Subacute sclerosing panencephalitis
Subacute sclerosing panencephalitis (SSPE) is a rare and chronic condition of the central nervous system that can develop up to ten years after a person has contracted measles. This progressive disorder is characterized by inflammation of the brain and subsequent nerve cell death, leading to severe neurological decline. The underlying cause of SSPE is an altered form of the measles virus, and factors that increase the risk include early childhood infection and lack of vaccination against measles. Children aged five to fifteen, as well as certain ethnic groups, are particularly vulnerable.
Symptoms of SSPE can manifest as abnormal behavior, irritability, memory loss, seizures, and progressive loss of motor functions, among others. Diagnosis typically involves a thorough medical history, physical examination, and various imaging and blood tests. While there is no cure for SSPE, treatment focuses on supportive care and may include medications to manage symptoms. Preventative measures hinge on vaccination against measles, ideally administered in early childhood, to reduce the likelihood of developing this devastating condition.
Subacute sclerosing panencephalitis
- ANATOMY OR SYSTEM AFFECTED: Brain, central nervous system, spinal cord
- ALSO KNOWN AS: Dawson disease
Definition
Subacute sclerosing panencephalitis (SSPE) is a rare, chronic central nervous system (brain and spine) condition that occurs up to ten years after getting the measles. It usually results in progressive deterioration from inflammation of the brain and nerve cell death. When left untreated, SSPE almost always leads to death.
Causes
SSPE is caused by an altered form of the measles virus. It occurs anywhere from two to ten years after contracting measles.
Risk Factors
Two factors that are thought to increase the risk of SSPE are infection in infancy and not being vaccinated against measles. Persons at higher risk for SSPE are children ages five to fifteen years, males, and persons of certain ethnic origins. Arabs and Sephardic Jews have an incidence that is six times higher than Ashkenazi Jews, and Caucasians have a four-fold higher incidence than African Americans in the United States.
Symptoms
Symptoms of SSPE include abnormal behavior, irritability, intellectual deterioration, memory loss, involuntary movements, seizures, an inability to walk, speech impairment with poor comprehension, difficulty swallowing, blindness, muteness, and coma.
Screening and Diagnosis
A doctor will ask about symptoms and medical history and will perform a physical exam. Other tests may include blood tests to look for the measles antibody; an electrocardiogram (ECG, EKG) to record the heart’s activity by measuring electrical currents through the heart muscle; a magnetic resonance imaging (MRI) scan (a scan that uses radio waves and a powerful magnet to produce detailed computer images), and a computed tomography (CT) scan (a detailed X-ray picture that identifies abnormalities of fine tissue structure).
Treatment and Therapy
A doctor should be consulted about the best treatment plan. Treatment options include supportive therapy. With advanced disease, tube feedings and nursing care may be necessary. Also, anticonvulsant medications can reduce some symptoms of SSPE. There is some evidence that certain medications (such as inosine pranobex, interferon alpha, interferon beta, and ribavirin) may help stabilize the disease, delay its progression, or both.
Prevention and Outcomes
The best way to prevent SSPE is to avoid contracting measles. One can do this by getting the measles vaccine (usually given at twelve to fifteen months of age and again at four to six or eleven to twelve years of age). Persons who are not vaccinated should avoid contact with people who are infected with measles until all the infected person’s symptoms are gone.
Bibliography
Campbell, H., et al. “Review of the Effect of Measles Vaccination on the Epidemiology of SSPE.” International Journal of Epidemiology 36 (2007): 1134-1148.
Daube, Jasper R., ed. Clinical Neurophysiology. 3d ed. New York: Oxford University Press, 2009.
EBSCO Publishing. DynaMed: Subacute Sclerosing Panencephalitis (SSPE). Available through http://www.ebscohost.com/dynamed.
‗‗‗‗‗‗‗. Health Library: Measles. Available through http://www.ebscohost.com.
Solomon, T., and R. Knee. “Subacute Sclerosing Panencephalitis.” In MedLink Neurology, edited by S. Gilman. San Diego, Calif.: MedLink, 2003.
"Subacute Sclerosing Panencephalitis." Radiopaedia, 25 Jan. 2025, radiopaedia.org/articles/subacute-sclerosing-panencephalitis-1?lang=us. Accessed 4 Feb. 2025.
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