Congratulations to Eric Payne, MD, MPH and Charles Howe, PhD, both from Mayo Clinic (Rochester, MN) who were $100,000 NORSE Institute grant recipients to study NLRP3 inflammasome dysfunction as a cause for FIRES and NORSE. The NORSE Institute partnered with NORD (National Organization of Rare Disorders) who issued the 2018 request for proposals.
A 2019 Request for Proposals for NORSE research has just been issued by NORD. This is for two NORSE Institute grants for either clinical or translational research of NORSE and FIRES for up to a combined total of $100,000 U.S. Download RFP.
A research roadmap for NORSE has been published in Neurology, April 2019.
A prospective, observational study of NORSE patients is being done by participating members of the Critical Care EEG Monitoring Research Consortium (CCEMRC). Clinical data and biological samples (serum, CSF, tissue) are being collected. The study includes patients ages 6 and older. For more information contact: Nicolas Gaspard, MD, Ph.D and Lawrence Hirsch, MD, via Contact page. Families can see a further explanation in the Patients and Families section.
A Family Registry of NORSE patients accepting information from families, patients and physicians outside of the CCEMRC is near launch. Contact: Tenielle Gofton, MD and Nicolas Gaspard, MD, Ph.D via Contact page. Families can see further explanation in the Patients and Families section.
Time to treatment in refractory status epilepticus is vital.
Association of Time to Treatment With Short-term Outcomes for Pediatric Patients With Refractory Convulsive Status Epilepticus. Gaínza-Lein M, Sánchez Fernández I, Loddenkemper T; Pediatric Status Epilepticus Research Group. JAMA Neurol. 2018 Apr 1;75(4):410-418.
The high degree of uncertainty in cause, treatment and outcome, and the extended ICU stay in NORSE necessitates integrated and candid communication among physicians themselves and between physicians and families. The Role of Hope, Compassion, and Uncertainty in Physicians' Reluctance to Initiate Palliative Care. Wong, NW, AMA J Ethics. 2018 Aug 1;20(8):E782-786.
NORSE Diagnostic Checklist
Disclaimer: This is a recommended checklist to investigate NORSE. It does not claim to be a complete list of tests to be done. The NORSE Institute is grateful to its Medical Advisory Board and to Dr Shivani Ghoshal for developing this NORSE checklist. (Last update: 10/31/18.) Constructive comments can be sent via Contact page.
Sample Treatment Algorithm
Disclaimer: This is a sample treatment algorithm for generalized convulsive status epilepticus in adults and children. The NORSE Institute is grateful to Dr Kent Owusu and Yale New Haven Health, Departments of Pharmacy and Neurology for allowing us to post their treatment algorithm. (Last update: 10/31/18.)
Consensus Definitions of NORSE and FIRES
Febrile Infection-related Epilepsy Syndrome (FIRES) is now considered a subcategory of NORSE. See abstract below: