New onset refractory status epilepticus (NORSE)
Claudine Sculier1, Nicolas Gaspard2
1Département de Neurologie, Université Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium; Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Seizure
|November 29, 2018
Summary
New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) are rare, devastating neurological conditions. Early recognition and prompt treatment are crucial for improving outcomes in these challenging epilepsy syndromes.
Area of Science:
- Neurology
- Epileptology
- Pediatric Neurology
Background:
- New-onset refractory status epilepticus (NORSE) is defined as refractory status epilepticus (RSE) without prior epilepsy or clear acute cause.
- Febrile infection-related epilepsy syndrome (FIRES) is a subgroup of NORSE, preceded by a febrile illness.
Purpose of the Study:
- To summarize clinical features, work-up, treatment, and prognosis of NORSE and FIRES.
- To provide a standardized diagnostic algorithm for these rare epilepsy syndromes.
Main Methods:
- Narrative review of the medical literature on NORSE and FIRES.
- Analysis of clinical presentations, diagnostic challenges, and treatment strategies.
Main Results:
- NORSE/FIRES primarily affect children and young adults, often with prodromal flu-like symptoms.
- Most cases progress to super refractory status epilepticus (SRSE) with high mortality (12-27%) and long-term disability.
- Autoimmune encephalitis is the most common identified cause; immunotherapy may be beneficial.
Conclusions:
- NORSE and FIRES are rare but severe epilepsy syndromes requiring urgent recognition.
- Comprehensive work-up is essential for identifying underlying causes and initiating timely treatment.
- Prompt management, including potential immunotherapy, can improve outcomes for patients with NORSE and FIRES.
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