California Encephalitis 

  • Author: Wayne E Anderson, DO; Chief Editor: Burke A Cunha, MD   more...
 
Updated: Jun 17, 2011
 

Background

California encephalitis is an arbovirus-induced, arthropod-borne encephalitis or encephalomeningitis. The virus is transmitted to humans through a mosquito bite.[1] (See Etiology.)

The condition was named California encephalitis after the first human case (caused by a virus called California virus) was described in Kern County, California, in 1946. Since then, most cases have been associated with La Crosse virus. La Crosse virus was first isolated from the brain of a 4-year-old boy who died of encephalitis in La Crosse County, Wisconsin.

Most patients with clinical symptoms recover completely; however, 20% of patients develop behavioral problems or recurrent seizures. Mortality rates are low (< 1%).

Patient education

For patient education information, see the Brain and Nervous System Center, as well as Encephalitis.

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Etiology

La Crosse virus, one of the bunyaviruses (ie, negative-polarity, single-stranded ribonucleic acid [RNA] viruses with a helical and enveloped nucleocapsid), causes California encephalitis. La Crosse virus is the most common cause of arboviral-induced pediatric encephalitis in the United States.

The Aedes triseriatus mosquito (forest-dwelling tree-hole mosquito) transmits La Crosse virus. Alternating cycles of infection occur between the mosquito and the vertebrate hosts, including humans. The mosquitoes obtain the virus after a blood meal from hosts who are in the viremia stage. The transmission cycle for La Crosse virus is demonstrated in the diagram below.

La Crosse virus transmission cycle. The virus is mLa Crosse virus transmission cycle. The virus is maintained by vertical transmission in Aedes triseriatus mosquitoes; the virus winters in infected eggs that are usually deposited in tree holes or in artificial containers holding rainwater. Horizontal transmission (by viral amplification in small vertebrates, eg, squirrels and chipmunks, and venereally among adult mosquitoes) is required to supplement vertical transmission. The role of deer in viral amplification is uncertain. Human infections are incidental to the transmission cycle.

After inoculation via a mosquito bite, the virus undergoes a local replication at the original skin site. A primary viremia occurs, with seeding of the reticuloendothelial system, mainly the liver, spleen, and lymph nodes.

With continued virus replication, a secondary viremia occurs, with seeding of the central nervous system (CNS). The probability of CNS infection depends on the efficiency of viral replication at the extraneural sites and the degree of viremia. The virus invades the CNS through either the cerebral capillary endothelial cells or the choroid plexus. Rarely, the virus is isolated from brain tissue.

Antibodies against the G1 part of the virus neutralize the virus, block fusion, and inhibit hemagglutination. They are also important in virus clearance and recovery and in prevention of reinfection.

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Epidemiology

Several epidemiologic factors influence arboviral encephalitis, including (1) the season, (2) the geographic location, (3) the regional climate conditions (eg, spring rainfall), and (4) patient age.

The highest incidence of arboviral encephalitis in the United States is in the midwestern states. Most cases occur in the late summer to early fall. The incidence is approximately 75 cases per year. Outdoor activities, especially in woodland areas, are associated with an increased risk of infection.

La Crosse encephalitis has been reported in 28 states; in areas where the disease is endemic, the incidence exceeds that of bacterial meningitis before the introduction of the Haemophilus influenzae vaccine. La Crosse encephalitis may be underrecognized, not only in terms its prevalence but also in terms of severity.

California encephalitis is more common in males than in females, probably because of more outdoor exposure. Clinical disease occurs almost exclusively in children aged 6 months to 16 years (peak, 4-10 y). The older the patient, the less likely he or she is to develop the clinical illness.

