FIRES
MCID: FBR064
MIFTS: 36

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 52 58
Acute Encephalitis with Refractory Repetitive Partial Seizures 52 58 36
Acute Non-Herpetic Encephalitis with Severe Refractory Status Epilepticus 52 58
Fever-Induced Refractory Epileptic Encephalopathy in School-Aged Children 52 58
Severe Refractory Status Epilepticus Owing to Presumed Encephalitis 52 58
Devastating Epileptic Encephalopathy in School-Aged Children 52 58
Idiopathic Catastrophic Epileptic Encephalopathy 52 58
Desc Syndrome 52 58
Aerrps 52 58
Fires 52 58
Status Epilepticus Owing to Presumed Encephalitis 52

Characteristics:

Orphanet epidemiological data:

58
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:

31
febrile infection-related epilepsy syndrome:
Clinical modifier sudden death


Classifications:

Orphanet: 58  
Rare neurological diseases


Summaries for Febrile Infection-Related Epilepsy Syndrome

NIH Rare Diseases : 52 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 encephalopathy and epilepsy. An important gene associated with Febrile Infection-Related Epilepsy Syndrome is PCDH19 (Protocadherin 19). The drugs Vortioxetine and Antidepressive Agents have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and testes, and related phenotypes are developmental regression and eeg abnormality

KEGG : 36 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 : 74 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 top 50) (show all 62)
# Related Disease Score Top Affiliating Genes
1 encephalopathy 30.8 SCN1A PCDH19
2 epilepsy 30.6 SCN1A POLG PCDH19
3 status epilepticus 30.5 SCN1A POLG PCDH19
4 visual epilepsy 29.9 SCN1A POLG
5 epileptic encephalopathy, early infantile, 6 29.7 SCN1A PCDH19
6 focal epilepsy 29.0 SCN1A PCDH19 IL1RN
7 west syndrome 29.0 SCN1A PCDH19 IL1RN
8 new-onset refractory status epilepticus 11.8
9 ocular motor apraxia 10.5
10 encephalitis 10.4
11 seizure disorder 10.4
12 viral encephalitis 10.3
13 subacute delirium 10.3
14 autoimmune disease 10.3
15 pharyngitis 10.2
16 learning disability 10.2
17 autoimmune encephalitis 10.2
18 cerebral atrophy 10.2
19 infectious encephalitis 10.2
20 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
21 cyanosis, transient neonatal 10.2
22 dysgraphia 10.2
23 herpes simplex 10.2
24 limbic encephalitis 10.2
25 non-herpetic acute limbic encephalitis 10.2
26 cytokine deficiency 10.1
27 alacrima, achalasia, and mental retardation syndrome 10.1
28 hemophagocytic lymphohistiocytosis 10.1
29 exanthem 10.1
30 middle east respiratory syndrome 10.1
31 hemiplegia 10.1
32 brain edema 10.1
33 febrile seizures 10.1
34 secondary hemophagocytic lymphohistiocytosis 10.1
35 rare epilepsy 10.1
36 plagiocephaly 9.9 SCN1A POLG
37 partial motor epilepsy 9.9 SCN1A POLG
38 landau-kleffner syndrome 9.9 SCN1A PCDH19
39 benign familial neonatal epilepsy 9.9 SCN1A PCDH19
40 encephalopathy due to defective mitochondrial and peroxisomal fission 1 9.9 SCN1A POLG
41 epileptic encephalopathy, early infantile, 9 9.9 SCN1A PCDH19
42 infancy electroclinical syndrome 9.9 SCN1A PCDH19
43 adolescence-adult electroclinical syndrome 9.9 SCN1A PCDH19
44 photosensitive epilepsy 9.8 SCN1A PCDH19
45 childhood electroclinical syndrome 9.8 SCN1A PCDH19
46 pyruvate dehydrogenase e1-alpha deficiency 9.8 SCN1A POLG
47 benign neonatal seizures 9.8 SCN1A PCDH19
48 benign familial infantile epilepsy 9.8 SCN1A PCDH19
49 autosomal dominant nocturnal frontal lobe epilepsy 9.8 SCN1A PCDH19
50 generalized epilepsy with febrile seizures plus 9.8 SCN1A 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:

