Journal
LANCET NEUROLOGY
Volume 12, Issue 2, Pages 157-165Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/S1474-4422(12)70310-1
Keywords
-
Categories
Funding
- The Dutch Cancer Society
- National Institutes of Health
- McKnight Neuroscience of Brain Disorders award
- The Fondo de Investigaciones Sanitarias
- Fundacio la Marato de TV3
- KWF fellowship of the Dutch Cancer Society [2009-4451]
- NIH [RO1NS077851, RO1CA89054, RC1NS068204]
- Fondo de Investigaciones Sanitarias (FIS, Spain) [11/01780, PS09/0193, 08/00285]
- National Institute on Aging [P50AG08702]
- Alzheimer's Association
- Alzheimer's Disease Drug Discovery Foundation
- Taub Institute
- Athena Diagnostics
- Euroimmun
- ICREA Funding Source: Custom
- Grants-in-Aid for Scientific Research [23591236] Funding Source: KAKEN
Ask authors/readers for more resources
Background Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune disorder in which the use of immunotherapy and the long-term outcome have not been defined. We aimed to assess the presentation of the disease, the spectrum of symptoms, immunotherapies used, timing of improvement, and long-term outcome. Methods In this multi-institutional observational study, we tested for the presence of NMDAR antibodies in serum or C SF samples of patients with encephalitis between Jan 1,2007, and Jan 1, 2012. All patients who tested positive for NMDAR antibodies were included in the study; patients were assessed at symptom onset and at months 4,8,12,18, and 24, by use of the modified Rankin scale (mRS). Treatment included first-line immunotherapy (steroids, intravenous immunoglobulin, plasmapheresis), second-line immunotherapy (ritincimab, cydophosphamide), and tumour removal. Predictors of outcome were determined at the Universities of Pennsylvania (PA, USA) and Barcelona (Spain) by use of a generalised linear mixed model with binary distribution. Results We enrolled 577 patients (median age 21 years, range 8 months to 85 years), 211 of whom were children (<18 years). Treatment effects and outcome were assessable in 501 (median follow-up 24 months, range 4-186): 472 (94%) underwent first-line immunotherapy or tumour removal, resulting in improvement within 4 weeks in 251 (53%). Of 221 patients who did not improve with first-line treatment, 125 (57%) received second-line immunotherapy that resulted in a better outcome (mRS 0-2) than those who did not (odds ratio [OR] 2.69, CI 1-24-5.80; p=0.012). During the first 24 months, 394 of 501 patients achieved a good outcome (mRS 0-2; median 6 months, IQR 2-12) and 30 died. At 24 months' follow-up, 203 (81%) of 252 patients had good outcome. Outcomes continued to improve for up to 18 months after symptom onset. Predictors of good outcome were early treatment (0.62,0.50-0.76; p<0.0001) and no admission to an intensive care unit (0.12,0.06-0-22; p<0.0001). 45 patients had one or multiple relapses (representing a 12% risk within 2 years); 46 (67%) of 69 relapses were less severe than initial episodes (p<0.0001). In 177 children, predictors of good outcome and the magnitude of effect of second-line immunotherapy were similar to those of the entire cohort. Interpretation Most patients with anti-NMDAR encephalitis respond to immunotherapy. Second-line immunotherapy is usually effective when first-line treatments fail. In this cohort, the recovery of some patients took up to 18 months.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available