Maarten J Titulaer1, Lindsey McCracken, Iñigo Gabilondo, Takahiro Iizuka, Izumi Kawachi, L Bataller, A Torrents, Myrna R Rosenfeld, Rita Balice-Gordon, Francesc Graus, Josep Dalmau. 1. From the Department of Neurology (M.J.T., I.G., M.R.R., F.G., J.D.), Hospital Clinic, Universitat de Barcelona/Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Spain; Department of Neurology and Neurosciences (M.J.T., L.M., M.R.R., R.B.-G., J.D.), Perelman School of Medicine, Hospital of the University of Pennsylvania, Philadelphia; Department of Neurology (T.I.), Kitasato University School of Medicine, Sagamihara; Department of Neurology (I.K.), Brain Research Institute, Niigata University, Japan; Department of Neurology (L.B.), Hospital Universitario La Fe, Valencia; Biostatistics and Data Management Platform (A.T.), IDIBAPS, Hospital Clinic; and Institució Catalana de Recerca i Estudis Avançats (ICREA) (J.D.), Barcelona, Spain.
Abstract
OBJECTIVE: To describe the clinical features and outcome of anti-NMDA receptor (NMDAR) encephalitis in patients ≥45 years old. METHOD: Observational cohort study. RESULTS: In a cohort of 661 patients with anti-NMDAR encephalitis, we identified 31 patients ≥45 years old. Compared with younger adults (18-44 years), older patients were more often male (45% vs. 12%, p < 0.0001), had lower frequency of tumors (23% vs. 51%, p = 0.002; rarely teratomas), had longer median time to diagnosis (8 vs 4 weeks, p = 0.009) and treatment (7 vs. 4 weeks, p = 0.039), and had less favorable outcome (modified Rankin Scale score 0-2 at 2 years, 60% vs. 80%, p < 0.026). In multivariable analysis, younger age (odds ratio [OR] 0.15, confidence interval [CI] 0.05-0.39, p = 0.0001), early treatment (OR 0.60, CI 0.47-0.78, p < 0.0001), no need for intensive care (OR 0.09, CI 0.04-0.22, p < 0.0001), and longer follow-up (p < 0.0001) were associated with good outcome. Rituximab and cyclophosphamide were effective when first-line immunotherapies failed (OR 2.93, CI 1.10-7.76, p = 0.031). Overall, 60% of patients older than 45 years had full or substantial recovery at 24 months follow-up. CONCLUSIONS: Anti-NMDAR encephalitis is less severe in patients ≥45 years old than in young adults, but the outcome is poorer in older patients. In this age group, delays in diagnosis and treatment are more frequent than in younger patients. The frequency of underlying tumors is low, but if present they are usually carcinomas instead of teratomas in younger patients. Early and aggressive immunotherapy will likely improve the clinical outcome.
OBJECTIVE: To describe the clinical features and outcome of anti-NMDA receptor (NMDAR) encephalitis in patients ≥45 years old. METHOD: Observational cohort study. RESULTS: In a cohort of 661 patients with anti-NMDAR encephalitis, we identified 31 patients ≥45 years old. Compared with younger adults (18-44 years), older patients were more often male (45% vs. 12%, p < 0.0001), had lower frequency of tumors (23% vs. 51%, p = 0.002; rarely teratomas), had longer median time to diagnosis (8 vs 4 weeks, p = 0.009) and treatment (7 vs. 4 weeks, p = 0.039), and had less favorable outcome (modified Rankin Scale score 0-2 at 2 years, 60% vs. 80%, p < 0.026). In multivariable analysis, younger age (odds ratio [OR] 0.15, confidence interval [CI] 0.05-0.39, p = 0.0001), early treatment (OR 0.60, CI 0.47-0.78, p < 0.0001), no need for intensive care (OR 0.09, CI 0.04-0.22, p < 0.0001), and longer follow-up (p < 0.0001) were associated with good outcome. Rituximab and cyclophosphamide were effective when first-line immunotherapies failed (OR 2.93, CI 1.10-7.76, p = 0.031). Overall, 60% of patients older than 45 years had full or substantial recovery at 24 months follow-up. CONCLUSIONS: Anti-NMDAR encephalitis is less severe in patients ≥45 years old than in young adults, but the outcome is poorer in older patients. In this age group, delays in diagnosis and treatment are more frequent than in younger patients. The frequency of underlying tumors is low, but if present they are usually carcinomas instead of teratomas in younger patients. Early and aggressive immunotherapy will likely improve the clinical outcome.
Authors: M J Titulaer; R Soffietti; J Dalmau; N E Gilhus; B Giometto; F Graus; W Grisold; J Honnorat; P A E Sillevis Smitt; R Tanasescu; C A Vedeler; R Voltz; J J G M Verschuuren Journal: Eur J Neurol Date: 2010-09-29 Impact factor: 6.089
Authors: Josep Dalmau; Amy J Gleichman; Ethan G Hughes; Jeffrey E Rossi; Xiaoyu Peng; Meizan Lai; Scott K Dessain; Myrna R Rosenfeld; Rita Balice-Gordon; David R Lynch Journal: Lancet Neurol Date: 2008-10-11 Impact factor: 44.182
Authors: Sarosh R Irani; Katarzyna Bera; Patrick Waters; Luigi Zuliani; Susan Maxwell; Michael S Zandi; Manuel A Friese; Ian Galea; Dimitri M Kullmann; David Beeson; Bethan Lang; Christian G Bien; Angela Vincent Journal: Brain Date: 2010-06 Impact factor: 13.501
Authors: Thaís Armangue; Marianna Spatola; Alexandru Vlagea; Simone Mattozzi; Marc Cárceles-Cordon; Eloy Martinez-Heras; Sara Llufriu; Jordi Muchart; María Elena Erro; Laura Abraira; German Moris; Luis Monros-Giménez; Íñigo Corral-Corral; Carmen Montejo; Manuel Toledo; Luis Bataller; Gabriela Secondi; Helena Ariño; Eugenia Martínez-Hernández; Manel Juan; Maria Angeles Marcos; Laia Alsina; Albert Saiz; Myrna R Rosenfeld; Francesc Graus; Josep Dalmau Journal: Lancet Neurol Date: 2018-07-23 Impact factor: 44.182