Clinical Trials

Inclusion Criteria - RISK Primary Inclusion Criteria: NORSE (~50 patients):

• New onset refractory status epilepticus with or without fever.
• Failure to respond to 2 or more first-line anti-seizure drugs.
• Age 0 to 18 years.
• Etiology for status epilepticus unknown:
  • No history of pre-existing epilepsy;
  • No pre-existing major developmental impairment;
  • No evidence of prodromal dyskinesia, hallucinations, or psychiatric symptoms;
  • No evidence of structural, cerebrovascular, neoplastic, metabolic, traumatic, or toxic etiology.

Inclusion Criteria - RISK Primary Inclusion Criteria: DRE (~150 patients):

• Epileptiform abnormality on EEG.
• Seizure frequency at least weekly.
• Failure to respond to 2 or more first-line anti-seizure drugs.
• Age 0 to 18 years.
• Clinical and electrophysiological profile not consistent with:
  • self-limited focal epilepsy syndrome;
  • unifocal epilepsy.
• Normal 3T epilepsy protocol MRI (if available).
• Normal epilepsy panel or no evidence of known epilepsy mutations by whole exome sequencing (if available).
• No evidence of autoimmune, cerebrovascular, neoplastic, metabolic, traumatic, or toxic etiology.

Inclusion Criteria - RISK Retrospective Recruitment Inclusion Criteria (~100 patients):

• Prior diagnosis consistent with NORSE or DRE.
• No evidence of autoimmune, infectious, structural, cerebrovascular, neoplastic, metabolic, or toxic etiology.
• Negative genetic epilepsy panel.
  • Note: No exclusions based on disease-modifying therapies, including immunomodulatory or immunosuppressive treatments (e.g., IV corticosteroids, plasma exchange, intravenous immunoglobulin, and anti-cytokine drugs such as anakinra or tocilizumab).

Exclusion Criteria:

• Validated autoimmune disorder.
• Smoker.
• Pregnant.
• Weight < 9 kg.

Eligibility last updated 1/21/22. Questions regarding updates should be directed to the study team contact.