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Encephalitis
Encephalitis
Encephalitis often causes only mild flu-like signs and symptoms — such as a fever or headache — or no symptoms at all. Sometimes the flu-like symptoms are
more severe. Encephalitis can also cause confused thinking, seizures, or problems with senses or movement.
Rarely, encephalitis can be life-threatening. Timely diagnosis and treatment are important because it's difficult to predict how encephalitis will affect each
individual.
Symptoms
Bulging fontanel
Most people with viral encephalitis have mild flu-like symptoms, such as:
Headache
Fever
Aches in muscles or joints
Fatigue or weakness
Sometimes the signs and symptoms are more severe, and might include:
Confusion, agitation or hallucinations
Seizures
Loss of sensation or paralysis in certain areas of the face or body
Muscle weakness
Problems with speech or hearing
Loss of consciousness
In infants and young children, signs and symptoms might also include:
Bulging in the soft spots (fontanels) of an infant's skull
Nausea and vomiting
Body stiffness
Poor feeding or not waking for a feeding
Irritability
When to see a doctor
Get immediate care if you are experiencing any of the more-severe symptoms associated with encephalitis. Severe headache, fever and altered consciousness
require urgent care.
Infants and young children with any signs or symptoms of encephalitis should receive urgent care.
Causes
The exact cause of encephalitis is often unknown. But when a cause is known, the most common is a viral infection. Bacterial infections and noninfectious
inflammatory conditions also can cause encephalitis.
There are two main types of encephalitis:
Primary encephalitis. This condition occurs when a virus or other agent directly infects the brain. The infection may be concentrated in one area or
widespread. A primary infection may be a reactivation of a virus that had been inactive after a previous illness.
Secondary encephalitis. This condition results from a faulty immune system reaction to an infection elsewhere in the body. Instead of attacking only the
cells causing the infection, the immune system also mistakenly attacks healthy cells in the brain. Also known as post-infection encephalitis, secondary
encephalitis often occurs two to three weeks after the initial infection.
What is encephalitis?
Encephalitis is an acute inflammation (swelling) of the brain usually resulting from either a viral infection or due to the body's own immune system mistakenly
attacking brain tissue.
In medicine, "acute" means it comes on abruptly and develops rapidly; it usually requires urgent care.
The most common cause is a viral infection. The brain becomes inflamed as a result of the body's attempt to fight off the virus.
Encephalitis occurs in 1 in every 1,000 cases of measles.
Encephalitis generally begins with fever and headache. The symptoms rapidly worsen, and there may be seizures (fits), confusion, drowsiness, and loss of
consciousness, and even coma.
Encephalitis can be life-threatening, but this is rare. Mortality depends on a number of factors, including the severity of the disease and age.
Younger patients tend to recover without many ongoing health issues, whereas older patients are at higher risk for complications and mortality.
When there is direct viral infection of the brain or spinal cord, it is called primary encephalitis. Secondary encephalitis refers to an infection which started off
elsewhere in the body and then spread to the brain.
Types
Different types of encephalitis have different causes.
Japanese encephalitis is spread by mosquitoes
Tick-borne encephalitis is spread by ticks
Rabies can be spread through a bite from a mammal
There is also primary or secondary encephalitis.
Primary or infectious encephalitis can result if a fungus, virus, or bacterium infects the brain.
Secondary, or post-infectious, encephalitis is when the immune system responds to a previous infection and mistakenly attacks the brain.
Symptoms
The patient typically has a fever, headache, and photophobia (excessive sensitivity to light). There may also be general weakness and seizures.
Less common symptoms
The individual may also experience nuchal rigidity (neck stiffness), which can lead to a misdiagnosis of meningitis. There may be stiffness of the limbs, slow
movements, and clumsiness. The patient may also be drowsy and have a cough.
More serious cases
In more serious cases, the person may experience very severe headaches, nausea, vomiting, confusion, disorientation, memory loss, speech problems, hearing
problems, hallucinations, as well as seizures and possibly coma. In some cases, the patient can become aggressive.
