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Subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by the measles virus. more...

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Symptoms

Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years, and then gradual, progressive psychoneurological deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma.

Diagnosis

Characteristic periodic activity is seen on EEG; pathologically, the white matter of both the hemispheres and brainstem are affected, as well as the cerebral cortex, and eosinophilic inclusion bodies are present in the cytoplasm nuclei of neurons and glial cells.

Prognosis

Death usually occurs within 3 years.

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Measles
From Gale Encyclopedia of Medicine, 4/6/01 by Rosalyn S. Carson-DeWitt

Definition

Measles is an infection, caused by a virus, which causes an illness displaying a characteristic skin rash. Measles is also sometimes called rubeola, 5-day measles, or hard measles.

Description

Measles infections appear all over the world. Prior to the current effective immunization program, large-scale measles outbreaks occurred on a two to three-year cycle, usually in the winter and spring. Smaller outbreaks occurred during the off-years. Babies up to about eight months of age are usually protected from contracting measles, due to immune cells they receive from their mothers in the uterus. Once someone has had measles infection, he or she can never get it again.

Causes & symptoms

Measles is caused by a type of virus called a paramyxovirus. It is an extremely contagious infection, spread through the tiny droplets that may spray into the air when an individual carrying the virus sneezes or coughs. About 85% of those people exposed to the virus will become infected with it. About 95% of those people infected with the virus will develop the illness called measles. Once someone is infected with the virus, it takes about 7-18 days before he or she actually becomes ill. The most contagious time period is the three to five days before symptoms begin through about four days after the characteristic measles rash has begun to appear.

The first signs of measles infection are fever, extremely runny nose, red, runny eyes, and a cough. A few days later, a rash appears in the mouth, particularly on the mucous membrane which lines the cheeks. This rash consists of tiny white dots (like grains of salt or sand) on a reddish bump. These are called Koplik's spots, and are unique to measles infection. The throat becomes red, swollen, and sore.

A couple of days after the appearance of the Koplik's spots, the measles rash begins. It appears in a characteristic progression, from the head, face, and neck, to the trunk, then abdomen, and next out along the arms and legs. The rash starts out as flat, red patches, but eventually develops some bumps. The rash may be somewhat itchy. When the rash begins to appear, the fever usually climbs higher, sometimes reaching as high as 105°F (40.5°C). There may be nausea, vomiting, diarrhea, and multiple swollen lymph nodes. The cough is usually more problematic at this point, and the patient feels awful. The rash usually lasts about five days. As it fades, it turns a brownish color, and eventually the affected skin becomes dry and flaky.

Many patients (about 5-15%) develop other complications. Bacterial infections, such as ear infections, sinus infections, and pneumonia are common, especially in children. Other viral infections may also strike the patient, including croup, bronchitis, laryngitis, or viral pneumonia. Inflammation of the liver, appendix, intestine, or lymph nodes within the abdomen may cause other complications. Rarely, inflammations of the heart or kidneys, a drop in platelet count (causing episodes of difficult-to-control bleeding), or reactivation of an old tuberculosis infection can occur.

An extremely serious complication of measles infection is swelling of the brain. Called encephalitis, this can occur up to several weeks after the basic measles symptoms have resolved. About one out of every 1,000 patients develops this complication, and about 10-15% of these patients die. Symptoms include fever, headache, sleepiness, seizures, and coma. Long-term problems following recovery from measles encephalitis may include seizures and mental retardation.

A very rare complication of measles can occur up to 10 years following the initial infection. Called subacute sclerosing panencephalitis, this is a slowly progressing, smoldering swelling and destruction of the entire brain. It is most common among people who had measles infection prior to the age of two years. Symptoms include changes in personality, decreased intelligence with accompanying school problems, decreased coordination, involuntary jerks and movements of the body. The disease progresses so that the individual becomes increasingly dependent, ultimately becoming bedridden and unaware of his or her surroundings. Blindness may develop, and the temperature may spike (rise rapidly) and fall unpredictably as the brain structures responsible for temperature regulation are affected. Death is inevitable.

Diagnosis

Measles infection is almost always diagnosed based on its characteristic symptoms, including Koplik's spots, and a rash which spreads from central body structures out towards the arms and legs. If there is any doubt as to the diagnosis, then a specimen of body fluids (mucus, urine) can be collected and combined with fluorescent-tagged measles virus antibodies. Antibodies are produced by the body's immune cells that can recognize and bind to markers (antigens) on the outside of specific organisms, in this case the measles virus. Once the fluorescent antibodies have attached themselves to the measles antigens in the specimen, the specimen can be viewed under a special microscope to verify the presence of measles virus.

