ABOUT NORSE & FIRES

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NORSE (New Onset Refractory Status Epilepticus) is a rare and catastrophic neurological condition that strikes suddenly in previously healthy children and adults with no history of epilepsy. It causes relentless, life-threatening seizures that are exceptionally difficult, and sometimes impossible, to stop.

Urgent admittance to intensive care is usually required, and many patients spend weeks or even months in an induced coma, undergoing complex, life-saving treatment.

During the acute stage of NORSE, around 1 in 5 people do not survive. For survivors, recovery can be a long and complex journey involving rehabilitation, chronic epilepsy, neurological disability and ongoing support. Although some survivors can make a good recovery.

Continue reading to learn more about NORSE, FIRES and the latest research.

THE IMPACT CAN BE DEVASTATING

Around 1 in 5 people do not survive the acute stage.
There is currently no cure.
The underlying cause remains poorly understood.
Recovery is possible, but survivors can be left with lifelong neurological disability, chronic epilepsy, and complex long-term care needs.

ABOUT FIRES

FIRES (Febrile Infection Related Epilepsy Syndrome) is a sub-type of NORSE, that most commonly affects children, although adults can also be affected. It develops following a fever or febrile illness, with seizures beginning between 24 hours and 14 days later.

ABOUT THE INFORMATION ON THIS PAGE

We know how overwhelming NORSE and FIRES can feel. That's why we've created this page to explain these rare conditions in clear, plain English.

It brings together current international expert guidance and published medical research to help patients, families, healthcare professionals and the wider public to better understand NORSE and FIRES.

The information reflects the current international understanding of these conditions and is informed by published research from leading researchers, clinicians and organisations such as the NORSE Institute and King's College London. As our understanding of NORSE and FIRES continues to evolve, we'll update this page to reflect the latest evidence.

Key References

Mantoan Ritter L, Nashef L. New-onset refractory status epilepticus (NORSE). Practical Neurology. 2021;21(2):119–127. A comprehensive UK overview of the current understanding, diagnosis and management of NORSE and FIRES.
BMJ Paediatrics
Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018. The internationally accepted consensus definitions for NORSE and FIRES.
Sculier C, Gaspard N. New-onset refractory status epilepticus (NORSE). Seizure. 2019. An overview of the condition, including current understanding of causes, immune mechanisms and treatment strategies
NORSE Institute. International patient, family and clinician resource dedicated to improving awareness, support and research into NORSE and FIRES.
Mantoan Ritter L, Selway R. Perspective: Vagal nerve stimulation in the treatment of new-onset refractory status epilepticus. Frontiers in Neurology. 2023. Reviews current understanding of NORSE, treatment-resistant seizures and future directions for research.

WHAT HAPPENS IN THE ACUTE STAGE

In the acute stage of NORSE and FIRES seizures begin suddenly and without warning. They escalate rapidly over hours or days into relentless, life-threatening seizures, that are exceptionally difficult to stop. This is known as refractory status epilepticus.

For many people, the seizures are preceded by what appears to be a mild viral illness, such as a cold, flu or stomach bug, something so common that no family or doctor could have predicted or prevented what followed.

At the same time as trying to stop the seizures, doctors will carry out extensive tests to look for the underlying cause. These may include brain scans, EEG monitoring, blood tests, spinal fluid (lumbar puncture) tests and other investigations to identify or rule out infections, autoimmune conditions, metabolic disorders and other possible causes.

Many patients require weeks or even months in intensive care, where treatment may include:

a medically induced coma
multiple anti-seizure medications and anaesthetic drugs
immunotherapies

Rapid seizure control is critical to reduce the risk of brain injury and other serious complications.

ARE NORSE & FIRES DIAGNOSES?

Not exactly. NORSE describes what is happening, not what is causing it. Doctors must urgently investigate many possible causes, of the sudden onset of seizures, including infections, autoimmune disorders, metabolic conditions, structural brain abnormalities.

In around half of NORSE cases, no cause is ever found. These are known as cryptogenic NORSE.

