NORSE
MCID: NWN001
MIFTS: 21

New-Onset Refractory Status Epilepticus (NORSE)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for New-Onset Refractory Status Epilepticus

MalaCards integrated aliases for New-Onset Refractory Status Epilepticus:

Name: New-Onset Refractory Status Epilepticus 52 58
Norse 52 58
De Novo Cryptogenic Refractory Multifocal Febrile Status Epilepticus 52
New Onset Refractory Status Epilepticus 52

Characteristics:

Orphanet epidemiological data:

58
new-onset refractory status epilepticus
Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 33 G41.8
Orphanet 58 ORPHA363558

Summaries for New-Onset Refractory Status Epilepticus

NIH Rare Diseases : 52 New-onset refractory status epilepticus (NORSE) is a life-threatening condition in which a healthy person without a previous history of seizures begins having seizures (new-onset) and within several days the seizure activity progresses to status epilepticus (SE). SE describes a state in which a person has one prolonged seizure or a cluster of seizures without recovery time in between the seizures. When the cause of SE cannot initially be identified and it cannot be controlled with standard anti-seizure medicine (refractory), the condition is called NORSE. SE leads to a comatose state, either directly due to the seizure activity or due to anesthesia therapy used try to stop the seizure activity. In NORSE, SE may last days, weeks, or months. NORSE can affect people of all ages, but is more common in young adults and children. After initial testing fails to find a cause for the refractory SE, more extensive testing is performed. In just under half of the cases of NORSE, a probable or possible cause is eventually found. When the cause cannot be found, cryptogenic NORSE is diagnosed. Cryptogenic is the medical term for unknown cause. In many cases of cryptogenic NORSE, the person had a fever between 24 hours and two weeks before seizures began. These cases are classified as a sub-type of NORSE called Febrile Infection-Related Epilepsy Syndrome (FIRES). Despite the fever, no known infection has been found to cause FIRES, and the fever may or may not be present when the seizures begin. There is currently no evidence that cryptogenic NORSE, including FIRES, is hereditary. If an underlying cause for NORSE is found, the treatment depends on the cause. There is no standard treatment for cryptogenic NORSE, including FIRES. Cryptogenic NORSE does not respond to standard treatment of SE and requires additional treatment with other anti-seizure medicines and/or medically inducing a coma using anesthesia to control seizures. Anesthesia therapy may not be completely effective in stopping seizure activity (super-refractory SE). NORSE can cause significant brain damage and between 20-30% of people do not survive. In some cases, people with NORSE have made a full recovery, but in most cases survivors will have life long epilepsy as well as mental and physical disabilities. Of note, FIRES previously was considered a separate condition that occurred only in children, while NORSE was described only in adults. However, FIRES is now considered a sub-type of cryptogenic NORSE (distinguished only by a preceding fever), and both can apply to people of any age.

MalaCards based summary : New-Onset Refractory Status Epilepticus, also known as norse, is related to febrile infection-related epilepsy syndrome and status epilepticus. Affiliated tissues include brain, testes and spinal cord.

Related Diseases for New-Onset Refractory Status Epilepticus

Diseases related to New-Onset Refractory Status Epilepticus via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 39)
# Related Disease Score Top Affiliating Genes
1 febrile infection-related epilepsy syndrome 12.0
2 status epilepticus 10.9
3 encephalitis 10.6
4 epilepsy 10.4
5 autoimmune encephalitis 10.4
6 ocular motor apraxia 10.3
7 rabies 10.3
8 visual epilepsy 10.3
9 seizure disorder 10.3
10 limbic encephalitis 10.2
11 phenylketonuria 10.0
12 arts syndrome 10.0
13 dermatitis, atopic 10.0
14 pervasive developmental disorder 10.0
15 encephalopathy 10.0
16 schizophrenia 10.0
17 hypothalamic hamartomas 10.0
18 focal cortical dysplasia, type ii 10.0
19 la crosse encephalitis 10.0
20 asymptomatic neurosyphilis 10.0
21 meningoencephalitis 10.0
22 japanese encephalitis 10.0
23 hemiplegia 10.0
24 cat-scratch disease 10.0
25 neutropenia 10.0
26 carotid stenosis 10.0
27 diarrhea 10.0
28 antiphospholipid syndrome 10.0
29 syphilis 10.0
30 compartment syndrome 10.0
31 hyperthyroidism 10.0
32 herpes simplex 10.0
33 vasculitis 10.0
34 herpes simplex encephalitis 10.0
35 neurosyphilis 10.0
36 primary angiitis of the central nervous system 10.0
37 persistent vegetative state 10.0
38 syncope 10.0
39 infectious encephalitis 10.0

Graphical network of the top 20 diseases related to New-Onset Refractory Status Epilepticus:



Diseases related to New-Onset Refractory Status Epilepticus

Symptoms & Phenotypes for New-Onset Refractory Status Epilepticus

Drugs & Therapeutics for New-Onset Refractory Status Epilepticus

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Health Indicators, Functioning and Health Care Services in Youths and Adults With Intellectual Disabilities. A Cross-sectional Multicentre Study. Completed NCT03889002

Search NIH Clinical Center for New-Onset Refractory Status Epilepticus

Genetic Tests for New-Onset Refractory Status Epilepticus

Anatomical Context for New-Onset Refractory Status Epilepticus

MalaCards organs/tissues related to New-Onset Refractory Status Epilepticus:

40
Brain, Testes, Spinal Cord, Heart, Temporal Lobe, Tongue

Publications for New-Onset Refractory Status Epilepticus

Articles related to New-Onset Refractory Status Epilepticus:

