Globally, the incidence of encephalitis is around 1.5 to 14 per 100,000 population per year.[4]Boucher A, Herrmann JL, Morand P, et al. Epidemiology of infectious encephalitis causes in 2016. Med Mal Infect. 2017 May;47(3):221-35.
http://www.ncbi.nlm.nih.gov/pubmed/28341533?tool=bestpractice.com
[5]Wang H, Zhao S, Wang S, et al. Global magnitude of encephalitis burden and its evolving pattern over the past 30 years. J Infect. 2022 Jun;84(6):777-87.
https://www.journalofinfection.com/article/S0163-4453(22)00211-0/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/35452715?tool=bestpractice.com
Approximately 20,000 cases occur each year in the US.[6]Vora NM, Holman RC, Mehal JM, et al. Burden of encephalitis-associated hospitalizations in the United States, 1998-2010. Neurology. 2014 Feb 4;82(5):443-51.
http://www.ncbi.nlm.nih.gov/pubmed/24384647?tool=bestpractice.com
True incidence is difficult to determine (due to the wide spectrum of clinical presentation, under-diagnosis, and under-reporting), and may be higher than hospital discharge data suggest in England, France, Italy, Canada, and Australia.[7]George BP, Schneider EB, Venkatesan A. Encephalitis hospitalization rates and inpatient mortality in the United States, 2000-2010. PloS One. 2014 Sep 5;9(9):e104169.
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0104169
http://www.ncbi.nlm.nih.gov/pubmed/25192177?tool=bestpractice.com
[8]Barbadoro P, Marigliano A, Ricciardi A, et al. Trend of hospital utilization for encephalitis. Epidemiol Infect. 2012 Apr;140(4):753-64.
http://www.ncbi.nlm.nih.gov/pubmed/21733247?tool=bestpractice.com
[9]Bernard S, Mailles A, Stahl JP, et al. Epidemiology of infectious encephalitis, differences between a prospective study and hospital discharge data. Epidemiol Infect. 2013 Nov;141(11):2256-68.
http://www.ncbi.nlm.nih.gov/pubmed/23168268?tool=bestpractice.com
[10]Huppatz C, Durrheim DN, Levi C, et al. Etiology of encephalitis in Australia, 1990-2007. Emerg Infect Dis. 2009 Sep;15(9):1359-65.
https://wwwnc.cdc.gov/eid/article/15/9/08-1540_article
http://www.ncbi.nlm.nih.gov/pubmed/19788802?tool=bestpractice.com
[11]Kulkarni MA, Lecocq AC, Artsob H, et al. Epidemiology and aetiology of encephalitis in Canada, 1994-2008: a case for undiagnosed arboviral agents? Epidemiol Infect. 2013 Nov;141(11):2243-55.
http://www.ncbi.nlm.nih.gov/pubmed/23148910?tool=bestpractice.com
[12]Granerod J, Cousens S, Davies NW, et al. New estimates of incidence of encephalitis in England. Emerg Infect Dis. 2013;19(9).
https://wwwnc.cdc.gov/eid/article/19/9/13-0064_article
http://www.ncbi.nlm.nih.gov/pubmed/23969035?tool=bestpractice.com
Infectious causes are most frequently identified causes of encephalitis. For infectious etiologies, there is no specific predominance in either sex, but frequently a bimodal age distribution is seen with peaks in the young and the elderly.[13]Solomon T, Michael BD, Smith PE, et al. Management of suspected viral encephalitis in adults--Association of British Neurologists and British Infection Association National Guidelines. J Infect. 2012 Apr;64(4):347-73.
https://www.doi.org/10.1016/j.jinf.2011.11.014
http://www.ncbi.nlm.nih.gov/pubmed/22120595?tool=bestpractice.com
Seasonal and geographic variations occur in some cases of viral encephalitis in the US and other parts of the world. There is increased incidence in summer and early fall (peaking July to October) for enteroviruses and most arboviruses, reflecting seasonal variations in pathogen and/or vector activity. Certain arboviruses show marked geographic variation. In Europe, tick-borne encephalitis (TBE) is increasing due to broadening of endemic areas and prolongation of the tick activity season.[14]Taba P, Schmutzhard E, Forsberg P, et al. EAN consensus review on prevention, diagnosis and management of tick-borne encephalitis. Eur J Neurol. 2017 Oct;24(10):1214-e61.
http://www.ncbi.nlm.nih.gov/pubmed/28762591?tool=bestpractice.com
TBE virus is endemic in rural and forested areas of central, eastern, and northern Europe.[15]UK Government guidance. Tick-borne encephalitis: epidemiology, diagnosis and prevention: Advice for health professionals on tick-borne encephalitis (TBE) including symptoms, diagnosis and epidemiology.
https://www.gov.uk/guidance/tick-borne-encephalitis-epidemiology-diagnosis-and-prevention#epidemiology
In 2021, there were 3027 cases of TBE across 25 EU/EEA countries. The highest number of confirmed cases were seen in Czechia, Sweden, and Germany.[16]European Centre for Disease Prevention and Control. Tick-borne encephalitis - annual epidemiological report for 2019. Apr 2024 [internet publication].
https://www.ecdc.europa.eu/en/publications-data/tick-borne-encephalitis-annual-epidemiological-report-2021
Incidence of encephalitis associated with HIV infections has decreased and stabilized since the late 1990s with the advent of antiretroviral therapies.[6]Vora NM, Holman RC, Mehal JM, et al. Burden of encephalitis-associated hospitalizations in the United States, 1998-2010. Neurology. 2014 Feb 4;82(5):443-51.
http://www.ncbi.nlm.nih.gov/pubmed/24384647?tool=bestpractice.com
In contrast, encephalitis associated with immunocompromised states induced in the setting of transplant or immune-mediated diseases has steadily increased.
Unlike infectious etiologies, there tends to be a female predominance in autoimmune encephalitis, with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis being the most commonly reported type of autoimmune encephalitis. Acute disseminated encephalomyelitis (ADEM) is a rare illness with an incidence of 0.2 to 0.4 per 100,000 children annually.[17]Banwell B, Kennedy J, Sadovnick D, et al. Incidence of acquired demyelination of the CNS in Canadian children. Neurology. 2009 Jan 20;72(3):232-9.
http://www.ncbi.nlm.nih.gov/pubmed/19153370?tool=bestpractice.com
[18]Wang CX. Assessment and management of acute disseminated encephalomyelitis (ADEM) in the pediatric patient. Paediatr Drugs. 2021 May;23(3):213-21.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8026386
http://www.ncbi.nlm.nih.gov/pubmed/33830467?tool=bestpractice.com
[19]Fontenot G, Johnston K, Cohen JC, et al. PCR amplification of HIV-1 proteinase sequences directly from lab isolates allows determination of five conserved domains. Virology. 1992 Sep;190(1):1-10.
http://www.ncbi.nlm.nih.gov/pubmed/1529522?tool=bestpractice.com
The most common age of presentation is between 3 and 7 years.[18]Wang CX. Assessment and management of acute disseminated encephalomyelitis (ADEM) in the pediatric patient. Paediatr Drugs. 2021 May;23(3):213-21.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8026386
http://www.ncbi.nlm.nih.gov/pubmed/33830467?tool=bestpractice.com
[20]Cole J, Evans E, Mwangi M, et al. Acute disseminated encephalomyelitis in children: an updated review based on current diagnostic criteria. Pediatr Neurol. 2019 Nov;100:26-34.
http://www.ncbi.nlm.nih.gov/pubmed/31371120?tool=bestpractice.com