58 31 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 developmental regression 58 31 hallmark (90%) Very frequent (99-80%) HP:0002376
2 eeg abnormality 58 31 hallmark (90%) Very frequent (99-80%) HP:0002353
3 lethargy 58 31 hallmark (90%) Very frequent (99-80%) HP:0001254
4 focal-onset seizure 58 31 hallmark (90%) Very frequent (99-80%) HP:0007359
5 behavioral abnormality 58 31 frequent (33%) Frequent (79-30%) HP:0000708
6 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
7 sinusitis 58 31 frequent (33%) Frequent (79-30%) HP:0000246
8 myalgia 58 31 frequent (33%) Frequent (79-30%) HP:0003326
9 cough 58 31 frequent (33%) Frequent (79-30%) HP:0012735
10 headache 58 31 frequent (33%) Frequent (79-30%) HP:0002315
11 autoimmunity 58 31 occasional (7.5%) Occasional (29-5%) HP:0002960
12 sudden death 58 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 56)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vortioxetine Approved, Investigational Phase 3 508233-74-7 9966051
2 Antidepressive Agents Phase 3
3 Serotonin 5-HT1 Receptor Agonists Phase 3
4 Psychotropic Drugs Phase 3
5 Serotonin Receptor Agonists Phase 3
6 Anti-Anxiety Agents Phase 3
7 Serotonin Uptake Inhibitors Phase 3
8 Serotonin 5-HT3 Receptor Antagonists Phase 3
9
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
10
Valproic acid Approved, Investigational Phase 2 99-66-1 3121
11
Phenytoin Approved, Vet_approved Phase 2 57-41-0 1775
12
Dextromethorphan Approved Phase 2 125-71-3 5360696 5362449
13
Guaifenesin Approved, Investigational, Vet_approved Phase 2 93-14-1 3516
14
Tocopherol Approved, Investigational Phase 2 1406-66-2, 54-28-4 14986
15
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
16
Pyridoxine Approved, Investigational, Nutraceutical, Vet_approved Phase 2 65-23-6 1054
17
Vitamin E Approved, Nutraceutical, Vet_approved Phase 2 59-02-9 14985
18 Tocotrienol Investigational Phase 2 6829-55-6
19 Sodium Channel Blockers Phase 2
20 Vitamin B 6 Phase 2
21 Vitamin B Complex Phase 2
22 Folate Phase 2
23 Anticonvulsants Phase 2
24 Diuretics, Potassium Sparing Phase 2
25 Vitamin B9 Phase 2
26 Respiratory System Agents Phase 2
27 Excitatory Amino Acid Antagonists Phase 2
28 Antitussive Agents Phase 2
29 Chlorpheniramine, phenylpropanolamine drug combination Phase 2
30 Trace Elements Phase 2
31 Micronutrients Phase 2
32 Vitamins Phase 2
33 Antioxidants Phase 2
34 Nutrients Phase 2
35 Protective Agents Phase 2
36 Tocotrienols Phase 2
37 Tocopherols Phase 2
38
Pyridoxal Experimental, Nutraceutical Phase 2 66-72-8 1050
39
Alprostadil Approved, Investigational 745-65-3 149351 5280723
40
Acetylcholine Approved, Investigational 51-84-3 187
41
Nitroprusside Approved, Investigational 15078-28-1 11963622
42
Verapamil Approved 52-53-9 2520
43
Nitrous oxide Approved, Vet_approved 10024-97-2 948
44
Sevoflurane Approved, Vet_approved 28523-86-6 5206
45
Nicotine Approved 54-11-5 942 89594
46
Nitric Oxide Approved 10102-43-9 145068
47
Bradykinin Investigational 58-82-2 439201
48 Kininogens
49 Vasodilator Agents
50 Anesthetics

Interventional clinical trials:

