The Frequency of autoimmune N-Methyl-D-Aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project
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BACKGROUND: In 2007, the California Encephalitis Project (CEP), which was established to study the epidemiology of encephalitis, began identifying cases of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Increasing numbers of anti-NMDAR encephalitis cases have been identified at the CEP, and this form rivals commonly known viral etiologies as a causal agent. We report here the relative frequency and differences among encephalitides caused by anti-NMDAR and viral etiologies within the CEP experience. METHODS: Demographic, frequency, and clinical data from patients with anti-NMDAR encephalitis are compared with those with viral encephalitic agents: enterovirus, herpes simplex virus type 1 (HSV-1), varicella-zoster virus (VZV), and West Nile virus (WNV). All examined cases presented to the CEP between September 2007 and February 2011 and are limited to individuals aged ≤30 years because of the predominance of anti-NMDAR encephalitis in this group. The diagnostic costs incurred in a single case are also included. RESULTS: Anti-NMDAR encephalitis was identified >4 times as frequently as HSV-1, WNV, or VZV and was the leading entity identified in our cohort. We found that 65% of anti-NMDAR encephalitis occurred in patients aged ≤18 years. This disorder demonstrated a predilection, which was not observed with viral etiologies, for females (P < .01). Seizures, language dysfunction, psychosis, and electroencephalographic abnormalities were significantly more frequent in patients with anti-NMDAR encephalitis (P < .05), and autonomic instability occurred exclusively in this group. DISCUSSION: Anti-NMDAR encephalitis rivals viral etiologies as a cause of encephalitis within the CEP cohort. This entity deserves a prominent place on the encephalitic differential diagnosis to avoid unnecessary diagnostic and treatment costs, and to permit a more timely treatment.
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In a center focused on the study of encephalitis of unclear etiology, the frequency of anti-NMDAR encephalitis surpassed that of any specific viral etiologies in young individuals.
“In a center focused in the study of encephalitis of unclear etiology the frequency of anti-NMDAR encephalitis surpassed that of any specific viral etiologies in young individuals.6”
From Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study (2013) · cites this paper as [6]
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- Authors
- Gable, M. S., Sheriff, H., Dalmau, J., Tilley, D. H., & Glaser, C. A.
- Affiliations
- California Department of Public Health (US)
- Institució Catalana de Recerca i Estudis Avançats (ES)
- United States Naval Medical Research Unit SOUTH (PE)
- Venue
- Clinical Infectious Diseases
- Publisher
- Oxford University Press (OUP)
- Published
- 2012-01-26
- Volume
- 54
- Issue
- 7
- Pages
- 899-904
- ISSN
- 1058-4838 (ISSN-L)
- 1537-6591
- PMC
- PMC3297648
- OpenAlex
- W2097556759
- Cited by
- 733
- References
- 2
- Citations by year
- 2026: 24 · 2025: 34 · 2024: 43 · 2023: 42 · 2022: 65 · 2021: 58 · 2020: 65 · 2019: 57 · 2018: 61 · 2017: 59 · 2016: 61 · 2015: 57 · 2014: 46 · 2013: 43 · 2012: 18
- FWCI
- 32.29
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Clinical Infectious Diseases (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- McKnight Foundation
- National Institutes of Health (2RO1CA89054-06A2)
- Centers for Disease Control and Prevention (U50/CCU915546-09)
- U.S. Public Health Service (U50/CCU915546-09)
- National Institute of Mental Health (R01MH094741)
- National Cancer Institute (R01CA089054, 2R01CA89054-06A2)
- National Institute of Neurological Disorders and Stroke (R01NS077851)
- Topics
- Health Sciences › Medicine › Neurology › Autoimmune Neurological Disorders and Treatments
- Health Sciences › Medicine › Infectious Diseases › Infectious Encephalopathies and Encephalitis
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Viral Infections and Immunology Research
- Keywords
- anti-NMDA receptor encephalitis, autoimmune encephalitis, viral encephalitis, herpes simplex virus type 1, varicella-zoster virus, West Nile virus, enterovirus, young adults, autonomic dysfunction, seizures, psychosis, differential diagnosis, diagnostic costs
- MeSH terms
- Adolescent, Adult, Autoimmune Diseases, California, Child, Child, Preschool, Encephalitis, Female, Humans, Infant, Infant, Newborn, Male, Virus Diseases, Prevalence, Receptors, N-Methyl-D-Aspartate, Health Care Costs, Age Distribution, Clinical Laboratory Techniques, Young Adult
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