Signs and symptoms in infants
Initially, encephalitis is harder to detect in young children and babies. Parents or guardians should look out for vomiting, a bulging fontanel (the soft area on the
top center of the head), incessant crying that does not get better when the baby is picked up and comforted, and body stiffness.
Causes
Encephalitis can develop as a result of a direct infection to the brain by a virus, bacterium, or fungus, or when the immune system responds to a previous
infection; the immune system mistakenly attacks brain tissue.
Primary (infectious) encephalitis can be split into three main categories of viruses:
Common viruses, including HSV (herpes simplex virus) and EBV (Epstein-Barr virus)
Childhood viruses, including measles and mumps
Arboviruses (spread by mosquitoes, ticks, and other insects), including Japanese encephalitis, West Nile encephalitis, and tick-borne encephalitis
Secondary encephalitis: could be caused by a complication of a viral infection. Symptoms start to appear days or even weeks after the initial infection. The
patient's immune system treats healthy brain cells as foreign organisms and attacks them. We still do not know why the immune system malfunctions in this way.
In more than 50 percent of encephalitis cases, the exact cause of the illness is not tracked down.
Encephalitis is more likely to affect children, older adults, individuals with weakened immune systems, and people who live in areas where mosquitoes and ticks
that spread specific viruses are common.
Treatment
Treatment for encephalitis focuses on alleviating symptoms. There are only a limited number of reliably tested specific antiviral agents that can help, one of
which is acyclovir; success is limited for most infections except when the condition is due to herpes simplex.
Corticosteroids may be administered to reduce the brain's inflammation, especially in cases of post-infectious (secondary) encephalitis. If the patient has severe
symptoms, they may need mechanical ventilation to help them breathe and other supportive treatment.
Anticonvulsants are sometimes given to patients who have seizures. Sedatives can be effective for seizures, restlessness, and irritability. For patients with mild
symptoms, the best treatment is rest, plenty of fluids, and Tylenol (paracetamol) for fever and headaches.
Diagnosis
Doctors who identify the classic symptoms in adults - fever, headache, confusion, and occasionally seizures, or irritability, poor appetite, and fever in young
children - may order further diagnostic tests.
A neurological examination generally finds that the patient is confused and drowsy.
If the neck is stiff, caused by irritation of the meninges (membranes that cover the brain and spinal cord), the doctor may consider a diagnosis of meningitis or
meningoencephalitis.
A lumbar puncture, which takes a sample of cerebrospinal fluid from the spine, might reveal higher-than-normal levels of protein and white blood cells.
However, this test is not always conclusive, in some cases, the results may come back normal even though the patient has encephalitis.
A CT scan may be useful in detecting changes in brain structure. It can also rule out other causes, such as stroke, an aneurysm, or a tumor. However, an MRI is
the best imaging option for encephalitis; it can identify the classic brain changes that suggest encephalitis.
An EEG (electroencephalograph) that monitors the electrical activity of the brain may show sharp waves in one or both of the temporal lobes in patients with
encephalitis.
The doctor might order a blood test if a West Nile virus infection is thought to be the cause.
Complications
The majority of patients who have encephalitis go on to have at least one complication, especially elderly patients, those who had symptoms of coma, and
individuals who did not receive treatment at an early stage.
Complications may include:
Loss of memory - especially among those who had herpes simplex virus encephalitis
Behavioral or personality changes - such as mood swings, bouts of frustration and anger, and anxiety
Epilepsy
Aphasia - language and speech problems
Prevention
Keeping up-to-date with vaccines is the most effective way of reducing the risk of developing encephalitis. These include vaccines for measles, mumps, rubella,
and if the virus exists in those areas, Japanese encephalitis and tick-borne encephalitis.
In areas known to have mosquitoes that carry encephalitis-causing viruses, individuals should take measures to reduce the risk of being bitten. This may include
wearing appropriate clothing, avoiding mosquito-infested areas, avoiding going outside at specific times during the day when there are large numbers of
mosquitoes about, keeping the home mosquito-free, using mosquito repellent, and making sure there is no stagnant water around the home.
What Is Encephalitis?
