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Encephalitis in-ˌsef-ə-ˈlīt-əs

By John E. Greenlee, MD, Professor and Executive Vice Chair, Department of Neurology, University of Utah School of Medicine

Encephalitis is inflammation of the brain that occurs when a virus directly infects the brain or when a virus, vaccine, or something else triggers inflammation. The spinal cord may also be involved, resulting in a disorder called encephalomyelitis.

  • People may have a fever, headache, or seizures, and they may feel sleepy, numb, or confused.

  • Magnetic resonance imaging of the head and a spinal tap are usually done.

  • Treatment involves relieving symptoms and sometimes using antiviral drugs.

Encephalitis is most commonly due to viruses, such as herpes simplex, herpes zoster, cytomegalovirus, or West Nile virus. It can occur in the following ways:

  • A virus directly infects the brain.

  • A virus that caused an infection in the past becomes reactivated and directly damages the brain.

  • A virus or vaccine triggers a reaction that makes the immune system attack brain tissue (an autoimmune reaction).

Sometimes bacteria cause encephalitis, usually as part of bacterial meningitis (called meningoencephalitis).

Protozoa—such as amebas, the protozoa that cause toxoplasmosis (in people who have AIDS), and those that causes malaria—can also infect the brain and cause encephalitis.

Sometimes a brain infection, a vaccine, cancer, or another disorder triggers a misguided immune reaction, causing the immune system to attack normal cells in the brain (an autoimmune reaction). As a result, the brain becomes inflamed. This disorder is called postinfectious encephalitis.

Rarely, encephalitis develops in people who have cancer—a disorder called paraneoplastic encephalitis. This type of encephalitis appears to result from the immune system's response to the tumor.

Types of Encephalitis

Infections that can directly lead to encephalitis can occur in epidemics or occasionally as isolated cases (sporadically).

Epidemic encephalitis

In the United States, the most common types of epidemic encephalitis are caused by one of the following:

Arboviruses are viruses transmitted to people through the bites of arthropods, usually mosquitoes, fleas, or ticks. (Arbovirus is short for arthropod-borne virus.) The viruses are transmitted to arthropods when arthropods bite infected animals or people. Many species of domestic animals and birds carry these viruses.

Epidemics occur in people only periodically—when the population of mosquitoes or infected animals increases. Epidemics tend to occur when arthropods are biting—for mosquitoes and ticks, usually during warm weather. Infection spreads from arthropod to person, not from person to person.

Many arboviruses can cause encephalitis. The different types of encephalitis that result are usually named for the place the virus was discovered or the animal species that typically carries it.

In the United States, mosquitoes spread several types of encephalitis, including the following:

  • La Crosse encephalitis is caused by the La Crosse virus (also called California virus). It is most common in the Midwest but can occur anywhere in the country. This encephalitis accounts for most cases in children. Many cases are mild and undiagnosed. Fewer than 1% of infected people die from it.

  • Eastern equine encephalitis occurs predominantly in the eastern United States. It affects mainly young children and people older than 55. In children younger than 1 year, it can cause severe symptoms and permanent nerve or brain damage. Over half of infected people die.

  • West Nile encephalitis, once present only in Europe and Africa, first appeared in the New York City area in 1999. It has spread throughout the United States. Several species of birds can be infected with the virus when they are bitten by an infected mosquito. This encephalitis affects mainly older people. This virus also causes a milder infection called West Nile fever, which is much more common. West Nile encephalitis develops in fewer than 1% of people who develop West Nile fever. About 10% of people with West Nile encephalitis die. However, those who have only West Nile fever usually recover fully.

  • St. Louis encephalitis occurs throughout the United States but particularly in the Southeast (including Florida), Texas, and some Midwestern states. Epidemics once occurred about every 10 years but are now rare.

  • Western equine encephalitis can occur throughout the United States but, for unknown reasons, has largely disappeared since 1988. It can affect all age groups but mainly children younger than 1 year.

There are few types on encephalitis spread by ticks. These include

  • Tick-borne encephalitis occurs in Northern Asia, Russia, and Europe. The infection usually causes a mild flu-like illness that clears up within a few days, but some people develop more severe symptoms. Because many cases occur in Europe and Russia, a vaccine is available there.

  • Powassan virus infection occurs mainly in Canada and in the North-Central and Northeastern United states. Powassan virus has also been associated with cases of encephalitis in Russia. The virus is similar to the one that causes tick-borne encephalitis in Europe. Infection usually causes mild illness, but a severe and sometimes fatal encephalitis may occur. Powassan virus is spread by the deer tick, which also transmits Lyme disease. The vaccine that is effective against tick-borne encephalitis in Europe and Russia is not effective against this virus.