Treatment

There are no treatments available to stop measles infection. Treatment is primarily aimed at helping the patient to be as comfortable as possible, and watching carefully so that antibiotics can be started promptly if a bacterial infection develops. Fever and discomfort can be treated with acetaminophen. Children with measles should never be given aspirin, as this has caused the fatal disease Reye's syndrome in the past. A cool-mist vaporizer may help decrease the cough. Patients should be given a lot of liquids to drink, in order to avoid dehydration from the fever.

Some studies have shown that children with measles encephalitis benefit from relatively large doses of vitamin A.

Alternative treatment

Botanical immune enhancement (with echinacea , for example) can assist the body in working through this viral infection. Homeopathic support also can be effective throughout the course of the illness. Some specific alternative treatments to soothe patients with measles include the Chinese herbs bupleurum (Bupleurum chinense) and peppermint (Mentha piperita), as well as a preparation made from empty cicada (Cryptotympana atrata) shells. The itchiness of the rash can be relieved with witch hazel (Hamamelis virginiana), chickweed (Stellaria media), or oatmeal baths. The eyes can be soothed with an eyewash made from the herb eyebright (Euphrasia officinalis). Practitioners of ayurvedic medicine recommend ginger or clove tea.

Prognosis

The prognosis for an otherwise healthy, well-nourished child who contracts measles is usually quite good. In developing countries, however, death rates may reach 15-25%. Adolescents and adults usually have a more difficult course. Women who contract the disease while pregnant may give birth to a baby with hearing impairment. Although only 1 in 1,000 patients with measles will develop encephalitis, 10-15% of those who do will die, and about another 25% will be left with permanent brain damage.

Prevention

Measles is a highly preventable infection. A very effective vaccine exists, made of live measles viruses which have been treated so that they cannot cause actual infection. The important markers on the viruses are intact, however, which causes an individual's immune system to react. Immune cells called antibodies are produced, which in the event of a future infection with measles virus will quickly recognize the organism, and kill it off. Measles vaccines are usually given at about 15 months of age; because prior to that age, the baby's immune system is not mature enough to initiate a reaction strong enough to insure long-term protection from the virus. A repeat injection should be given at about 10 or 11 years of age. Outbreaks on college campuses have occurred among unimmunized or incorrectly immunized students.

Key Terms

Antibodies
Cells made by the immune system which have the ability to recognize foreign invaders (bacteria, viruses), and thus stimulate the immune system to kill them.
Antigens
Markers on the outside of such organisms as bacteria and viruses, which allow antibodies to recognize foreign invaders.
Encephalitis
Swelling, inflammation of the brain.
Koplik's spots
Tiny spots occurring inside the mouth, especially on the inside of the cheek. These spots consist of minuscule white dots (like grains of salt or sand) set onto a reddened bump. Unique to measles.

Further Reading

For Your Information

    Books

  • Gershon, Anne. "Measles (Rubeola)." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
  • Ray, C. George. "Pathogenic Viruses." In Sherris Medical Microbiology: An Introduction to Infectious Diseases, edited by Kenneth J. Ryan. Norwalk, CT: Appleton and Lange, 1994.
  • Stoffman, Phyllis. The Family Guide to Preventing and Treating 100 Infectious Diseases. New York: John Wiley and Sons, Inc., 1995.

    Periodicals

  • Borton, Dorothy. "Keeping Measles at Bay: Use These Four Techniques to Stop the Spread." Nursing 27, 12 (December 1997): 26.
  • Chavez, Gilberto F., and Arthur A. Ellis. "Pediatric Hospital Admissions for Measles: Lessons from the 1990 Epidemic." The Western Journal of Medicine 165, 1-2 (July/August 1996): 20+.
  • Hussey, Greg. "Managing Measles: Integrated Case Management Reduces Disease Severity." British Medical Journal 314, 7077 (February 1, 1997): 316+.
  • Klass, Perri. "Rash Decision." American Health 14, 4 (May 1995): 102+.
  • "Progress Toward Global Measles Control and Elimination, 1990-1996." The Journal of the American Medical Association 278, 17 (November 5, 1997): 1396+.
  • Ramsey, Alison. "Childhood Diseases Are Back." Reader's Digest 148, 886 (February 1996): 73+.

    Organizations

  • Centers for Disease Control and Prevention. (404) 332-4559. http://www.cdc.gov.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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