Encephalitis

Encyclopedia of Medicine by Richard Robinson

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Definition

Encephalitis is an inflammation of the brain, usually caused by a direct viral infection or a hypersensitivity reaction to a virus or foreign protein. Brain inflammation caused by a bacterial infection is sometimes called cerebritis. When both the brain and spinal cord are involved, the disorder is called encephalomyelitis. An inflammation of the brain's covering, or meninges, is called meningitis.

Description

Encephalitis is an inflammation of the brain. The inflammation is a reaction of the body's immune system to infection or invasion. During the inflammation, the brain's tissues become swollen. The combination of the infection and the immune reaction to it can cause headache and a fever, as well as more severe symptoms in some cases.

Approximately 2000 cases of encephalitis are reported to the Centers for Disease Control in Atlanta, GA each year. The viruses causing primary encephalitis can be epidemic or sporadic. The polio virus is an epidemic cause. Arthropod-borne viral encephalitis is responsible for most epidemic viral encephalitis. The viruses live in animal hosts and mosquitos that transmit the disease. The most common form of non-epidemic or sporadic encephalitis is caused by the herpes simplex virus, type 1 (HSV-1) and has a high rate of death. Mumps is another example of a sporadic cause.

Causes & symptoms

Causes

There are more than a dozen viruses that can cause encephalitis, spread by either human-to human contact or by animal bites. Encephalitis may occur with several common viral infections of childhood. Viruses and viral diseases that may cause encephalitis include:

• Chickenpox
• Measles
• Mumps
• Epstein-Barr virus (EBV)
• Cytomegalovirus infection
• HIV
• Herpes simplex
• Herpes zoster (shingles)
• Herpes B
• Polio
• Rabies
• Mosquito-borne viruses (arboviruses).

Primary encephalitis is caused by direct infection by the virus, while secondary encephalitis is due to a post-infectious immune reaction to viral infection elsewhere in the body. Secondary encephalitis may occur with measles, chickenpox, mumps, rubella, and EBV. In secondary encephalitis, symptoms usually begin 5-10 days after the onset of the disease itself and are related to the breakdown of the myelin sheath that covers nerve fibers.

In rare cases, encephalitis may follow vaccination against some of the viral diseases listed above. Creutzfeldt-Jakob disease, a very rare brain disorder caused by an infectious particle called a prion, may also cause encephalitis.

Mosquitoes spread viruses responsible for equine encephalitis (eastern and western types), St. Louis encephalitis, California encephalitis, and Japanese encephalitis. Lyme disease, spread by ticks, can cause encephalitis, as can Colorado tick fever. Rabies is most often spread by animal bites from dogs, cats, mice, raccoons, squirrels, and bats and may cause encephalitis.

Equine encephalitis is carried by mosquitoes that do not normally bite humans but do bite horses and birds. It is occasionally picked up from these animals by mosquitoes that do bite humans. Japanese encephalitis and St. Louis encephalitis are also carried by mosquitoes. The risk of contracting a mosquito-borne virus is greatest in mid- to late summer, when mosquitoes are most active, in those rural areas where these viruses are known to exist. Eastern equine encephalitis occurs in eastern and southeastern United States; western equine and California encephalitis occur throughout the West; and St. Louis encephalitis occurs throughout the country. Japanese encephalitis does not occur in the United States, but is found throughout much of Asia. The viruses responsible for these diseases are classified as arbovirus and these diseases are collectively called arbovirus encephalitis.

Herpes simplex encephalitis, the most common form of sporadic encephalitis in western countries, is a disease with significantly high mortality. It occurs in children and adults and both sides of the brain are effected. It is theorized that brain infection is caused by the virus moving from a peripheral location to the brain via two nerves, the olfactory and the trigeminal (largest nerves in the skull).

Herpes simplex encephalitis is responsible for 10% of all encephalitis cases and is the main cause of sporadic, fatal encephalitis. In untreated patients, the rate of death is 70% while the mortality is 15-20% in patients who have been treated with acyclovir. The symptoms of herpes simplex encephalitis are fever, rapidly disintegrating mental state, headache, and behavioral changes.

Symptoms

The symptoms of encephalitis range from very mild to very severe and may include:

• Headache
• Fever
• Lethargy (sleepiness, decreased alertness, and fatigue)
• Malaise
• Nausea and vomiting
• Visual disturbances
• Tremor
• Decreased consciousness (drowsiness, confusion, delirium, unconsciousness)
• Stiff neck
• Seizures.

Symptoms may progress rapidly, changing from mild to severe within several days or even several hours.

Diagnosis

Diagnosis of encephalitis includes careful questioning to determine possible exposure to viral sources. Tests which can help confirm the diagnosis and rule out other disorders include:

• Blood tests. These are to detect antibodies to viral antigens, and foreign proteins.
• Cerebrospinal fluid analysis (spinal tap). This detects viral antigens, and provides culture specimens for the virus or bacteria that may be present in the cerebrospinal fluid.
• Electroencephalogram (EEG)
• CT and MRI scans.

A brain biopsy (surgical gathering of a small tissue sample) may be recommended in some cases where treatment to date has been ineffective and the cause of the encephalitis is unclear. Definite diagnosis by biopsy may allow specific treatment that would otherwise be too risky.