I thought the word NORSE related to Vikings, not a seizure disorder. So I was in disbelief when a doctor proposed that our son’s continuous cascade of seizures was due to NORSE or new-onset refractory status epilepticus.

NORSE is defined as an acute, prolonged crisis in which one epilepsy seizure follows another without recovery of consciousness and with no known cause for these seizures. Daniel had no history of seizures, and there was no history of epilepsy in the family. Even the word epilepsy was unfamiliar to me.

Currently, there is no established treatment for NORSE, and, by definition, it does not respond to the first two lines of standard anti-seizure drugs. Although some preliminary findings have suggested that early immunotherapy can be beneficial, much more research is needed.

What is known about NORSE is that it requires immediate, urgent treatment and supportive medical care that necessitates the coordinated expertise of ICU and epilepsy teams. The expected outcomes for NORSE are devastating: roughly one third of the people die, one third suffer profound morbidity, and a small third recover varying function but most live with recurring epilepsy for the rest of their lives. 

Daniel's Story

Daniel Wong

Our son, Daniel, age 22, had just graduated from Stanford and was working in a new job as a financial analyst in an investment fund. He had complained of having the flu but said he was recovering when we spoke with him on a Sunday afternoon in October 2013. The next day, his roommate found him standing, half-dressed but unresponsive in their apartment. Daniel was rushed to the hospital where he began to have recurring seizures and a high fever.

Since none of the anti-epileptic drugs administered could stop his seizures, he was placed in an induced coma to protect his brain. These and other measures, including attempting a ketogenic diet and IVIG (intravenous immunoglobulin) later in his treatment, proved futile. Our Daniel died after 78 days in an induced coma, never once regaining consciousness.

My plea is to the doctors:

Use and communicate the term NORSE with other doctors. Share your knowledge of NORSE by writing case studies.

The term NORSE is a relatively new one. Although uncommon, these cases have long been observed, and they frequently have been generally categorized as encephalitis.   

There is an ongoing registry of NORSE patients through the Critical Care EEG Monitoring Research Consortium (Principal investigator Nicolas Gaspard). The researchers involved with this registry all have experience with NORSE patients and they are willing to share information. I urge doctors to collaborate and to write up their NORSE cases so that they can be compiled to complement the information from the NORSE registry. 

Discuss the possibility of the NORSE diagnosis, not only with all medical staff on the case, but also the family (and the patient when possible).

An open discussion of NORSE as a possible diagnosis allows everyone to be onboard with the treatment plan for this uncharted disorder. Given the high mortality expected, a discussion of palliative care should take place long before hard decisions are imminent.

Although NORSE is presumed to be uncommon, with greater education and awareness, diagnoses may rise. Hopefully, NORSE research will yield not only effective treatment for people with NORSE, but also offer insight into less catastrophic forms of epilepsy and their treatments.

In the meantime, people with NORSE and their families may feel shell-shocked by their singular experience. People with NORSE and families who would like to connect with others affected by NORSE can email me or visit the NORSE Institute.

Nora Wong