Encephalitis is defined as inflammation of the brain. This definition means encephalitis is different from meningitis, which is defined as inflammation of the layers
of tissue, or membranes, covering the brain. Unfortunately, in some people, both of the diseases may coexist and lead to a more complex diagnosis and
treatment plan; in addition, both conditions share many of the same symptoms so they may be difficult to distinguish. There are many causes of
encephalitis: viruses, bacteria, parasites, chemicals, and even autoimmune reactions. This article is designed to discuss general features of encephalitis; it is not
designed to be all inclusive as book chapters have been written on individual causes. The reader wanting more information than is present in this introduction is
urged to click on the links provided and to check the references provided at the end of this article.
In clinical practice, most doctors consider encephalitis to be a viral illness. Viruses such as those responsible for causing cold sores, mumps, measles,
and chickenpoxcan also cause encephalitis; they will not be further discussed in this article because their major disease manifestations, symptoms, and
complications are detailed in other articles. Major causes of viral encephalitis are herpesviruses and the arboviruses. Arboviruses are spread by insects such as
mosquitoes and ticks. The equine (meaning horse), West Nile, Japanese, La Crosse, and St. Louis encephalitisviruses are all mosquito-borne arboviruses. Although
viruses are the most common source of infection, bacteria, fungi, parasites, chemicals, and autoimmune reactions (limbic encephalitis) can also be responsible
for encephalitis. However, current data suggest that these are far less common than viral infections as causes of encephalitis.
Viral encephalitis resembles the flu in terms of its symptoms and usually lasts for two to three weeks. It can vary from mild to life-threatening and even cause
death. Most people with a mild infection can recover fully. Those with a more severe infection can recover although they may have damage to their nervous
system. This damage can be permanent. Some other general features of viral encephalitis are as follows:
Age, season, geographic location, regional climate conditions, and the strength of the person's immune system play a role in development of the disease
and severity of the illness.
Herpes simplex (the virus causing cold sores) remains the most common virus involved in encephalitis in the United States and throughout the world.
These viruses are usually transmitted from person to person.
In the United States, there are five main encephalitis-causing viruses spread by mosquitoes: West Nile, Eastern equine encephalitis (EEE), Western
equine encephalitis (WEE), La Crosse, and St. Louis encephalitis. Two types of Powassan viruses, an infrequent cause of encephalitis, are transmitted by at least
two types of ticks.
Venezuelan equine encephalitis is found in South America. It can be a rare cause of encephalitis in the southwestern United States, particularly Texas.
The infection is very mild, and nervous system damage is rare.
Japanese encephalitis virus is the most common arbovirus in the world (virus transmitted by blood-sucking mosquitoes or ticks) and is responsible for
50,000 cases and 15,000 deaths per year worldwide. Most of China, Southeast Asia, and the Indian subcontinent are affected.
What Causes Viral Encephalitis?
Herpes simplex (HSV): This type of virus causes cold sores of the mouth and lesions of the genitals. HSV is transmitted directly through human contact.
Newborns can also get the virus by passing through an infected birth canal. Once inside the body, the virus travels through nerve fibers and can cause an
infection of the brain. The virus may also undergo a period of latency in which it is inactive. At a later time, emotional or physical stress can reactivate the virus to
cause an infection of the brain. It causes the most subacute (between acute and chronic) and chronic (lasting three or more months) encephalitis infections in
humans.
Arbovirus: Hosts are animals such as birds, pigs, chipmunks, and squirrels that carry the virus. Mosquitoes (known as vectors, or ways of transmitting the
virus) feed on these animals and become infected. The virus grows and cycles between the hosts and the vectors. Humans become infected through mosquito
bites. Once inside the body, the virus replicates and travels in the bloodstream. If there is a large enough amount of the virus, the brain can become infected. The
majority of cases occur between June and September when the mosquitoes are most active. In warmer climates, the disease can occur year-round.
o West Nile virus (WNV): This virus was first isolated from an adult woman with a fever in the West Nile District of Uganda in 1937. The nature of
the virus was studied in Egypt in the 1950s. In 1957, as a result of an outbreak in Israel in the elderly, the WNV became recognized as a cause for severe
inflammation of the spinal cord and brain in humans. In the early 1960s, it was first noted that horses were becoming ill in Egypt and France. This virus then
emerged in North America in 1999, with encephalitis reported in humans and horses.