  • Colorado tick fever occurs in areas of western United States and Canada that are 4,000 to 10,000 feet above sea level. Colorado tick fever causes a flu-like illness. Occasionally, people with Colorado tick fever develop meningitis or encephalitis. It is rarely transmitted by blood transfusion.

  • Bourbon virus infection is very rare. It was reported in only a single person in Bourbon County, Kansas.

  • Heartland virus, which has been identified in Missouri and Tennessee, causes a flu-like illness.

Several viruses that cause encephalitis were once present in only a few parts of the world but now are spreading, probably because travel has increased. These viruses include

  • Chikungunya virus

  • Japanese encephalitis virus

  • Venezuelan equine encephalitis

All are spread by mosquitoes.

The Chikungunya virus was first identified in Africa but has spread to Southeast Asia, India, China, some parts of Europe, the Caribbean and Central, South, and North America. Most people with chikungunya disease feel better within a week. However, chikungunya disease can lead to severe encephalitis and even death, especially in infants and people over age 65.

The Japanese encephalitis virus is a common cause of encephalitis in Asia. The virus is not common in the United States.

Venezuelan equine encephalitis occurs mainly in parts in South and Central America. The Venezuelan equine encephalitis virus caused an epidemic of encephalitis in Texas in 1971 but now rarely causes encephalitis in the United States.

In the United States, Japanese encephalitis and Venezuelan equine encephalitis occur mainly in travelers returning from areas where the viruses that cause these infections are common.

The Zika virus was first identified in the Zika Forest of Uganda, then spread to the South Pacific islands, then South America, Central America, the Caribbean, Mexico, and Florida. Zika infection may cause fever, joint and muscle aches, headache, and a red, bumpy rash. Having Zika virus infection during pregnancy can cause microcephaly and severe brain damage in the baby.

Powassan virus is related to West Nile, St. Louis encephalitis, and tick-borne encephalitis viruses. Powassan virus infections have been recognized in Canada and Russia (southeastern Siberia, northeast of Vladivostok). In the United States, Powassan disease has been reported primarily from northeastern states and the Great Lakes region.

There are two types of Powassan virus in the United States, and both can cause disease in humans: The first type appears to be associated with Ixodes cookei or Ixodes marxi ticks. The other type is sometimes called deer tick virus because it is spread by the same tick that spreads Lyme disease. However, unlike in Lyme disease, in which the tick must be attached for 24 to 48 hours to spread disease, Powassan virus can be spread more quickly. Disease may be transmitted if an infected tick is attached for as little as 15 minutes.

Although rare, Powassan virus encephalitis appears to be increasing since 2006. About 75 cases of Powassan disease have been reported in the United States over the past 10 years. Cases occur in the late spring to mid-fall when ticks are most active.

People infected with Powassan virus cases may have nerve damage after recovery. And Powassan virus is more likely to cause death than other tick-borne encephalitis viruses.

There is no vaccine for Powassan virus.

Sporadic encephalitis

In the United States, sporadic encephalitis is usually caused by herpes simplex virus type 1. Herpes simplex virus causes up to one third of cases of encephalitis. This encephalitis occurs at any time of the year and is fatal if not treated.

Rabies is a significant cause of encephalitis in developing countries and still causes a few cases of encephalitis in the United States.

Human immunodeficiency virus (HIV) causes a slowly developing brain infection, resulting in HIV-associated encephalopathy (also called HIV-associated or AIDS dementia).

Reactivation of a previous infection

Encephalitis can result from reactivation of a virus, including

Reactivation can occur long after people have the infection. A reactivated infection can severely damage the brain.

Autoimmune encephalitis

After certain viral infections or vaccines, the body’s immune system sometimes attacks the layers of tissue that wrap around nerve fibers (called the myelin sheath) in the brain and spinal cord. The attack occurs because proteins in myelin resemble those in the virus. As a result, nerve transmission becomes very slow. The resulting disorder, called acute disseminated encephalomyelitis, resembles multiple sclerosis except that symptoms do not come and go as they do in multiple sclerosis. The viruses most often involved include enteroviruses, Epstein-Barr virus, hepatitis A or B virus, human immunodeficiency virus (HIV), and influenza viruses. Before vaccination of children became widespread, the viruses that cause measles, rubella, chickenpox, and mumps used to be common causes of acute disseminated encephalomyelitis.

An autoimmune encephalitis may also develop if the immune system produces antibodies that attack proteins on the surface of nerve cells called N-methyl-d-aspartate (NMDA) receptors. The resulting encephalitis is called anti-NMDA receptor encephalitis. Some evidence suggests that anti-NMDA receptor encephalitis is a more common type of encephalitis than was previously thought. It occasionally develops after encephalitis due to the herpes simplex virus, even when that encephalitis was successfully treated.

