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What

Subacute sclerosing panencephalitis (SSPE) is a progressive disease of the brain caused by an unusual response to the measles virus. Following a measles infection, the virus may rarely persist within the brain in an inactive state. The virus may then reactivate several years later to cause SSPE, a swelling of the brain with associated mental and physical deterioration.

Who

SSPE affects primarily children and adolescents. It occurs rarely in the United States in fewer than one per one million children, with less than 10 new cases each year. SSPE is not inherited, and boys are affected more often than girls. Most children with SSPE have a measles infection before two years of age, with the first signs of SSPE presenting eight to ten years later. The incidence of SSPE among non-immunized children is 100-200 times higher than among those who have been immunized effectively. Measles vaccine does not cause SSPE.

Signs and Symptoms

A child with SSPE will initially experience a change in behavior, irritability, memory loss, and difficulty with learning and schoolwork. Subsequently, an increase in neurological damage results in additional, more obvious symptoms, including involuntary spasms and seizures. Eventually, further mental deterioration occurs, with associated impairment in speech and comprehension. A child will become unable to walk and may experience blindness.

Possible Causes

It is unclear why some children develop SSPE following a measles infection while most do not. Indeed, the mechanism by which the measles virus persists in the brains of children with SSPE is also poorly understood.

Diagnosis

The diagnosis of SSPE is considered clinically in a child with a previous measles infection who is experiencing mental deterioration and spasms. To confirm the diagnosis, the blood and spinal fluid are tested to determine if antibodies to the measles virus are present at high levels. Sometimes an EEG, or brain wave test, and an MRI are also conducted to detect characteristic abnormalities.

Treatment

Supportive care is important in children with SSPE, through maintenance of nutritional status and physical therapy. Medications are used to prevent or control seizures. Currently, there is no adequate therapy available for the treatment of SSPE; however, some medications have been shown in certain studies to improve survival and decrease disability. Over the past decade, isoprinosine (Inosiplex), an antiviral drug which activates the body’s immune system against the measles virus, has been used with variable success. The effectiveness of other medications, including interferon alpha and ribavirin, is controversial. Vitamin A should not be used as a treatment of SSPE. Further research on SSPE is ongoing. By far, the most effective means of preventing SSPE is through immunization of children against measles.

Prognosis

While for most children the disease is fatal within one to three years after onset (usually due to pneumonia), spontaneous remission of SSPE has been reported occasionally.

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

Encephalitis Information Resource
This site provides general information about SSPE and recounts one family’s experience with the disease. It also has a link to ongoing research.

Institute of Child Health
This site provides families with an excellent description of disease progression and suggestions for supportive care. It also has some information about available support and ongoing research.

National Institute of Neurological of Disorders and Stroke
Well written information on SSPE.

Google Search for Subacute Sclerosing Panencephalitis (SSPE)

References and Sources

Behrman RE, Kliegman R, Jenson HB. Nelson Textbook of Pediatrics: 16th Edition. Bonthius DJ, Stanek N, Grose C. Subacute Sclerosing Panencephalitis, a Measles Complication, in an Internationally Adopted Child. Emerging Infectious Diseases. Volume 6, Number 4, 2000. Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J 78:63-70, 2002. Jenson HB, Baltimore RS. Pediatric Infectious Diseases: Principles and Practice. 2nd Revision Edition, 2002.