NORSE
MCID: NWN001
MIFTS: 20

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 53 59
Norse 53 59
De Novo Cryptogenic Refractory Multifocal Febrile Status Epilepticus 53
New Onset Refractory Status Epilepticus 53

Characteristics:

Orphanet epidemiological data:

59
new-onset refractory status epilepticus
Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA363558
ICD10 via Orphanet 34 G41.8

Summaries for New-Onset Refractory Status Epilepticus

NIH Rare Diseases : 53 New-Onset Refractory Status Epilepticus, also known as 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 heart.

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 12)
# Related Disease Score Top Affiliating Genes
1 febrile infection-related epilepsy syndrome 11.8
2 status epilepticus 10.7
3 encephalitis 10.2
4 epilepsy 10.1
5 cat-scratch disease 10.0
6 rabies 10.0
7 herpes simplex 10.0
8 herpes simplex encephalitis 10.0
9 neurosyphilis 10.0
10 primary angiitis of the central nervous system 10.0
11 phenylketonuria 10.0
12 limbic 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

Search Clinical Trials , 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:

41
Brain, Testes, Heart, Tongue

Publications for New-Onset Refractory Status Epilepticus

Articles related to New-Onset Refractory Status Epilepticus:

(show top 50) (show all 54)
# Title Authors Year
1
New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): State of the art and perspectives. ( 29476535 )
2018
2
Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. ( 29399791 )
2018
3
A favorable outcome of intensive immunotherapies for new-onset refractory status epilepticus (NORSE). ( 30083346 )
2018
4
Tocilizumab treatment for new onset refractory status epilepticus. ( 30408233 )
2018
5
New onset refractory status epilepticus (NORSE). ( 30482654 )
2018
6
Ancient DNA reveals the chronology of walrus ivory trade from Norse Greenland. ( 30089624 )
2018
7
Disequilibrium, Adaptation, and the Norse Settlement of Greenland. ( 30363683 )
2018
8
NORSE Versus FIRES: What's in a Name? ( 30464728 )
2018
9
Rabies encephalitis presenting with new-onset refractory status epilepticus (NORSE). ( 29620067 )
2017
10
New onset refractory status epilepticus due to primary angiitis of the central nervous system. ( 29062691 )
2017
11
Successful surgical management of New Onset Refractory Status Epilepticus (NORSE) presenting with gelastic seizures in a 3A year old girl. ( 28725554 )
2017
12
Plasma Exchange as a Therapeutic Modality in a Rare Case of Cryptogenic New Onset Refractory Status Epilepticus (NORSE). ( 28892919 )
2017
13
Survey of the diagnostic and therapeutic approach to new-onset refractory status epilepticus. ( 28222319 )
2017
14
New-onset refractory status epilepticus treated with vagus nerve stimulation: A case report. ( 28267658 )
2017
15
New-Onset Refractory Status Epilepticus Associated With the Use of Synthetic Cannabinoids. ( 28366218 )
2017
16
New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features. ( 28396650 )
2017
17
18F-FDG PET/MRI in cryptogenic new-onset refractory status epilepticus: a potential marker of disease location, activity and prognosis? ( 28991656 )
2017
18
A spicy status: Synthetic cannabinoid (spice) use and new-onset refractory status epilepticus-A case report and review of the literature. ( 29238581 )
2017
19
Limbic and new onset refractory tonic status epilepticus (NORSE) in anti-NMDAR encephalitis. ( 30214986 )
2017
20
New-Onset Refractory Status Epilepticus: More Investigations, More Questions. ( 27462243 )
2016
21
New-onset refractory status epilepticus: Etiology, clinical features, and outcome. ( 26296517 )
2015
22
'The great imitator': Neurosyphilis and new-onset refractory status epilepticus (NORSE) syndrome. ( 25859427 )
2015
23
New-onset refractory status epilepticus (NORSE)--The potential role for immunotherapy. ( 26010959 )
2015
24
New-onset refractory status epilepticus in an adult with an atypical presentation of cat-scratch disease: successful treatment with high-dose corticosteroids. ( 26044784 )
2015
25
An Unusual Case of Triazophos Poisoning Presenting with New-Onset Refractory Status Epilepticus. ( 26862284 )
2015
26
New Onset Refractory Status Epilepticus in a Young Man with H1N1 Infection. ( 25379313 )
2014
27
New onset refractory status epilepticus as an unusual presentation of a suspected organophosphate poisoning. ( 25580311 )
2014
28
Plasma exchange in cryptogenic new onset refractory status epilepticus. ( 23068971 )
2013
29
Successful surgical treatment of an inflammatory lesion associated with new-onset refractory status epilepticus. ( 23724839 )
2013
30
New onset refractory status epilepticus (NORSE) as the heralding manifestation of herpes simplex encephalitis. ( 23887985 )
2013
31
Five cases of new onset refractory status epilepticus (NORSE) syndrome: outcomes with early immunotherapy. ( 23333762 )
2013
32
New-onset refractory status epilepticus mimicking herpes virus encephalitis. ( 24163672 )
2013
33
New-onset refractory status epilepticus with restricted DWI and neuronophagia in the pulvinar. ( 20308685 )
2010
34
Cryptogenic New Onset Refractory Status Epilepticus (NORSE) in adults-Infectious or not? ( 19013586 )
2009
35
New onset refractory status epilepticus (NORSE). ( 19356770 )
2009
36
Norse Greenland settlement: reflections on climate change, trade, and the contrasting fates of human settlements in the North Atlantic Islands. ( 21847839 )
2007
37
Comparing Norse animal husbandry practices: paleoethnobotanical analyses from Iceland and Greenland. ( 21847841 )
2007
38
The NORSE (new-onset refractory status epilepticus) syndrome: defining a disease entity. ( 16123813 )
2005
39
mtDna and the islands of the North Atlantic: estimating the proportions of Norse and Gaelic ancestry. ( 11179019 )
2001
40
Norse-led protest stubs it out. ( 11965953 )
2000
41
Atopic diseases in Sami and Norse schoolchildren living in northern Norway. ( 10565563 )
1999
42
Palatine torus in the Greenlandic Norse. ( 1605314 )
1992
43
The illicit love visit: an archaeology of old Norse sexuality. ( 11622767 )
1991
44
The Tromsø heart study. Distribution of serum cholesterol between high density and lower density lipoproteins in subjects of Norse, Finnish and Lappish ethnic origin. ( 204160 )
1978
45
Littorina littorea: occurrence in a northern Newfoundland beach terrace, predating Norse settlements. ( 4862838 )
1968
46
Littorina littorea: Occurrence in a Northern Newfoundland Beach Terrace, Predating Norse Settlements. ( 17737491 )
1968
47
Blood group study of old Norse isolates in Upper Setesdal, Norway. ( 6036762 )
1967
48
Littorina littorea as an Indicator of Norse Settlements. ( 17835593 )
1963
49
Littorina littorea: An Indicator of Norse Settlement in North America? ( 17841569 )
1963
50
The extinction of the Norse settlements in Greenland. ( 13434726 )
1956

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

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