(show all 28)
# 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 Active, not 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 A Worldwide Post-Market Surveillance Registry to Assess the Medigus Ultrasonic Surgical Endostapler (MUSE™) System for the Treatment of GERD Unknown status NCT02366169
10 Effect of Woodsmoke on Vascular Function in Healthy Firefighters Unknown status NCT01495325
11 European Survey: Risk of Cyanide Poisoning in Smoke Inhalation, Symptoms, Key Treatment and Outcome (RISK) Completed NCT01386788
12 Evaluation of the Effectiveness and Safety of Transcorneal Electrical Stimulation to Improve Visual Function After Ocular Trauma Completed NCT02019927
13 Effect of Omentectomy on Metabolic Syndrome, Acute Phase Reactants & Inflammatory Mediators in Patients Undergoing LRYGBP: A Randomized Trial Completed NCT00923260
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 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
16 Use of Biomass Briquettes: Its Effect on Indoor Air Pollution and on Pneumococcal Nasopharyngeal Carriage. A Randomized Clinical Trial Completed NCT01660659
17 Make Safe Happen App Evaluation Study Completed NCT02751203
18 An Intervention Model for the Prevention of Infertility in Healthy Young Men Living in Highly Polluted Areas Completed NCT04012385
19 An EPIC Based BPA to Enhance Quit Line Referral and Use Completed NCT03229356
20 Early Auditory Referral in Primary Care (EAR-PC) Completed NCT03004937
21 A Longitudinal Study of Exhaled Nitric Oxide in Children Completed NCT00991874
22 Using Rapid Cycle Randomized Controlled Trials to Optimize the Influenza Clinical Decision Support Tool Recruiting NCT03415425
23 Using Rapid Cycle Randomized Controlled Trials to Study a Tobacco Cessation Best Practice Advisory. Recruiting NCT03714191
24 Internet-Delivered Cognitive Behaviour Therapy (ICBT) for Public Safety Personnel (PSP): Examination of Engagement, Outcomes, Strengths and Challenges Recruiting NCT04127032
25 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
26 To Identify Persons Who Are Susceptible to WSP-induced Inflammation and Examine the Role of GSTM1 and Other Factors in This Susceptibility Active, not recruiting NCT02767973
27 Establishing the Dynamic Range of Healthy Pediatric Inflammatory Responses to Vaccinations and Identifying the Role of IL1RN Polymorphisms in Controlling This Response. Enrolling by invitation NCT04112355
28 Internet-Delivered Cognitive Behaviour Therapy for PTSD Among Public Safety Personnel: Open Cohort Preference Trial for Transdiagnostic or Disorder-Specific Therapy Not yet recruiting NCT04335487

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:

40
Brain, Eye, Testes, Thalamus

Publications for Febrile Infection-Related Epilepsy Syndrome

Articles related to Febrile Infection-Related Epilepsy Syndrome:

