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Madisons Foundation - Moms And Dads In Search Of Needed Support
Progressive Rubella Panencephalitis (PRP)
Monday, 01 August 2005
Last Updated Monday, 01 August 2005
What
Progressive Rubella Panencephalitis (PRP) is a rare complication of having a rubella infection. Rubella infection can occur in the womb (congenital) or after birth (acquired). PRP represents a persistent and slowly progressive rubella infection of the central nervous system (the brain and the spinal cord) causing inflammation in the brain (panencephalitis) and progressive neurologic deterioration.Who
Rubella is a viral disease that causes infections worldwide, but Hispanics born in Central and South America have accounted for a large portion of recent cases. Rubella typically has the highest incidence during the spring months but may be seen throughout the year. It occurs equally in males and females. Typically a person has life-long immunity against rubella after successfully recovering from a rubella infection, although it is possible for reinfection to occur. Due to routine childhood vaccination against rubella, the incidence of congenital rubella syndrome in the United States is very low and estimated at 4-11 cases per year. For those children who eventually develop PRP, it usually occurs toward the end of the first decade of life or in the early teenage years. PRP more commonly follows cases of congenital rubella rather than rubella acquired after birth.Signs and Symptoms
PRP may develop several years after a documented rubella infection. Some of the features or rubella are described below: Children with congenital rubella syndrome may have a variety of symptoms. They usually have some form of heart disease, a small head, low birth weight, bone disease, and vision and hearing problems with or without mental retardation. Often these children will have purplish lesions on the skin. Many children who are born without symptoms will develop manifestations of the disease within the first 5 years of life. These may include developmental problems (including poor motor, intellectual, and language development), hepatitis, hormone deficiencies leading to diabetes and stunted growth, and also progressive rubella panencephalitis. Children who acquire a rubella infection after birth have symptoms that are variable and generally mild. Approximately 25-50% of cases are without symptoms. The first signs of rubella are seen about 16 to 18 days after the virus is acquired and lasts for about 2 to 3 weeks. The principal manifestations of symptomatic disease are low-grade fever, rash, and swollen lymph nodes. The lymph nodes are especially affected in the neck and may cause a slightly stiff neck. The rash usually resolves completely within 2 to 4 days. Less common symptoms include malaise, anorexia, headaches, low platelet count, and progressive rubella panencephalitis. PRP usually develops between 8 and 19 years of age. Some symptoms suggestive of PRP include progressive intellectual deterioration, seizures, jerky and uncoordinated movements, spastic movements, and myoclonus (rapid contractions of a group of muscles). Often the first signs to be noticed are deterioration of school performance and behavioral changes which eventually progress to global dementia.Possible Causes
Rubella is caused by the rubella virus that is present in the blood, mucus, and other secretions in the nose and throat of an infected person. Intimate person-to-person contact is generally required to pass the rubella virus by coughing, sneezing or contaminated articles to another person who inhales droplets of contaminated secretions. Infants who are born with rubella obtained it through the placenta and may be able to spread the virus for many weeks or months after birth. The child who acquires rubella after birth may be able to spread it from 2 weeks before the onset of a rash to 1 week after the rash fades. The exact reason why some people with rubella infections eventually get PRP is currently unknown. Some believe that PRP develops when a rubella infection is reactivated, while others believe that PRP develops due to a slow but progressive rubella infection.Diagnosis
A complete history and physical examination are needed to diagnose any type of rubella infection. If congenital rubella is suspected, a maternal history of prior rubella or exposure to rubella (especially during the first trimester of pregnancy) is a key issue. After birth, tests of the baby’s hearing and vision are routinely done. The head circumference is measured and an ultrasound or CT scan of the baby’s brain is obtained. . Viral cultures of secretions from the throat, tears, urine, feces, or cerebrospinal fluid (protective fluid around the spinal cord and brain) can help to determine if the rubella virus is present. A blood test for various types of rubella antibodies (protective proteins) can help distinguish between a congenital or acquired infection. X-Rays of the bones are obtained to look for signs of infection or deformities. A variety of other tests may be done to exclude other diseases that could also cause the presenting symptoms. The diagnosis of PRP is usually made by testing the blood and the cerebrospinal fluid for specific markers that are common in PRP.Treatment
The most effective and important way to prevent PRP is by preventing rubella infection. The vaccine for rubella is currently widely available and can be given alone or as a combination MMR (measles, mumps, rubella) vaccine. The vaccine is recommended for children between the ages of 12 and 15 months and upon entry to grade school or junior high school. Because the vast majority of people in the United States are vaccinated against rubella, the most important method for controlling rubella now is to reduce exposure of susceptible women in the childbearing age group to people infected with rubella. Those who are infected with rubella should limit their contacts with others since the virus can be spread fairly easily for at least 2 weeks (infection after birth) and up to 2 years (congenital infection). There is currently no specific treatment for congenital rubella or rubella acquired after birth, but the infection usually resolves without medication. Treatment with immunoglobulins (to help boost the immune system to fight a rubella infection that is acquired after birth) has occasionally been successful, but research is still ongoing regarding this method of treatment. While there is no specific treatment for PRP currently, research is ongoing in the hope of finding effective medications to treat and cure PRP.Prognosis
The prognosis for children who acquire a rubella infection after birth is excellent. Most of these infections resolve without treatment and very few of these eventually develop into Progressive Rubella Panencephalitis. The development of PRP typically takes several years to progress and, unless research provides a cure, it will eventually be fatal. In order to avoid the threat of Progressive Rubella Panencephalitis it is imperative that all children and women of childbearing age be vaccinated against rubella. To avoid congenital rubella infection, pregnant women must avoid contact with people who have a known rubella infection.Connect with other parents
In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.Weblinks
MedlinePlus: Rubella
This is a good website that explains rubella and has some helpful pictures and definitions of medical terms.
CERHR: Rubella
This website has general information about rubella and links to other helpful resources.
MayoClinic.com
This is a great website with information about rubella and an explanation of encephalitis.
Centers for Disease Control: Rubella
This website has detailed information about rubella and the vaccine.
Kids Health
Some basic information about encephalitis.
Encephalitis Info Page
More helpful information about encephalitis, course, features, outcomes.