encephalitis

encephalitis, from Greek enkephalos (“brain”) and itis (“inflammation”), inflammation of the brain. Inflammation affecting the brain may also involve adjoining structures; encephalomyelitis is inflammation of the brain and spinal cord, and meningoencephalitis is inflammation of the brain and meninges (the membranes covering the brain).

Causes

Encephalitis is most often caused by an infectious organism, though it is sometimes caused by noninfective agents, including chemicals such as lead, arsenic, and mercury. Although encephalitis can be produced by many different types of organisms, such as bacteria, protozoans, and helminths (worms), the most frequent causal agents are viruses. The encephalitis-producing viruses are divided into two groups: (1) those that invade the body and produce no damage until they are carried by the bloodstream to neurons in the brain (e.g., the rabies and arthropod-borne viruses) and (2) those that invade the body and first injure nonnervous tissues and then secondarily invade brain cells (e.g., the viruses causing herpes simplex, herpes zoster, dengue, acquired immune deficiency syndrome [AIDS], and yellow fever).

In rare instances, encephalitis develops because the immune system attacks healthy cells in the brain, leading to brain inflammation; this condition is known as autoimmune encephalitis. The condition occurs sporadically and is sometimes associated with cancer or a prior infection.

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Symptoms and treatment

Symptoms common to most types of encephalitis are fever, headache, drowsiness, lethargy, coma, tremors, and a stiff neck and back. Convulsions may occur in patients of any age but are most common in infants. Characteristic neurological signs include uncoordinated and involuntary movements, weakness of the arms, legs, or other parts of the body, or unusual sensitivity of the skin to various types of stimuli. These symptoms and signs and an examination of the cerebrospinal fluid by a lumbar puncture (spinal tap) can usually establish the presence of encephalitis, but they do not necessarily establish the cause, which often remains unknown. This makes specific treatment difficult, and, even when the causative virus is known, there may be no effective treatment. Treatment of encephalitis is therefore largely supportive. Patients require intensive medical care, with continuous monitoring of their heart and respiratory functions and management of their fluid and electrolyte balances.

Symptoms remaining after recovery from the acute phase of brain inflammation vary considerably, depending on the type of encephalitis and on the age and general health of the patient. Many individuals are weak and debilitated after an attack but recover with no serious effects. Some encephalitides may cause irreparable brain damage. Prognosis depends upon the specific viral agent that caused the encephalitis; mortality can be as high as 70 percent.

Autoimmune encephalitis is characterized by a wide variety of psychiatric and neurologic symptoms. These symptoms can include aggression, euphoria, fear, compulsive behaviour, impaired thinking, forgetfulness, loss of balance, seizures, and problems with speech and vision.

Demyelinating encephalitis

A large number of acute encephalitides are of the type known as demyelinating encephalitis, which may develop in children as a complication of viral diseases such as measles or chickenpox or as a result of vaccination against such viral diseases as smallpox. Damage to the nerve cell body does not occur, but the insulation (myelin sheath) surrounding the nerve fibres is gradually destroyed.

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Epidemics of encephalitis

Outbreaks of encephalitis occur periodically. One of the most common epidemic forms is Japanese encephalitis, which is caused by a mosquito-borne virus and results in acute illness. Japanese encephalitis is found primarily in Asia. Other viral forms of encephalitis, such as St. Louis encephalitis and La Crosse encephalitis, cause sporadic disease in some areas of the United States. Strains of Venezuelan equine encephalitis (VEE), Western equine encephalitis (WEE), and Eastern equine encephalitis (EEE) virus can also cause disease in humans. In the late 1960s some 200,000 people in central Colombia were infected with the Venezuelan strain, which had also spread north through Central America and Mexico and into the United States, causing illness in thousands more people and killing tens of thousands of equines as it spread. A 2002 West Nile virus epidemic in the United States was the largest human encephalitis outbreak recorded in the Western Hemisphere at the time. EEE virus is also found in the United States, where it is a rare cause of illness. It occurs most commonly along the East Coast but is also found in the Great Lakes region. About 30 percent of persons infected with EEE virus die.

Encephalitis lethargica, or sleeping sickness (to be distinguished from African sleeping sickness, or African trypanosomiasis), occurred in epidemics in Europe and in the United States about the time of World War I but has not been reported since 1930, although certain individuals may rarely exhibit residual symptoms (postencephalitic parkinsonism). The causative agent of sleeping sickness was never established, although the influenza virus was suspected.