From the Guidelines
New-onset refractory status epilepticus (NORSE) is a rare but severe condition characterized by prolonged seizures that don't respond to standard anti-seizure medications in patients without a history of epilepsy. The management of NORSE is crucial to prevent further brain damage and improve patient outcomes.
Key Characteristics of NORSE
- Prolonged seizures that don't respond to standard anti-seizure medications
- Patients without a history of epilepsy
- Requires aggressive seizure control and comprehensive diagnostic workup
Management of NORSE
- Initial management includes aggressive seizure control with benzodiazepines like lorazepam or midazolam, followed by second-line agents such as fosphenytoin, valproate, or levetiracetam 1
- For refractory cases, anesthetic agents like propofol, midazolam, or ketamine may be necessary with continuous EEG monitoring in an ICU setting
- Immunotherapy with IV methylprednisolone and/or IVIG should be considered early, as many NORSE cases have an autoimmune etiology
Diagnostic Workup
- Comprehensive diagnostic workup including brain MRI, CSF analysis, autoimmune panels, and infectious disease testing is crucial to identify underlying causes
- Multidisciplinary management involving neurologists, intensivists, and immunologists is necessary to address both seizure control and the underlying etiology, which improves outcomes in this life-threatening condition 1 Key points to consider in the management of NORSE include:
- Aggressive seizure control
- Comprehensive diagnostic workup
- Multidisciplinary management
- Early consideration of immunotherapy These key points are crucial in improving patient outcomes and preventing further brain damage in NORSE patients.
From the Research
Definition and Characteristics of New-Onset Refractory Status Epilepticus (NORSE)
- NORSE is a recently described entity characterized by refractory status epilepticus in individuals without a prior history of epilepsy 2, 3, 4, 5.
- It is often difficult to treat due to the cryptogenic etiology, with no clear acute or active structural, toxic, or metabolic cause 2, 3, 5.
- NORSE mainly affects school-age children and young adults, with a prodromal phase of flu-like symptoms preceding the onset of status epilepticus in many cases 3.
Clinical Features and Diagnosis
- Status epilepticus in NORSE usually starts with repeated focal seizures with secondary bilateralization, often evolving to super refractory status epilepticus (SRSE) 3.
- No specific imaging or laboratory abnormalities have been identified for early diagnosis, and half of adult cases remain of unknown etiology 3.
- A standardized diagnostic algorithm is recommended, including an extensive evaluation with MRI brain, CSF studies, radiologic scans, and serological autoimmune and infectious investigations 2, 3.
Treatment and Prognosis
- Treatment of NORSE often involves antiepileptic medications, anesthetics, and immunotherapies, with variable success rates 2, 3, 4, 6.
- Early recognition and treatment are crucial for improving outcomes, which are often poor, with high short-term mortality and long-term disability and epilepsy 3, 5.
- Novel treatments, such as plasma exchange therapy 2 and vagus nerve stimulator (VNS) therapy 4, have shown promise in some cases, but further research is needed to establish their efficacy.