NORSE and FIRES are extremely rare, affecting around one in a million people each year. However, experts believe they are also under-recognised and under-reported.

HOW IS NORSE & FIRES CONFIRMED?

There is no single test or biomarker that can confirm NORSE or FIRES. Instead, doctors bring together a person's symptoms, medical history, test results and by ruling out other possible causes. This makes identification especially challenging.

NORSE and FIRES are not diseases confirmed by a single test. They describe a clinical syndrome - a pattern of symptoms that occurs together - after other possible causes have been carefully excluded.

Key features of NORSE & FIRES:

Distinct Pattern: Often begins after a mild illness (FIRES after a febrile illness), followed by relentless, hard-to-control seizures.

Often No Cause Found: In most cases the exact cause is never found, though immune and inflammatory processes may play a role.

Diagnosis of Exclusion: Extensive testing is required to eliminate other possible causes with similar symptoms.

WHY ARE THE SEIZURES SO DIFFICULT TO STOP?

The seizures are so difficult to stop because they don't behave like typical epilepsy. Standard anti-seizure medications are often not enough because they do not address the underlying inflammatory and immune response that researchers believe contributes to the seizures in many patients.

Growing evidence suggests that, in many cases of cryptogenic NORSE and FIRES, the seizures may result from a combination of genetic susceptibility, immune dysregulation, inflammation and other factors that researchers are still working to understand.

Researchers believe this combination of factors may contribute to an abnormal immune and inflammatory response, including within the brain, which may help drive the prolonged, aggressive and treatment-resistant seizures characteristic of NORSE and FIRES.

This helps explain why anti-seizure medications alone are often not enough to stop the seizures, and why many patients also receive immunotherapies alongside other treatments.

Because researchers believe inflammation and immune dysregulation contribute to the seizures in many patients, treatment focuses on controlling the seizures while also targeting the suspected inflammatory and immune response. This is why many patients receive a combination of anti-seizure medications, anaesthetic drugs and immunotherapies.

Note: Immunotherapies are used to try to reduce the suspected inflammation that may be contributing to the seizures. Learn more below.

WHY OUTCOMES ARE SO POOR

NORSE and FIRES are neurological emergencies. Brain injury can occur because prolonged, seizures and an intense inflammatory response can damage the brain. Early recognition, and specialist treatment are critical to reduce this damage and improve outcomes.

Prolonged Seizures: Your brain is made up of billions of brain cells (neurons) that constantly communicate using tiny electrical and chemical messages. During a seizure, these messages become uncontrolled, creating an electrical storm in the brain.

If seizures continue for too long (over 30 minutes), they can damage brain cells. The longer the seizures continue, the greater the risk of permanent brain injury, which can affect memory, thinking, speech and movement.

Prolonged seizures can increase inflammation within the brain. This inflammation may then increase the likelihood of further seizures and make them even more difficult to stop, creating a vicious cycle that can make NORSE and FIRES particularly difficult to treat.

Inflammation: In many patients, researchers believe the body's immune and inflammatory responses become dysregulated. This may lead to the excessive release of inflammatory proteins called cytokines, which can contribute to inflammation within the brain. This inflammation may cause brain swelling (cerebral oedema), damage brain cells and make seizures more difficult to stop, creating yet another vicious cycle.

Brain Shrinking & Scarring: In some patients, repeated seizures and ongoing inflammation can lead to brain shrinkage (atrophy) and the formation of scar tissue. These changes can cause lasting neurological challenges and often make seizures even more resistant to treatment.

Stopping the seizures is only the beginning. Many survivors of NORSE and FIRES continue to live with lifelong neurological disability, chronic epilepsy and complex rehabilitation needs.

WHAT ARE CYTOKINES?

Cytokines are natural proteins that act as chemical messengers, helping the immune system communicate and fight infection.

Researchers believe that in many cases of cryptogenic NORSE and FIRES, the normal immune response becomes dysregulated, and doesn't switch off as it should, leading to the excessive release of certain inflammatory cytokines.

