Subacute sclerosing panencephalitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Subacute sclerosing panencephalitis is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by defective measles virus (which can be a result of a mutation of the virus itself). SSPE is ‘incurable’ but the condition can be managed by medication if treatment is started at an early stage. Much of the work on SSPE has been completed by the NINDS.

Ordinarily, the measles virus does not cause brain damage, but an abnormal immune response to measles or, possibly, certain mutant forms of the virus may cause severe illness and death. This leads to brain inflammation (swelling and irritation) that may last for years.

SSPE is an incredibly rare condition although there is still relatively high incidence in Asia and the Middle East. However, the number of reported cases is declining since the introduction of the measles vaccine — eradication of the measles virus prevents the SSPE mutation and therefore the progression of the disease or even the initial infection itself.

SSPE tends to occur several years after an individual has measles, even though the person seems to have fully recovered from the illness. 1 in 100,000 people infected with measles are at risk.

Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years (6–15 on average), and then gradual, progressive psychoneurological deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma.

No cure for SSPE exists. However, certain antiviral drugs may slow the progression of the disease.

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.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Ordinarily, the measles virus does not cause brain damage, but an abnormal immune response to measles or, possibly, certain mutant forms of the virus may cause severe illness and death. This leads to brain inflammation (swelling and irritation) that may last for years.

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Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

SSPE is an incredibly rare condition although there is still relatively high incidence in Asia and the Middle East. However, the number of reported cases is declining since the introduction of the measles vaccine — eradication of the measles virus prevents the SSPE mutation and therefore the progression of the disease or even the initial infection itself.

The disease generally occurs in children and adolescents.

Males are more often affected than females.

SSPE has been reported in all parts of the world, but in western countries it is considered a rare disease. Fewer than 10 cases per year are reported in the United States, a dramatic decrease that followed the nationwide measles immunization program.

In some countries like India, over 2 cases of SSPE per 100,000 people are reported each year.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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SSPE tends to occur several years after an individual has measles, even though the person seems to have fully recovered from the illness. 1 in 100,000 people infected with measles are at risk.

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Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Prior to death, there may be:

• Behavior changes
• Dementia
• Stupor and coma
• Seizures and subsequent injuries

• Death usually occurs within 3 years.

• If the diagnosis is made during stage 1 of the SSPE infection then it is possible to cure the disease. However, once SSPE progresses to stage 2 then it is universally fatal in all occurrences.

• The standard rate of decline spans anywhere between 1–3 years after the onset of the infection.

• The progression of each stage is unique to the sufferer and cannot be predicted although the pattern or symptoms/signs can be.

• Although the prognosis is bleak for SSPE past stage 1, it should be noted that there is a 5% remission rate — this may be either a full remission or an improvement in condition giving a longer progression period or at least a longer period with the less severe symptoms.

• Regardless of the stage that the infection is at, treatment with inosine pranobex combined with interferon can give up to a 50% remission/improvement rate.

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