Subacute sclerosing panencephalitis
SSPE; Subacute sclerosing leukoencephalitis; Dawson encephalitis; Measles - SSPE; Rubeola - SSPESubacute sclerosing panencephalitis (SSPE) is a progressive, disabling, and deadly brain disorder related to measles (rubeola) infection.
Measles
Measles is a very contagious (easily spread) illness caused by a virus.
Read Article Now Book Mark ArticleThe disease develops many years after the measles infection.
Causes
Normally, the measles virus does not cause brain damage. However, an abnormal immune response to measles or, possibly, certain mutant forms of the virus may cause severe illness and death. This response leads to brain inflammation (swelling and irritation) that may last for years.
SSPE has been reported in all parts of the world, but in western countries it is a rare disease.
Very few cases are seen in the United States since the nationwide measles vaccination program began. SSPE tends to occur several years after a person has measles, even though the person seems to have fully recovered from the illness. Males are more often affected than females. The disease generally occurs in children and adolescents.
Symptoms
Symptoms of SSPE occur in four general stages. With each stage, the symptoms are worse than the stage before:
- Stage I: There may be personality changes, mood swings, or depression. Fever and headache may also be present. This stage may last up to 6 months.
- Stage II: There may be uncontrolled movement problems including jerking and muscle spasms. Other symptoms that may occur in this stage are loss of vision, dementia, and seizures.
Uncontrolled movement problems
Uncontrollable movements include many types of movements that you cannot control. They can affect the arms, legs, face, neck, or other parts of the ...
Read Article Now Book Mark Article - Stage III: Jerking movements are replaced by writhing (twisting) movements and rigidity. Death may occur from complications.
- Stage IV: Areas of the brain that control breathing, heart rate, and blood pressure are damaged. This leads to coma and then death.
Exams and Tests
There may be a history of measles in an unvaccinated child. A physical examination may reveal:
- Damage to the optic nerve, which is responsible for sight
- Damage to the retina, the part of the eye that receives light
- Muscle twitching
- Poor performance on motor (movement) coordination tests
The following tests may be performed:
- Electroencephalogram (EEG)
Electroencephalogram
An electroencephalogram (EEG) is a test to measure the electrical activity of the brain.
Read Article Now Book Mark Article - Brain MRI
Brain MRI
A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...
Read Article Now Book Mark Article - Serum antibody titer to look for signs of previous measles infection
Antibody titer
Antibody titer is a laboratory test that measures the level of antibodies in a blood sample.
Read Article Now Book Mark Article - Spinal tap
Spinal tap
Cerebrospinal fluid (CSF) collection is a test to look at the fluid that surrounds the brain and spinal cord. CSF acts as a cushion, protecting the b...
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Treatment
No cure for SSPE exists. Treatment is generally aimed at controlling symptoms. Certain antiviral medicines and medicines that boost the immune system may be tried to slow the progression of the disease.
Support Groups
The following resources can provide more information on SSPE:
- National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/health-information/disorders/encephalitis?search-term=Subacute%20Sclerosing%20Panencephalitis
- National Organization for Rare Disorders -- rarediseases.org/rare-diseases/subacute-sclerosing-panencephalitis/
Outlook (Prognosis)
SSPE is always fatal. People with this disease die 1 to 3 years after diagnosis. Some people may survive longer.
When to Contact a Medical Professional
Contact your health care provider if your child has not completed their scheduled vaccines. The measles vaccine is included in the MMR vaccine.
Prevention
Immunization against measles is the only known prevention for SSPE. The measles vaccine has been highly effective in reducing the number of affected children.
Measles vaccine
All content below is taken in its entirety from the CDC MMR (Measles, Mumps, & Rubella) Vaccine Information Statement (VIS): www. cdc. gov/vaccines/h...
Read Article Now Book Mark ArticleMeasles immunization should be done according to the recommended American Academy of Pediatrics and Centers for Disease Control schedule.
References
Gershon AA. Measles virus (rubeola). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 160.
Mason WH, Gans HA. Measles. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 273.
Review Date: 7/26/2022
Reviewed By: Evelyn O. Berman, MD, Assistant Professor of Neurology and Pediatrics at University of Rochester, Rochester, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.