess the Medigus Ultrasonic Surgical Endostapler (MUSE™) System for the Treatment of GERD Recruiting NCT02366169
22 To Identify Persons Who Are Susceptible to WSP-induced Inflammation and Examine the Role of GSTM1 and Other Factors in This Susceptibility Recruiting NCT02767973
23 Complex Fractionated Atrial Electrocardiograms (CFAEs) Spatiotemporal Dispersion Guided Ablation Versus Pulmonary Vein Isolation (PVI) Guided Ablation in Persistent Atrial Fibrillation, a Multicenter Randomized Trial Recruiting NCT02696265
24 An Intervention Model for the Prevention of Infertility in Healthy Young Men Living in Highly Polluted Areas Active, not recruiting NCT04012385
25 An EPIC Based BPA to Enhance Quit Line Referral and Use Enrolling by invitation NCT03229356

Search NIH Clinical Center for Febrile Infection-Related Epilepsy Syndrome

Genetic Tests for Febrile Infection-Related Epilepsy Syndrome

Anatomical Context for Febrile Infection-Related Epilepsy Syndrome

MalaCards organs/tissues related to Febrile Infection-Related Epilepsy Syndrome:

41
Brain, Testes, Eye, Thalamus

Publications for Febrile Infection-Related Epilepsy Syndrome

Articles related to Febrile Infection-Related Epilepsy Syndrome:

(show top 50) (show all 88)
# Title Authors PMID Year
1
Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. 38 6
29399791 2018
2
New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. 38 6
29476535 2018
3
Etiology is the key determinant of neuroinflammation in epilepsy: Elevation of cerebrospinal fluid cytokines and chemokines in febrile infection-related epilepsy syndrome and febrile status epilepticus. 38
31283843 2019
4
Inflammation and reactive oxygen species in status epilepticus: Biomarkers and implications for therapy. 38
31171434 2019
5
The short-term and long-term outcome of febrile infection-related epilepsy syndrome in children. 38
31035103 2019
6
Therapeutic effect of Anakinra in the relapsing chronic phase of febrile infection-related epilepsy syndrome. 38
31168503 2019
7
Febrile infection-related epilepsy syndrome (FIRES) in an adult patient: an early neuroradiological finding. 38
31093785 2019
8
New onset refractory status epilepticus (NORSE). 38
30482654 2019
9
Treatment options in pediatric super-refractory status epilepticus. 38
30528076 2019
10
New onset refractory status epilepticus research: What is on the horizon? 38
30894443 2019
11
Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. 38
30779222 2019
12
The spectrum of neuroimaging findings in febrile infection-related epilepsy syndrome (FIRES): A literature review. 38
30854647 2019
13
Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies. 38
31371963 2019
14
Ketogenic Diet as a Treatment for Super-Refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome. 38
31105638 2019
15
Febrile Infection-Related Epilepsy Syndrome (FIRES) with Multifocal Subcortical Infarcts, A New Imaging Phenotype. 38
29980149 2018
16
Fueling the FIRES: Hemophagocytic lymphohistiocytosis in febrile infection-related epilepsy syndrome. 38
30132834 2018
17
Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and neuroimaging follow-up. 38
29453111 2018
18
Use of high b value diffusion-weighted magnetic resonance imaging in acute encephalopathy/encephalitis during childhood. 38
28838686 2018
19
Febrile infection-related epilepsy syndrome (FIRES) with super-refractory status epilepticus revealing autoimmune encephalitis due to GABAAR antibodies. 38
29203057 2018
20
Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents. 38
29745347 2018
21
Autoimmune Epilepsies. 38
29103423 2017
22
Early ictal and interictal patterns in FIRES: The sparks before the blaze. 38
28555777 2017
23
DNM1 encephalopathy: A new disease of vesicle fission. 38
28667181 2017
24
"Symptomatic" infection-associated acute encephalopathy in children with underlying neurological disorders. 38
27780632 2017
25
Febrile Infection-Related Epilepsy Syndrome: Clinical Review and Hypotheses of Epileptogenesis. 38
27919115 2017
26
Effectiveness of Electroconvulsive Therapy for Refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome. 38
27434885 2017
27
Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases. 38
27655472 2017
28
Biallelic SCN10A mutations in neuromuscular disease and epileptic encephalopathy. 38
28078312 2017
29
Febrile Infection-Related Epilepsy Syndrome (FIRES): A Literature Review and Case Study. 38
28898171 2017
30
Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature. 38
29624228 2017
31
Moyamoya in a Patient with FIRES: A First Case Report. 38
28436815 2017
32
Febrile infection-related epilepsy syndrome treated with anakinra. 38
27770579 2016
33
Acute encephalitis with refractory, repetitive partial seizures: A case report and literature review. 38
27882170 2016
34
Ketogenic diet treatment for pediatric super-refractory status epilepticus. 38
27475280 2016
35
Acute encephalitis with refractory, repetitive partial seizures: Pathological findings and a new therapeutic approach using tacrolimus. 38
26906012 2016
36
Cytokine-related and sodium channel polymorphism as candidate predisposing factors for childhood encephalopathy FIRES/AERRPS. 38
27538648 2016
37
High-dose phenobarbital with intermittent short-acting barbiturates for acute encephalitis with refractory, repetitive partial seizures. 38
27273286 2016
38
Clinical profile and treatment outcome of febrile infection-related epilepsy syndrome in South Indian children. 38
27293328 2016
39
Chronological Evolution of Magnetic Resonance Imaging Findings in Children With Febrile Infection-Related Epilepsy Syndrome. 38
26597039 2016
40
Marked improvement in febrile infection-related epilepsy syndrome after lidocaine plus MgSO4 treatment in a 12-year-old girl. 38
27358770 2016
41
Rasmussen Syndrome and Other Inflammatory Epilepsies. 38
26060905 2015
42
Effect of levetiracetam in acute encephalitis with refractory, repetitive partial seizures during acute and chronic phase. 38
25174548 2015
43
Autoimmune encephalitis and its relation to infection. 38
25637289 2015
44
Lidocaine treatment in refractory status epilepticus resulting from febrile infection-related epilepsy syndrome: a case report and follow-up. 38
25343330 2015
45
Hippocampal Changes in Febrile Infection-Related Epilepsy Syndrome (FIRES). 38
26379807 2015
46
Evaluation and treatment of autoimmune neurologic disorders in the pediatric intensive care unit. 38
25727510 2014
47
[A case of acute encephalitis with refractory repetitive partial seizures successfully controlled by very-high-dose phenobarbital therapy found in a boy]. 38
25558588 2014
48
Cognitive outcomes in febrile infection-related epilepsy syndrome treated with the ketogenic diet. 38
25332495 2014
49
Ketogenic diet therapy is effective in encephalitis with refractory seizures. 38
24735348 2014
50
Actual insights into the clinical management of febrile seizures. 38
24477659 2014

Variations for Febrile Infection-Related Epilepsy Syndrome

Expression for Febrile Infection-Related Epilepsy Syndrome

Search GEO for disease gene expression data for Febrile Infection-Related Epilepsy Syndrome.

Pathways for Febrile Infection-Related Epilepsy Syndrome

GO Terms for Febrile Infection-Related Epilepsy Syndrome

Sources for Febrile Infection-Related Epilepsy Syndrome

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