(show top 50) (show all 96)
# Title Authors PMID Year
1
Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. 61 52
29399791 2018
2
New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. 52 61
29476535 2018
3
Efficacy of different treatment modalities for acute and chronic phases of the febrile infection-related epilepsy syndrome: A systematic review. 61
32417686 2020
4
New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. 61
32372459 2020
5
Therapeutic hypothermia for pediatric refractory status epilepticus May Ameliorate post-status epilepticus epilepsy. 61
32330677 2020
6
Extinguishing Febrile Infection-Related Epilepsy Syndrome: Pipe Dream or Reality? 61
32185792 2020
7
Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome. 61
32330681 2020
8
Genetic diagnoses in epilepsy: The impact of dynamic exome analysis in a pediatric cohort. 61
31957018 2020
9
Inflammation and reactive oxygen species in status epilepticus: Biomarkers and implications for therapy. 61
31171434 2019
10
Febrile infection-related epilepsy syndrome (FIRES) in an adult patient: an early neuroradiological finding. 61
31093785 2019
11
Febrile Infection-Related Epilepsy Syndrome Treated Successfully With Anakinra in a 21-Year-Old Woman. 61
31682750 2019
12
Centromedian thalamic nuclei deep brain stimulation and Anakinra treatment for FIRES - Two different outcomes. 61
31446001 2019
13
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. 61
31283843 2019
14
Therapeutic effect of Anakinra in the relapsing chronic phase of febrile infection-related epilepsy syndrome. 61
31168503 2019
15
The short-term and long-term outcome of febrile infection-related epilepsy syndrome in children. 61
31035103 2019
16
New onset refractory status epilepticus (NORSE). 61
30482654 2019
17
Treatment options in pediatric super-refractory status epilepticus. 61
30528076 2019
18
New onset refractory status epilepticus research: What is on the horizon? 61
30894443 2019
19
Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. 61
30779222 2019
20
The spectrum of neuroimaging findings in febrile infection-related epilepsy syndrome (FIRES): A literature review. 61
30854647 2019
21
Ketogenic Diet as a Treatment for Super-Refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome. 61
31105638 2019
22
Febrile infection-related epilepsy syndrome (FIRES): prevalence, impact and management strategies. 61
31371963 2019
23
Febrile Infection-Related Epilepsy Syndrome (FIRES) with Multifocal Subcortical Infarcts, A New Imaging Phenotype. 61
29980149 2018
24
Fueling the FIRES: Hemophagocytic lymphohistiocytosis in febrile infection-related epilepsy syndrome. 61
30132834 2018
25
Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and neuroimaging follow-up. 61
29453111 2018
26
Use of high b value diffusion-weighted magnetic resonance imaging in acute encephalopathy/encephalitis during childhood. 61
28838686 2018
27
Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents. 61
29745347 2018
28
Febrile infection-related epilepsy syndrome (FIRES) with super-refractory status epilepticus revealing autoimmune encephalitis due to GABAAR antibodies. 61
29203057 2018
29
Autoimmune Epilepsies. 61
29103423 2017
30
Early ictal and interictal patterns in FIRES: The sparks before the blaze. 61
28555777 2017
31
DNM1 encephalopathy: A new disease of vesicle fission. 61
28667181 2017
32
"Symptomatic" infection-associated acute encephalopathy in children with underlying neurological disorders. 61
27780632 2017
33
Effectiveness of Electroconvulsive Therapy for Refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome. 61
27434885 2017
34
Febrile Infection-Related Epilepsy Syndrome: Clinical Review and Hypotheses of Epileptogenesis. 61
27919115 2017
35
Biallelic SCN10A mutations in neuromuscular disease and epileptic encephalopathy. 61
28078312 2017
36
Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature. 61
29624228 2017
37
Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases. 61
27655472 2017
38
Febrile Infection-Related Epilepsy Syndrome (FIRES): A Literature Review and Case Study. 61
28898171 2017
39
Moyamoya in a Patient with FIRES: A First Case Report. 61
28436815 2017
40
Febrile infection-related epilepsy syndrome treated with anakinra. 61
27770579 2016
41
Acute encephalitis with refractory, repetitive partial seizures: A case report and literature review. 61
27882170 2016
42
Ketogenic diet treatment for pediatric super-refractory status epilepticus. 61
27475280 2016
43
Acute encephalitis with refractory, repetitive partial seizures: Pathological findings and a new therapeutic approach using tacrolimus. 61
26906012 2016
44
Cytokine-related and sodium channel polymorphism as candidate predisposing factors for childhood encephalopathy FIRES/AERRPS. 61
27538648 2016
45
High-dose phenobarbital with intermittent short-acting barbiturates for acute encephalitis with refractory, repetitive partial seizures. 61
27273286 2016
46
Clinical profile and treatment outcome of febrile infection-related epilepsy syndrome in South Indian children. 61
27293328 2016
47
Chronological Evolution of Magnetic Resonance Imaging Findings in Children With Febrile Infection-Related Epilepsy Syndrome. 61
26597039 2016
48
Marked improvement in febrile infection-related epilepsy syndrome after lidocaine plus MgSO4 treatment in a 12-year-old girl. 61
27358770 2016
49
Rasmussen Syndrome and Other Inflammatory Epilepsies. 61
26060905 2015
50
Effect of levetiracetam in acute encephalitis with refractory, repetitive partial seizures during acute and chronic phase. 61
25174548 2015

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

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....