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Contributor Information and Disclosures
Author

Wayne E Anderson, DO  Assistant Professor of Internal Medicine/Neurology, College of Osteopathic Medicine of the Pacific Western University of Health Sciences; Clinical Faculty in Family Medicine, Touro University College of Osteopathic Medicine; Clinical Instructor, Departments of Neurology and Pain Management, California Pacific Medical Center

Wayne E Anderson, DO is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Law, Medicine & Ethics, California Medical Association, and San Francisco Medical Society

Disclosure: Cephalon Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; King Honoraria Speaking and teaching; Forest Honoraria Speaking and teaching

Coauthor(s)

Emad Soliman, MD, MSc  Consulting Staff, Department of Neurology, St John's Riverside Hospital

Emad Soliman, MD, MSc is a member of the following medical societies: American Academy of Neurology and American Medical Association

Disclosure: Nothing to disclose.

Norvin Perez, MD  Medical Director, Juneau Urgent and Family Care

Norvin Perez, MD is a member of the following medical societies: American College of Emergency Physicians and American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary D Nettleman, MD, MS, MACP  Professor and Chair, Department of Medicine, Michigan State University College of Human Medicine

Mary D Nettleman, MD, MS, MACP is a member of the following medical societies: American College of Physicians, Association of Professors of Medicine, Central Society for Clinical Research, Infectious Diseases Society of America, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Joseph F John Jr, MD, FACP, FIDSA, FSHEA  Clinical Professor of Medicine, Molecular Genetics and Microbiology, Medical University of South Carolina College of Medicine; Associate Chief of Staff for Education, Ralph H Johnson Veterans Affairs Medical Center

Disclosure: Nothing to disclose.

Chief Editor

Burke A Cunha, MD  Professor of Medicine, State University of New York School of Medicine at Stony Brook; Chief, Infectious Disease Division, Winthrop-University Hospital

Burke A Cunha, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and Infectious Diseases Society of America

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors gratefully acknowledge the contributions of previous authors Eleftherios Mylonakis, MD, and Eduardo Gotuzzo, MD, to the development and writing of the source article.

References
  1. Halperin JJ, ed. Encephalitis: Diagnosis and Treatment. New York, NY: Informa Healthcare; 2008.

  2. Sokol DK, Kleiman MB, Garg BP. LaCrosse viral encephalitis mimics herpes simplex viral encephalitis. Pediatr Neurol. Nov 2001;25(5):413-5. [Medline].

  3. Wurtz R, Paleologos N. La Crosse encephalitis presenting like herpes simplex encephalitis in an immunocompromised adult. Clin Infect Dis. Oct 2000;31(4):1113-4. [Medline].

  4. de los Reyes EC, McJunkin JE, Glauser TA, Tomsho M, O'Neal J. Periodic lateralized epileptiform discharges in La Crosse encephalitis, a worrisome subgroup: clinical presentation, electroencephalogram (EEG) patterns, and long-term neurologic outcome. J Child Neurol. Feb 2008;23(2):167-72. [Medline].

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La Crosse virus transmission cycle. The virus is maintained by vertical transmission in Aedes triseriatus mosquitoes; the virus winters in infected eggs that are usually deposited in tree holes or in artificial containers holding rainwater. Horizontal transmission (by viral amplification in small vertebrates, eg, squirrels and chipmunks, and venereally among adult mosquitoes) is required to supplement vertical transmission. The role of deer in viral amplification is uncertain. Human infections are incidental to the transmission cycle.
Brain biopsy specimen from a 7-year-old boy with severe La Crosse encephalitis (hematoxylin and eosin stain, 200X). Perivascular infiltration with mononuclear cells is present on light microscopy. This biopsy material tested positively for La Crosse virus antigen on direct immunofluorescence assay.
Left image of a CT scan of an 8-year-old boy with severe La Crosse encephalitis complicated by uncal herniation (obtained on the second hospital day) reveals brain edema with associated obliteration of perimesencephalic cisterns (arrows). On the right, a T2-weighted magnetic resonance image obtained from a 7-year-old boy with severe La Crosse encephalitis shows focal areas of increased signal intensity in the right temporoparietal and left frontotemporal regions (arrows).
 
 
 
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