TY - JOUR
T1 - NMDA-receptor encephalitis in Denmark from 2009 to 2019
T2 - a national cohort study
AU - Nissen, Mette Scheller
AU - Ørvik, Maren Synnøve
AU - Nilsson, Anna Christine
AU - Ryding, Matias
AU - Lydolph, Magnus
AU - Blaabjerg, Morten
N1 - Funding Information:
This research was conducted using the Danish National Biobank resource, supported by the Novo Nordisk Foundation. Authors acknowledge the Autoimmune Laboratory at the Dept. of Clinical Immunology, Odense University Hospital and the Autoimmune Laboratory at Statens Serum Institut, Denmark. In addition, all the involved hospitals who kindly provided clinical information on patients.
Funding Information:
MSN received grant support by Odense University Hospital and the Faculty of Health Sciences, University of Southern Denmark. No award/grant number.
PY - 2022/3
Y1 - 2022/3
N2 - 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.
AB - 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.
KW - Anti-N-methyl-d-aspartate receptor encephalitis
KW - Autoimmune encephalitis
KW - Cohort studies
KW - NMDA-receptor
KW - Post-herpes simplex virus 1 encephalitis
KW - Humans
KW - Neoplasm Recurrence, Local
KW - Male
KW - Receptors, N-Methyl-D-Aspartate
KW - Anti-N-Methyl-D-Aspartate Receptor Encephalitis/therapy
KW - Denmark/epidemiology
KW - Adult
KW - Female
KW - N-Methylaspartate
KW - Retrospective Studies
KW - Cohort Studies
U2 - 10.1007/s00415-021-10738-9
DO - 10.1007/s00415-021-10738-9
M3 - Journal article
C2 - 34351472
AN - SCOPUS:85111850060
SN - 0340-5354
VL - 269
SP - 1618
EP - 1630
JO - Journal of Neurology
JF - Journal of Neurology
IS - 3
ER -