Condition 101 About Subacute Sclerosing Panencephalitis

What is the definition of Subacute Sclerosing Panencephalitis?

Subacute sclerosing panencephalitis (SSPE) is a progressive, disabling, and deadly brain disorder related to measles (rubeola) infection.

The disease develops many years after the measles infection.

What are the alternative names for Subacute Sclerosing Panencephalitis?

SSPE; Subacute sclerosing leukoencephalitis; Dawson encephalitis; Measles - SSPE; Rubeola - SSPE

What are the causes for Subacute Sclerosing Panencephalitis?

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.

What are the symptoms for Subacute Sclerosing Panencephalitis?

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.
  • 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.

What are the current treatments for Subacute Sclerosing Panencephalitis?

No cure for SSPE exists. Treatment is generally aimed at controlling symptoms. Certain antiviral drugs and drugs that boost the immune system may be tried to slow the progression of the disease.

What are the support groups for Subacute Sclerosing Panencephalitis?

The following resources can provide more information on SSPE:

  • National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/Disorders/All-Disorders/Subacute-Sclerosing-Panencephalitis-Information-Page
  • National Organization for Rare Disorders -- rarediseases.org/rare-diseases/subacute-sclerosing-panencephalitis/

What is the outlook (prognosis) for Subacute Sclerosing Panencephalitis?

SSPE is always fatal. People with this disease die 1 to 3 years after diagnosis. Some people may survive longer.

When should I contact a medical professional for Subacute Sclerosing Panencephalitis?

Call your health care provider if your child has not completed their scheduled vaccines. The measles vaccine is included in the MMR vaccine.

How do I prevent Subacute Sclerosing Panencephalitis?

Immunization against measles is the only known prevention for SSPE. The measles vaccine has been highly effective in reducing the numbers of affected children.

Measles 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.

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Latest Advances On Subacute Sclerosing Panencephalitis

  • Condition: Subsegmental Pulmonary Embolism (SSPE) in Patients with Cancer
  • Journal: Blood advances
  • Treatment Used: Anticoagulation Therapy
  • Number of Patients: 206
  • Published —
This study identified the incidence and risk factors of recurrent proximal pulmonary embolism (clot) within 1 subsegmental pulmonary embolism (SSPE) in patients with cancer.
  • Condition: Sclerosing Panencephalitis
  • Journal: Pediatric neurology
  • Treatment Used: Aprepitant
  • Number of Patients: 62
  • Published —
In this study, researchers evaluated the safety and effectiveness of aprepitant for the treatment of sclerosing panencephalitis.

Clinical Trials For Subacute Sclerosing Panencephalitis

Clinical Trial
  • Status: Not yet recruiting
  • Phase: Phase 3
  • Intervention Type: Other, Drug
  • Participants: 1466
  • Start Date: March 2021
STOPping Anticoagulation for Isolated or Incidental Subsegmental Pulmonary Embolism