Disease at a Glance

Summary
New-onset refractory status epilepticus (NORSE) occurs when a person without a previous history of seizures, experiences status epilepticus (SE) without a clear cause. SE describes a state in which a person has one prolonged seizure, or a cluster of seizures without recovery time in between. The symptoms of NORSE typically begin with a mild fever and general symptoms of illness. People with NORSE may also experience behavioral and cognitive symptoms. SE associated with NORSE can last days, weeks, or months and may lead to a comatose state. A likely cause is identified in about half of all people with NORSE. The most common causes of NORSE include autoimmune disorders, paraneoplastic disorders, and viral encephalitis. Studies indicate that changes in certain genes, including the SCN2A and IL1RN gene(s) may increase the risk to develop NORSE. When the cause cannot be found, cryptogenic NORSE is diagnosed. Most people with cryptogenic NORSE experience a fever prior to seizure development. These cases are classified as a sub-type of NORSE, called Febrile Infection-Related Epilepsy Syndrome (FIRES). A diagnosis of NORSE is suspected in people who develop SE that does not respond to typical treatment and that does not have an obvious cause. A diagnostic work-up may include brain imaging, cerebrospinal fluid studies, and blood tests. Electroencephalography (EEG) is used to monitor seizures throughout the illness.
Estimated Number of People with this Disease

This section is currently in development.

What Information Does GARD Have For This Disease?

Many rare diseases have limited information. Currently GARD is able to provide the following information for this disease:

*Data may be currently unavailable to GARD at this time.
When do symptoms of this disease begin?
The most common ages for symptoms of a disease to begin is called age of onset. Age of onset can vary for different diseases and may be used by a doctor to determine the diagnosis. For some diseases, symptoms may begin in a single age range or several age ranges. For other diseases, symptoms may begin any time during a person's life.
Prenatal
Before Birth
Newborn
Birth-4 weeks
Infant
1-23 months
Child
2-11 years
Adolescent
12-18 years
Adult Selected
19-65 years
Older Adult
65+ years
The common ages for symptoms to begin in this disease are shown above by the colored icon(s).

Symptoms

These symptoms may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom.
This disease might cause these symptoms:
Nervous System

23 Symptoms

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Nervous System

The nervous system is made up of the brain, spinal cord, and nerves. Common symptoms of problems in the nervous system include trouble moving, speaking, swallowing, breathing, or learning. Problems with memory, senses, or mood may also occur. Nervous system diseases are usually diagnosed and treated by neurologists.

Causes

This section is currently in development. 

Last Updated: Nov. 8, 2021