Mette Scheller Nissen1,2,3, Maren Synnøve Ørvik4, Anna Christine Nilsson5,6, Matias Ryding5,7, Magnus Lydolph8, Morten Blaabjerg9,10,11,12. 1. Department of Neurology, Odense University Hospital, J.B. Winsløws Vej 4, 5000, Odense C, Denmark. Mette.scheller.nissen2@rsyd.dk. 2. Department of Clinical Research, University of Southern Denmark, Odense, Denmark. Mette.scheller.nissen2@rsyd.dk. 3. Brain Research-Inter Disciplinary Guided Excellence (BRIDGE), Odense, Denmark. Mette.scheller.nissen2@rsyd.dk. 4. Department of Neurology, Telemark Hospital, Skien, Norway. 5. Department of Clinical Research, University of Southern Denmark, Odense, Denmark. 6. Department of Clinical Immunology, Odense University Hospital, Odense, Denmark. 7. Brain Research-Inter Disciplinary Guided Excellence (BRIDGE), Odense, Denmark. 8. Danish National Biobank, Statens Serum Institut, Copenhagen, Denmark. 9. Department of Neurology, Odense University Hospital, J.B. Winsløws Vej 4, 5000, Odense C, Denmark. Morten.Blaabjerg1@rsyd.dk. 10. Department of Clinical Research, University of Southern Denmark, Odense, Denmark. Morten.Blaabjerg1@rsyd.dk. 11. Brain Research-Inter Disciplinary Guided Excellence (BRIDGE), Odense, Denmark. Morten.Blaabjerg1@rsyd.dk. 12. Department of Neurobiology Research, Institute of Molecular Medicine, University of Southern Denmark, Odense, Denmark. Morten.Blaabjerg1@rsyd.dk.
Abstract
BACKGROUND: To describe the national Danish N-methyl-D-aspartate receptor encephalitis (NMDARE) cohort. METHODS: All NMDAR immunoglobulin G (IgG) positive cases in Denmark from 2009 to 2019 were included. Medical information was assessed retrospectively for clinical phenotype, workup, treatment and outcome. RESULTS: Seventy-seven patients were NMDAR IgG positive in serum/CSF. Fifty-five fulfilled the criteria of NMDARE, 18 did not and 4 had missing data. Incidence was 0.17/100,000 persons per year in 2018, and incidence rates increased since 2009. Of the 55 NMDARE patients (median age 27; 60% female), 9 had post-herpes simplex (HSE) NMDARE and 7 had a tumor (four teratomas). MRI was normal in 51% of patients. Brain FDG PET was performed in 17 patients, and was abnormal in 47% of patients with a normal MRI. First-line therapy was administered to 91%, and 24% required second-line therapy. Maintenance therapy during recovery was given 84% of patients, with no effect on relapse-risk. ICU admission occurred in 29%. Poor outcome (mRS > 2) was reported in 27% and dependent on age and etiology. Patients > 45 years had a poorer outcome (71% vs 8%, p < 0.0001), more frequently post-HSE NMDARE (47% vs 3%, p < 0.0001) and underlying malignancies (18% vs 0%). CONCLUSION: The incidence of NMDARE in Denmark is currently 0.17/100,000 persons per year, and has increased since 2009. NMDARE patients in Denmark display a higher median age, lower female:male ratio, a less frequent tumor association and need for ICU admission. Maintenance therapy did not reduce relapse rate. Poor outcome was seen with higher age, likely related to underlying etiology.
BACKGROUND: To describe the national Danish N-methyl-D-aspartate receptor encephalitis (NMDARE) cohort. METHODS: All NMDAR immunoglobulin G (IgG) positive cases in Denmark from 2009 to 2019 were included. Medical information was assessed retrospectively for clinical phenotype, workup, treatment and outcome. RESULTS: Seventy-seven patients were NMDAR IgG positive in serum/CSF. Fifty-five fulfilled the criteria of NMDARE, 18 did not and 4 had missing data. Incidence was 0.17/100,000 persons per year in 2018, and incidence rates increased since 2009. Of the 55 NMDARE patients (median age 27; 60% female), 9 had post-herpes simplex (HSE) NMDARE and 7 had a tumor (four teratomas). MRI was normal in 51% of patients. Brain FDG PET was performed in 17 patients, and was abnormal in 47% of patients with a normal MRI. First-line therapy was administered to 91%, and 24% required second-line therapy. Maintenance therapy during recovery was given 84% of patients, with no effect on relapse-risk. ICU admission occurred in 29%. Poor outcome (mRS > 2) was reported in 27% and dependent on age and etiology. Patients > 45 years had a poorer outcome (71% vs 8%, p < 0.0001), more frequently post-HSE NMDARE (47% vs 3%, p < 0.0001) and underlying malignancies (18% vs 0%). CONCLUSION: The incidence of NMDARE in Denmark is currently 0.17/100,000 persons per year, and has increased since 2009. NMDARE patients in Denmark display a higher median age, lower female:male ratio, a less frequent tumor association and need for ICU admission. Maintenance therapy did not reduce relapse rate. Poor outcome was seen with higher age, likely related to underlying etiology.
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