The virus cycles between the Culex mosquito and hosts such as birds, horses, cats, bats, chipmunks, skunks, squirrels, and domestic
rabbits. The mosquito feeds on the infected hosts, carries the virus in its salivary glands, and then passes it on to humans or other animals during a blood meal. It
usually takes three to 15 days from the time of infection to the onset of disease symptoms. In the U.S., crows became a major host, but the disease would kill the
crows; as a result, the number of infections dropped dramatically as the crow population died off.
West Nile encephalitis is not transmitted from person to person (such as through touching or kissing or from a health-care worker
caring for a sick person) nor can it be passed from animal to human. Blood transfusions are the exception; the virus may be passed among people by blood
transfusions if the donor is infected.
The chance that people will become severely ill from a mosquito bite is extremely small. According to the Centers for Disease Control
and Prevention (CDC), even in areas where mosquitoes are reported to carry the virus, much fewer than 1% of them are infected. Furthermore, less than 1% of
the people who get bitten and become infected will become severely ill. Therefore, the majority of cases are mild, and people can fully recover. For example, the
CDC reported 690 people infected in 2011, with a total of 43 deaths.
Prognosis is usually guarded in the extremes of age (infants, young children, and the elderly). Death rates as a result of West Nile
encephalitis range from 3%-15% and are highest among the elderly. At the present time, there is no documented evidence to suggest that a pregnancy is at risk
due to WNV infection. It is assumed that if a person contracts WNV, he or she will develop a natural immunity that is lifelong. However, it may wane in later
years. Currently, there is no commercially available vaccine for humans.
A relative of West Nile virus, Powassan virus, was discovered in 1958, but its vector is the blacklegged (deer) tick. It is very rare; only
about 60 individuals have been diagnosed since 1958. There is no vaccine available. It has a 10% death rate.
La Crosse encephalitis: The first case occurred in La Crosse, Wisconsin, in 1963. Since then, the largest number of cases has been identified in woodland
areas of the midwestern and mid-Atlantic United States. This virus is the most common cause of mosquito-borne encephalitis in children younger than 16 years
of age. Each year, about 75 cases are reported to the Centers for Disease Control and Prevention (CDC). The virus cycles between the daytime-biting treehole
mosquito (Aedes triseriatus) and hosts such as chipmunks and squirrels. Some investigators consider the cause of California encephalitis to be the La Crosse virus.
The La Crosse encephalitis virus can cause adverse effects on IQ and school performance. About 80-100 people are diagnosed each year in the U.S., and 1% of
people with this infection may die.
St. Louis encephalitis: Since 1964, an average of about 102 people is reported infected per year. Outbreaks can occur throughout most of the United
States, although large urban epidemics have occurred in the midwestern and southeastern regions. The last major epidemic of St. Louis encephalitis occurred in
the Midwest from 1974-1977. There were 2,500 cases in 35 states reported to the CDC. Additionally, there were 20 reported cases in New Orleans in 1999. The
virus cycles between birds and the Culex mosquitoes breeding in stagnant water. It grows in both the mosquito and the bird but does not make either one sick.
Only the infected mosquito can transmit the disease to humans during the blood meal. The virus cannot be transmitted from person to person through kissing or
touching nor can it be transmitted from the infected bird. The disease tends to affect mostly adults and is generally milder in children.
What Are Other Less Frequent Causes of Encephalitis?
Eastern equine encephalitis (EEE): According to the CDC, there have been confirmed cases in the United States of EEE since 1964 with a rate of about 0-
21 diagnosed infections per year (average about six per year). This virus is found along the East and Gulf Coasts. The virus causes severe disease in
horses, puppies, and birds such as pheasants, quails, and ostriches. In humans, flu-like symptoms develop four to 10 days after the bite of an infected mosquito.
