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Start free with EleplanNew-onset refractory status epilepticus
ORPHA:363558Disease
Also called NORSE
What it is
New-onset refractory status epilepticus is an acute encephalopathy with inflammation-mediated status epilepticus characterized by an acute refractory status epilepticus, typically of the tonic-clonic type, following prodromal symptoms of confusion, fever, fatigue, headache, symptoms of gastrointestinal or upper respiratory tract infection, behavioral changes or hallucinations. Brain MRI abnormalities and abnormal findings in CSF, including pleocytosis and/or elevated protein levels, are frequently found during acute episode. Treatment-resistant epilepsy, cognitive and psychiatric impairments are usual consequences.
Key facts
- Age of onset
- Adult
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Very common80–99%
3Common30–79%
18- Abnormal circulating interleukin concentration
- Abnormal head movements
- Autoimmunity
- Bilateral tonic-clonic seizure with focal onset
- Brain imaging abnormality
- Cognitive impairment
- CSF pleocytosis
- EEG with frontal epileptiform discharges
- EEG with generalized slow activity
- EEG with temporal epileptiform discharges
- Facial tics
- Fever
- Focal autonomic seizure
- Focal aware motor seizure
- Focal impaired awareness seizure
- Focal T2 hyperintense basal ganglia lesion
- Functional motor deficit
- Increased CSF protein concentration
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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