Symptoms of Encephalitis

Before symptoms of encephalitis start, people may have digestive symptoms, such as nausea, vomiting, diarrhea, or abdominal pain. Or they may feel as if they are getting a cold or the flu and have cough, fever, a sore throat, a runny nose, swollen lymph nodes, and muscle aches.

Symptoms of encephalitis include

  • Fever

  • Headache

  • Personality changes or confusion

  • Seizures

  • Paralysis or numbness

  • Sleepiness that can progress to coma and death

People may vomit and have a stiff neck, but these symptoms tend to be less common and less severe than when caused by meningitis.

Encephalitis due to the herpes simplex virus causes headache, fever, and flu-like symptoms at first. People also have seizures, sometimes accompanied by strange smells, vivid flashbacks, or sudden, intense emotions. As the encephalitis progresses, people become confused, have difficulty speaking and remembering, have repeated seizures, then lapse into coma.

HIV-associated encephalopathy can cause gradual personality changes, problems with coordination, and dementia.

If the spinal cord is affected, parts of the body may feel numb and weak. Which parts are affected depend on which parts of the spinal cord are affected (see Figure: Where Is the Spinal Cord Damaged?). People may have difficulty controlling bladder and bowel function. If the infection is severe, people may lose sensation, become paralyzed, and lose control of the bladder and bowels.

Recovery from viral encephalitis may take a long time. Some people do not completely recover. The likelihood of dying depends on what the cause is and how quickly the infection is treated.

Did You Know...

  • Long after a case of measles or chickenpox, the virus can be reactivated and cause inflammation in the brain.

Diagnosis of Encephalitis

  • Magnetic resonance imaging

  • A spinal tap

Doctors suspect encephalitis based on symptoms, especially if an epidemic is in progress. Magnetic resonance imaging (MRI) and a spinal tap are usually done.

MRI can detect typical abnormalities in the brain, confirming encephalitis. If MRI is not available, computed tomography (CT) may be done. It can help doctors exclude disorders that can cause similar symptoms (such as stroke and brain tumor) and check for problems that can make doing a spinal tap dangerous.

A spinal tap (lumbar puncture) is done to obtain a sample of cerebrospinal fluid, which flows through the tissues (meninges) that cover the brain and spinal cord. Normally, the spinal fluid contains very few white blood cells. But when the brain and meninges are inflamed, the number of white cells in cerebrospinal fluid increases. To identify the virus causing encephalitis, doctors take samples of blood and cerebrospinal fluid and test them for antibodies to the virus when the person is sick and later when the person is recovering. Sometimes techniques are used to grow (culture) viruses in the cerebrospinal fluid so that they can be identified more easily. Some enteroviruses (such as those that can cause polio-like illnesses) can be cultured, but most other viruses cannot.

The polymerase chain reaction (PCR) technique is used to identify many of the viruses that can cause encephalitis. PCR, which produces many copies of a gene, is used to detect the genetic material of these viruses in a sample of cerebrospinal fluid. Prompt identification of the herpes simplex virus by PCR is difficult, so treatment is usually started immediately if the herpes simplex virus is thought to be the cause. Prompt treatment is essential because the encephalitis it causes is destructive and, if untreated, is often fatal. Immediate treatment can help lessen the severity of symptoms and prevent death.

Rarely, a sample of brain tissue is removed and examined under a microscope (biopsy) to determine whether herpes simplex virus or another organism is the cause.

Sometimes, even after extensive testing, no virus, bacteria, or other cause of infection is identified. In such cases, the cause may be autoimmune or paraneoplastic encephalitis because tests cannot always confirm those disorders.

Treatment of Encephalitis

  • Depending on the possible cause, an antiviral drug, an antibiotic, corticosteroids, and/or other drugs

  • Measures to relieve symptoms and, if needed, provide life support

If herpes simplex virus and varicella zoster virus cannot be excluded, the antiviral drug acyclovir is given. Acyclovir is effective against herpes simplex and herpes zoster viruses. Cytomegalovirus encephalitis can be treated with the antiviral drug ganciclovir and/or other antiviral drugs. Sometimes several antibiotics are also given in case the cause is bacteria.

For HIV-associated encephalopathy, a combination of drugs used to treat HIV infection (antiretroviral drugs) helps the immune system function better and delays the progression of the infection and its complications, including dementia.

Autoimmune encephalitis is usually treated with the following:

  • Corticosteroids (prednisone or methylprednisolone)

  • Plasma exchange, which removes the abnormal antibodies from the blood, or immune globulin (antibodies obtained from the blood of people with a normal immune system), which is given intravenously

For other viruses and most other causes, no specific treatment is available. Treatment usually involves relieving symptoms (such as seizures and fever) and, when necessary, providing life support (for example, with a breathing tube) until the infection subsides—in about 1 to 2 weeks.

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