Usually, human illnesses are preceded by those in horses. EEE can cause death in 50%-75% of human infections; 90% of infected people have mild to severe
impairments. Those who recover may suffer severe permanent brain damage such as mental retardation, seizures, paralysis, and behavior abnormalities.
Western equine encephalitis (WEE): This virus was isolated from the brain of a horse with encephalitis in California in 1930. The worst epidemic was in
Canada and the western U.S. when over 300,000 horses and mules were diagnosed, along with over 3,300 humans in 1941. Since 1964, there have been at least
639 confirmed cases, but currently only a few per year are reported. However, it remains a cause of encephalitis in the western part of the United States and
Canada. In 1994, there were two confirmed and several suspected cases of WEE reported in Wyoming. In 1997, 35 strains of WEE virus were isolated from
mosquitoes collected in Scotts Bluff County, Nebraska. The WEE virus cycles between certain types of birds (small, mostly songbirds) and Culex
tarsalismosquitoes, a species associated with irrigated agriculture and stream drainage. The virus has also been found in several other mammals. Horses and
humans become sick through bites by infected mosquitoes. Infants are particularly affected and can have permanent problems such as seizure disorders and
developmental delay as a result of the infection. A vaccine is not available for humans. WEE is becoming more frequently encountered in the U.S.
Venezuelan equine encephalitis (VEE): This virus is found in Central and South America and is a rare cause of encephalitis in the southwestern part of
the United States. It is an important cause of encephalitis in horses and humans in South America. From 1969-1971, an outbreak from South America to Texas
killed over 200,000 horses. In 1995, there were an estimated 90,000 human infections with VEE in Columbia and Venezuela. The virus cycles between forest-
dwelling rodents and mosquito vectors, especially the species Culex. VEE infection in humans is much less severe than that of WEE and EEE. While adults tend to
develop a flu-like illness, children tend to develop overt encephalitis. Deaths are rare in humans but are common in horses. There is an effective vaccine for
horses but none for humans.
Japanese encephalitis: This virus is responsible for 50,000 cases and 15,000 deaths per year. Most of China, Southeast Asia, and the Indian subcontinent
are affected. The geographic distribution is expanding. Rarely, cases may appear in United States civilians and military personnel traveling to and living in Asia.
Children and young adults are mostly affected. Older adults are affected when there are epidemics in new locations. The virus cycles between domestic pigs, wild
birds, and Culex tritaeniorhynchus mosquitoes, which breed in rice fields. The disease is not transmitted through human contact, pigs, or birds. Only the
mosquitoes can transmit the disease during feedings.
Zika virus: This virus is spread by the bite of an infected Aedes species mosquito; the virus can be passed from a pregnant woman to her fetus, and this
has been associated with certain birth defects, including microcephaly, Guillain-Barré disease, and disseminated encephalomyelitis. A major outbreak of this
disease began in 2015 and is ongoing in Brazil and has spread to other countries. Zika typically is spread via the mosquito vector, but transmission through sexual
intercourse has also been documented.
The following is a short summary of the viruses that cause the majority of encephalitis infections, although they may also cause other diseases.
Herpes Human-to-human
United States/the world Prompt treatment with acyclovir increases survival to 90%
encephalitis contact
Majority are mild cases. Less than 1% of those infected will become
West Nile Africa, West Asia, Middle East, United Mosquito/mostly
severely ill. Full recovery is expected. A vaccine for humans is not
encephalitis States birds
commercially available.
Western
Western United States and Often occurs in horses.
equine Mosquito/birds
Canada Particularly affects infants
encephalitis
Venezuelan
equine Western Hemisphere Mosquito/rodents Rare in United States; low mortality rate, rare after-effects
encephalitis
A special cause of viral encephalitis is HIV. This virus is mainly known for its damage to the human immune system. However, as HIV disease progresses, some
individuals develop encephalitis symptoms termed AIDS dementia complex. It results in cognitive disorders (There is memory loss, abstract thinking and verbal
fluency decline, and motor control may be markedly decreased.). Other causes of encephalitis are as follows but will not be further discussed in detail in this
article; the reader is referred to the links provided:
Bacteria, such as N. meningitidis, and those that cause Lyme disease, syphilis, tuberculosis, and occasionally other bacteria such as Mycoplasma spp.
have been implicated in a few individuals.