(show top 50) (show all 190)
# Title Authors PMID Year
1
New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. 61 52
29476535 2018
2
Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. 52 61
29399791 2018
3
Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents. 52 61
29745347 2018
4
Etiological assessment of status epilepticus. 61
32331701 2020
5
New-Onset Refractory Status Epilepticus-Related Claustral Hyperintensities. 61
32544915 2020
6
How patients and clinicians experience the utility of a personalized clinical feedback system in routine practice. 61
32558933 2020
7
New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. 61
32372459 2020
8
A case of very prolonged new onset refractory status epilepticus (NORSE) with no evidence of autoimmune activation and a good neurological recovery. 61
32458254 2020
9
The PET Sandwich: Using Serial FDG-PET Scans with Interval Burst Suppression to Assess Ictal Components of Disease. 61
32253731 2020
10
Abdominal Compartment Syndrome: A Rare Complication of Pentobarbital Treatment for New-Onset Refractory Status Epilepticus. 61
32223826 2020
11
Editors' note: New onset refractory status epilepticus research: What is on the horizon? 61
32229637 2020
12
Reader response: New onset refractory status epilepticus research: What is on the horizon? 61
32229638 2020
13
Author response: New onset refractory status epilepticus research: What is on the horizon? 61
32229639 2020
14
Reversible splenial lesion in a patient with new-onset refractory status epilepticus (NORSE). 61
31909228 2020
15
Is Dupuytren's disease really a 'disease of the Vikings'? 61
31663799 2020
16
Troy D. Zars: a personal tribute to a scientist, colleague, and friend. 61
32233837 2020
17
New-onset super refractory status epilepticus: A case-series. 61
31757748 2020
18
Rabies encephalitis presenting with new-onset refractory status epilepticus-Update. 61
32190425 2020
19
Is rat hippocampus section immunostaining an indicator for immunotherapy in cryptogenic adult new-onset refractory status epilepticus (NORSE)? 61
32066091 2020
20
New-Onset Refractory Status Epilepticus with Underlying Autoimmune Etiology: a Case Report. 61
32435752 2020
21
New-onset refractory status epilepticus: A retrospective cohort study. 61
31830676 2020
22
Hashimoto encephalopathy in the 21st century. 61
31882532 2020
23
New-Onset Refractory Status Epilepticus in Children: Etiologies, Treatments, and Outcomes. 61
31568262 2020
24
New-onset Refractory Status Epilepticus Involving the Limbic System, Spinal Cord, and Peripheral Nerves. 61
31511489 2020
25
Buried in water, burdened by nature-Resilience carried the Iron Age people through Fimbulvinter. 61
32315354 2020
26
Prognostic significance of subsequent extra-temporal involvement in cryptogenic new onset refractory status epilepticus (NORSE) initially diagnosed with limbic encephalitis. 61
31669912 2019
27
Continuous EEG Findings in Autoimmune Encephalitis. 61
31800465 2019
28
Disappearance of Icelandic Walruses Coincided with Norse Settlement. 61
31513267 2019
29
Sagas of the Solanaceae: Speculative ethnobotanical perspectives on the Norse berserkers. 61
31404578 2019
30
Avoiding anaesthetics after multiple failed drug-induced comas: an unorthodox approach to management of new-onset refractory status epilepticus (NORSE). 61
31708492 2019
31
Encephalitis with Anti-SOX1 Antibodies Presenting with New-Onset Refractory Status Epilepticus. 61
31591846 2019
32
Bilateral fronto-insular FLAIR hyperintensities: discussion on a case of new-onset refractory status epilepticus. 61
31004289 2019
33
The genetic landscape of Scotland and the Isles. 61
31481615 2019
34
Reversible splenial lesion in new-onset refractory status epilepticus: A case report. 61
31254907 2019
35
[Recent progress in autoimmune encephalitis and its related disorders]. 61
31341132 2019
36
Schizophrenia following new-onset refractory status epilepticus secondary to antiphospholipid syndrome. 61
31380826 2019
37
Predicting the loss of organic archaeological deposits at a regional scale in Greenland. 61
31296877 2019
38
New horizons at L'Anse aux Meadows. 61
31308231 2019
39
New onset refractory status epilepticus (NORSE). 61
30482654 2019
40
Communication Challenges: A Spotlight on New-Onset Refractory Status Epilepticus. 61
30935709 2019
41
Rare Complication of Carotid Stenting: New-Onset Refractory Status Epilepticus: A Study of Five Patients. 61
31007435 2019
42
New onset refractory status epilepticus research: What is on the horizon? 61
30894443 2019
43
Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. 61
30779222 2019
44
A New Hose to Extinguish the FIRES? 61
30955421 2019
45
FDG-PET and MRI in the Evolution of New-Onset Refractory Status Epilepticus. 61
30679215 2019
46
Super-Refractory Status Epilepticus Treated with High Dose Perampanel: Case Series and Review of the Literature. 61
31565443 2019
47
Vagus Nerve Stimulation (VNS) in Super Refractory New Onset Refractory Status Epilepticus (NORSE). 61
30805233 2019
48
Making way for a clinical feedback system in the narrow space between sessions: navigating competing demands in complex healthcare settings. 61
31700530 2019
49
Tocilizumab treatment for new onset refractory status epilepticus. 61
30408233 2018
50
Unusual cause of fever, vision loss and super refractory status epilepticus in association with simian virus 40 (SV40). 61
30317196 2018

Variations for New-Onset Refractory Status Epilepticus

Expression for New-Onset Refractory Status Epilepticus

Search GEO for disease gene expression data for New-Onset Refractory Status Epilepticus.

Pathways for New-Onset Refractory Status Epilepticus

GO Terms for New-Onset Refractory Status Epilepticus

Sources for New-Onset Refractory Status Epilepticus

3 CDC
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10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
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32 ICD10
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48 NCI
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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
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