New-onset refractory status epilepticus: A retrospective cohort study

Area of Science:

• Neurology
• Epileptology
• Immunology

Background:

• New-onset refractory status epilepticus (NORSE) is a critical neurological emergency.
• Understanding NORSE's characteristics, causes, and outcomes is vital for patient management.

Purpose of the Study:

• To comprehensively describe the clinical, laboratory, neuroimaging, and electroencephalographic features of adult NORSE.
• To investigate the etiology, treatment strategies, and short- and long-term outcomes in adult NORSE patients.

Main Methods:

• Retrospective cohort study of consecutive adult patients diagnosed with NORSE.
• Data collected from 2010 to 2018 at a single institution.

Main Results:

• Of 20 NORSE patients, 45% had prodromal febrile illness and 60% showed CSF inflammation.
• 30% met criteria for definite autoimmune encephalitis (AE), 40% for probable AE.
• 70% received immunotherapy; 75% progressed to super-refractory status epilepticus (SRSE); 35% died within six months.
• 40% of survivors had good outcomes (mRS 0-2); 80% developed epilepsy, with 75% of those having drug-resistant epilepsy.

Conclusions:

• NORSE is a syndrome with high mortality and risk of intractable epilepsy, often linked to autoimmune encephalitides.
• Despite extensive evaluation, a clear etiology remained unidentified in the majority of cases.
• Prospective studies are essential to establish optimal diagnostic and therapeutic approaches for NORSE.