Fungi such as Candida, Mucormycosis, Cryptococcus, and others
Rabies virus
Parasites such as Toxoplasma (often seen in HIV-infected patients) or the parasite Naegleria
Allergies to vaccinations
Autoimmune disease such as Rasmussen's encephalitis
Cancers involving the brain tissue
Prion caused encephalitis (rare) such as bovine spongiform encephalitis or mad cow disease
Myalgic encephalitis or chronic fatigue syndrome (no defined cause)
Chemical encephalitis such as that seen with alcohol (Wernicke-Korsakoff syndrome) due to a decline in liver functions and ultimately, affecting the
brain tissue, or by drug use
In recent years, researchers have begun more intensive studies of some types of encephalitis. In 2012, the CDC began a multicenter study of epidemic myalgic
encephalomyelitis (or chronic fatigue syndrome or CFS) that is ongoing to better understand this problem.
Another cause of encephalitis under study is encephalitis believed to be caused by an autoantibody attack on subunits of brain N-methyl-d-aspartate (NMDA)
glutamate receptors. The autoantibodies are termed anti-NMDA receptor antibodies, and the disease is termed NMDA receptor encephalitis, first identified in
2007. The disease is found mainly in young women (over 80%) and has been associated with ovarian teratomas (germ cell tumors). Some investigators think it
may have been seen previously as an outbreak of encephalitis of unknown cause termed (epidemic) encephalitis lethargica that occurred worldwide between
1918 and 1928. Recent research suggests that the anti-NMDA receptor symptoms (seizures, unresponsiveness, motor-control problems, and others) caused by
this autoimmune disease can be treated with immunotherapy to reduce or halt symptoms in some patients. Some individuals believe this disease is related
to autism but currently no convincing evidence has shown such a relationship.
Is Encephalitis Contagious?
The answer to this question depends upon the underlying cause of encephalitis. For example, some herpes viruses are contagious from person to person and can
cause encephalitis. In this case, encephalitis is considered to be contagious. Viruses that are transmitted through the bite of infected insects are not considered
contagious from person to person. Other causes of encephalitis such as autoimmune problems or chemical encephalitis are not contagious.
What Is the Incubation Period and Contagious Period for Encephalitis?
The contagious period and incubation period for encephalitis depends on the underlying cause of encephalitis. For example, some herpesviruses have an
incubation period of about three to seven days on average but may range from about one to three weeks. The contagious period for some viruses may include
the incubation period and the time it takes for the lesions (blisters, for example, in shingles) to crust over. Consequently, the contagious period and incubation
period for encephalitis depends upon the cause of encephalitis being contagious; noncontagious encephalitis has no contagious or incubation period.
What Are Encephalitis Symptoms and Signs?
he signs and symptoms of encephalitis are the same for adults and children. Infants may have poor feeding, irritability, vomiting, bulging fontanel, and body
stiffness; such symptoms in an infant always constitute a medical emergency.
Signs and symptoms may last for two to three weeks, are flu-like, and can include one or more of the following which in some individuals may become
progressively worse and continue over time:
o Fever
o Fatigue, muscle weakness, rhythmic muscle contractions, muscle pain
o Sore throat
o Stiff neck and back
o Loss of appetite
o Vomiting and nausea
o Headache
o Confusion
o Irritability
o Unsteady gait, weakness
o Problems with coordination
o Drowsiness
o Visual sensitivity to light
More severe cases may involve these signs and symptoms:
o Seizures
o Muscle weakness
o Paralysis
o Memory loss
o Sudden impaired judgment
o Delirium and/or hallucinations
o Disorientation
o Poor responsiveness or altered level of consciousness
United States. National Institute of Neurological Disorders and Stroke. "NINDS Rasmussen's Encephalitis Information Page." Dec. 19, 2011.
<http://www.ninds.nih.gov/disorders/rasmussen/rasmussen.htm>.