These inflammatory signals may contribute to inflammation within the brain, make seizures more difficult to stop, and increase the risk of brain injury.

STAGES OF NORSE & FIRES

The Intial Stage

NORSE and FIRES begin suddenly and explosively, usually after a mild illness. This is known as the acute stage - the most dangerous and life-threatening phase of the condition.

Seizures escalate rapidly becoming continuous and resistant to treatment. This is known as super-refractory status epilepticus.

No clear cause is usually identified in the early stages.

Standard anti-seizure medications often fail to control the seizures.

Treatment Protocol - Acute Stage

There is no specific or universally effective treatment for NORSE and FIRES. Neurologists and intensive care teams must work together to tailor the most appropriate plan for each individual. The NORSE Institute in the US has developed a recommended treatment algorithm for clinicians — click here to view it.

Urgent Critical Care Is Essential

Patients need urgent admittance to a specialist critical care unit. Children and adults often require to be placed in a medically induced coma for weeks or even months in an attempt to stop the seizures and limit potential brain injury.

Because NORSE and FIRES are life-threatening and life-changing, treatment is typically aggressive and may include:

Multiple anti-seizure medications

Anaesthetic/sedative drugs

Immunotherapy to calm suspected brain inflammation

Intensive monitoring and organ support

This prolonged and intense medical intervention places enormous physical and emotional strain on both the patient and their family.

A Multi-disciplinary Team Is Essential

NORSE and FIRES are highly complex and require coordinated care across multiple specialties, including:

Neurology and critical care

Immunology and rheumatology

Infectious disease

Anaesthesiology

Nutrition and metabolic support

Palliative care

These teams work together to control seizures, manage complications, and provide vital supportive care.

Why Early Recognition Matters

Early, specialist treatment is critical to improve outcomes and limit long-term injury - TIME IS BRAIN. Prolonged seizures can cause irreversible brain damage if not treated quickly and effectively. Every minute counts.

The Chronic Stage

Surviving the acute phase is only the beginning. When seizures gradually reduce, patients enter the chronic stage - the start of a long and often extremely challenging recovery.

While some children and adults make a good recovery, many are left with lasting challenges because of the severity of seizures and brain inflammation.

Common long-term effects include:

Cognitive or developmental delays

Physical impairments

Emotional and psychological challenges

Chronic, often drug-resistant epilepsy

Social and learning difficulties

Abnormal EEG activity

Risk of regression

Families may face long-term care needs, frequent hospital visits, ongoing therapies, and significant reduced quality of life for both survivors and families.

SURVIVING NORSE & FIRES

Whilst many survive NORSE and FIRES, those who do, can face significant, long-term neurological challenges, including

Developmental delays

Cognitive difficulties

Motor impairments

Chronic and difficult-to-control epilepsy

Many survivors require ongoing therapies such as physical therapy, cognitive rehabilitation, and speech therapy to help improve their abilities and quality of life.

Managing drug-resistant epilepsy, cognitive challenges, and mental health needs requires a multidisciplinary care team working together to support both the patient and their family.

Supporting Families and Caregivers

The impact of NORSE and FIRES extends beyond the individual. Families and caregivers experience immense emotional and psychological stress throughout this lifelong journey. Access to counselling and support groups can provide vital comfort, understanding, and connection with others facing similar challenges.

Post-Intensive Care Syndrome (PICS)

Both patients and their families are at risk of Post-Intensive Care Syndrome (PICS). This syndrome is where the experience of critical care leads to long-term health impacts - physically, mentally, and emotionally. Read here this insightful article by Nora Wong, Founder of the NORSE Institute, and Raquel Farias-Moeller - click here.

Apply for Family Grants

If your family member is experiencing NORSE or FIRES, we want to help. Families can apply for a grant to support ongoing care and get the resources they need.

Reach out to us today to find out how we can assist you during this difficult time. To apply for a family grant, please contact us on rachel@samssuperheroes.co.uk.

Please note you must be resident in England or Wales.

MORE ABOUT FAMILY GRANTS