Authors :
First Author: abdulaziz Alaskar, MBBS – Prince Sattam bin abdulaziz university
Presenting Author: Mohammed Asiry, –
Majed AlJohani, MBBS – King Saud University; Mohammed Asiry, MBBS – King Faisal Specialist Hospital and Research Center; Aleksander Dionisio, MBBS – King Faisal Specialist Hospital and Research Center; Khalid Alqadi, MBBS – King Salman Ibn Abdulaziz Medical City
Rationale: New-Onset Refractory Status Epilepticus (NORSE) is a rare and severe form of refractory status epilepticus without an apparent underlying cause at presentation or prior history of epilepsy.
This retrospective cohort study aimed to describe NORSE's clinical features, etiology, treatment, and outcomes in a quaternary-level hospital in Saudi Arabia.
Methods: This retrospective cohort study aimed to investigate NORSE patients admitted to King Faisal Specialist Hospital and Research Center in Riyadh and Jeddah between January 2010 and December 2021. Inclusion criteria involved patients over the age of 14 years who met the 2018 consensus definition for NORSE.
Data were collected from medical charts, including demographic information, comorbid conditions, prodromal symptoms, hospital course, length of stay, medications, and complications. Electroencephalogram (EEG) imaging reports, laboratory test results, and pathology data were also recorded. The severity was assessed using the Status Epilepticus Severity Score (STESS) and modified Rankin Scale (mRS) score.
The study outcomes were measured using the modified Rankin Scale (mRS), with a good outcome defined as an mRS score of 0-2 and a poor outcome defined as 3-5. Long-term outcome data included follow-up time, diagnosis of epilepsy or chronic use of immunotherapy, and the number and type of antiseizure medicines.
Results:
We found 24 adult patients presenting with NORSE between 2010 and 2021. Most patients had no comorbidities, and fever/infectious symptoms were the most common prodromal symptoms. Laboratory findings showed elevated inflammatory serum and cerebrospinal fluid markers in most patients. Brain MRI revealed T2/FLAIR hyperintensity patterns, predominantly affecting limbic and perisylvian structures. FDG-PET cerebral imaging showed focal hypermetabolism or hypometabolism in some cases. The etiology of NORSE varied, with immune-related causes being the most common. Treatment involved the use of anti-seizure medicines, intravenous anesthetics and immunotherapy. Long-term outcomes were poor, with a high mortality rate and most survivors developed drug-resistant epilepsy.
Conclusions:
This study provides valuable insights into NORSE's clinical characteristics and management, highlighting the importance of early recognition and aggressive treatment to improve outcomes with the necessity to subclassify patients according to the presumed etiology. Further research and prospective trials are needed to understand better and optimize managing this challenging condition.
Funding: NA