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For further information, see CMDT Part 32-03: Other Neurotropic Viruses

Key Features

Essentials of Diagnosis

  • Acute febrile illness; rash may be present; stiff neck progressing to stupor, coma, and convulsions

  • Upper motor neuron lesion signs: exaggerated deep tendon reflexes, absent superficial reflexes, and spastic paralysis

  • Cerebrospinal fluid opening pressure and protein are often increased with lymphocytic pleocytosis

General Considerations

  • Caused by arthropod-borne viruses

  • The mosquito-borne pathogens include

    • Togaviruses (most of which are of the genus Alphavirus: Western, Eastern, Venezuelan equine encephalitis, chikungunya, and Mayaro virus)

    • Flaviviruses (causing West Nile fever, St. Louis encephalitis, Japanese encephalitis, and Murray Valley encephalitis; dengue, Zika, yellow fever can present with encephalitis)

    • Bunyaviruses (the California serogroup of viruses, including the Jamestown Canyon and the La Crosse viruses)

  • The tick-borne causes of encephalitis include

    • The flavivirus of Powassan encephalitis (North America)

    • Tick-borne encephalitis virus of Europe and Asia

    • The Colorado tick fever reovirus

  • West Nile virus

    • Leading cause of domestically acquired arboviral disease in the United States; most cases are identified in Texas and California

    • In 2019, 971 cases were reported to the CDC from 43 US states and the District of Columbia

    • 65% of cases in 2019 were classified as neuroinvasive disease

    • Outbreaks with West Nile infection tend to occur between mid-July and early September

    • Climatic factors, including elevated mean temperatures and rainfall, correlate with increased West Nile infection

    • Transmission

      • Mosquitos infected by biting infected birds can infect people and other mammals

      • However, the virus cannot be transmitted from infected humans and other mammals to other biting mosquitoes

      • Human-to-human transmission is usually related to blood transfusion and organ transplantation

    • Since 2003, all blood donations in the United States are screened with nucleic acid amplification assays for West Nile virus

  • Eastern equine encephalitis and Powassan encephalitis virus have also been shown to be transmitted by organ transplantation

  • La Crosse virus

    • Average number of neuroinvasive cases reported annually is 63 cases

    • Most cases are reported from Midwestern states

    • Occur from late spring to early fall

    • Less severe forms of California serogroup viruses include Jamestown Canyon virus and California encephalitis virus

    • Risk factors for development of neuroinvasive disease and increased mortality include

      • Black race

      • Diabetes mellitus

      • Chronic kidney disease

      • Hepatitis C virus infection

Clinical Findings

Symptoms and Signs

  • Incubation period is 2–14 days

  • Disease manifestations are strongly age-dependent

    • Acute febrile syndrome and mild neurologic symptoms are more common in the young

    • Aseptic meningitis and poliomyelitis-like syndromes are seen in the middle aged

    • Frank encephalopathy is seen in the elderly

  • The infection is symptomatic in about 20% of cases, and of those, less than 1% progress to neuroinvasive disease including meningitis, encephalitis, and flaccid paralysis

  • Symptoms include acute febrile illness and a nonpruritic maculopapular rash (variably present)

  • Meningitis is indistinguishable from other viral meningitis

  • West Nile virus encephalitis presents with fever ...

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