Authors :
Presenting Author: Matthew Gruen, BS – Yale University School of Medicine
Anthony Jimenez, BS – Yale University School of Medicine
Margaret Gopaul, PhD – Yale University School of Medicine
Quyen Cao, BS – Yale University School of Medicine
Ayush Batra, MD – Feinberg School of Medicine, Northwestern University
Leah Blank, MD – Icahn School of Medicine at Mount Sinai
Charlotte Damien, MD – Hôpital Universitaire de Bruxelles
Gregg Day, MD – Mayo Clinic
Krista Eschbach, MD – University of Colorado Anschutz Medical Campus – Children’s Hospital Colorado
Elizabeth Gerard, MD – Feinberg School of Medicine, Northwestern University
Teneille Gofton, MD – London Health Sciences Centre (LHSC)
Stephen Hantus, MD – Cleveland Clinic Epilepsy Center
Nathalie Jette, MD – Icahn School of Medicine at Mount Sinai
Amy Jongeling, MD, PhD – NYU Grossman School of Medicine
Peter Kang, MD – Washington University School of Medicine
Karnig Kazazian, BS – London Health Sciences Centre (LHSC)
Marissa Kellogg, MD – Oregon Health & Science University (OHSU)
Minjee Kim, MD – Feinberg School of Medicine, Northwestern University
Bahar Madani, Student Research Assistant – Oregon Health & Science University (OHSU)
Mikaela Morales, BS – Seattle Children's Hospital.
Vineet Punia, MD – Cleveland Clinic
Claude Steriade, MD – NYU Grossman School of Medicine
Aaron Struck, MD – University of Wisconsin-Madison
Olga Taraschenko, MD, PhD – University of Nebraska Medical Center
Nathan Torcida Sedano, MD – Hôpital Universitaire de Bruxelles
Mark Wainwright, MD, PhD – Seattle Children's Hospital
Jiyeoun (Jenna) Yoo, MD – Icahn School of Medicine at Mount Sinai
Nicolas Gaspard, MD, PhD – Hôpital Universitaire de Bruxelles
Lawrence Hirsch, MD – Yale University School of Medicine
Aurélie Hanin, PharmD, PhD – Yale University School of Medicine
Rationale:
The prognosis of New-onset refractory status epilepticus (NORSE) is often poor, with high prevalence of long-term cognitive deficits, behavioral disturbances, and epilepsy, all of which impair quality of life. Understanding outcomes and their trajectories will guide long-term psychological and social support needs following diagnosis, help plan clinical trials, and help determine if there is a progressive component to the disorder in some patients.
Methods:
34 patients were enrolled by 17 centers in the Yale NORSE Biorepository. The self-reported Neuro-QOL scale was used to assess physical, mental, and social health in adults (13 items, 102 questions) and children aged 7-17 (8 items, 66 questions). The questions were scored from 1 (least impairment) to 5 (most impairment). Quality of life impairment (QOL-I) scores were calculated by summing item scores and dividing by the maximum possible scores. Following NORSE, outcomes were evaluated within 3-6 months for 33 patients (26 adults and 7 children), 12 months for 25 patients (21 adults and 4 children), and 24 months for 19 patients (16 adults and 3 children).
Results: For adults at 3-6 months, the median overall QOL-I score was 45.2%, with the highest impairment in satisfaction with social roles and activities (55.0%) and fatigue (53.2%). The median QOL-I score improved to 41.4% at 12 months. At 24 months, patients continued to improve overall (median QOL-I of 38.7%), and cognitive function became the most impaired outcome (57.5%). Patients with higher QOL-I scores had worse functional outcomes evaluated with the Glasgow Outcome Scale-Extended (GOS-E, rho -0.675, p< 0.001). Patients without post-NORSE-epilepsy, compared to those with epilepsy, had a significantly improved rating for participation in social roles and activities (p=0.037) and a trend towards less fatigue (p=0.073) at 24 months. Children had better outcomes than adults, with a median GOS-E of 7 at 3-6 months. They reported QOL-I scores of 34.5% at 3-6 months, 32.0% at 12 months, and 33.0% at 24 months.