Subacute Sclerosing Panencephalitis (SSPE)

Subacute sclerosing panencephalitis Latest News

The Maharashtra state government recently informed the Bombay High Court that it is yet to frame a policy to deal with Subacute Sclerosing Panencephalitis (SSPE), an extremely rare but fatal neurodegenerative disease affecting children that has a fatality rate of 95%.

About Subacute sclerosing panencephalitis

• SSPE, also known as Dawson disease, is a progressive, disabling, and deadly brain disorder related to measles (rubeola) infection.
• The disease develops many years after the measles infection, even though the person seems to have fully recovered from the illness. 
• SSPE has been reported in all parts of the world, but in western countries it is a rare disease.
• Males are more often affected than females. The disease generally occurs in children and adolescents.
• SSPE is a rare condition. It occurs in about 1 per 50,000 cases of natural measles.

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Subacute sclerosing panencephalitis Cause

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

Subacute sclerosing panencephalitis Symptoms

• The first symptoms of SSPE may be poor performance in schoolwork, forgetfulness, temper outbursts, distractibility, sleeplessness, and hallucinations. 
• Sudden muscular jerks of the arms, head, or body may occur. 
• Eventually, seizures may occur, together with abnormal uncontrollable muscle movements. Intellect and speech continue to deteriorate.
• Later, the muscles become increasingly rigid, and swallowing may become difficult. 
• The swallowing difficulty sometimes causes people to choke on their saliva, resulting in pneumonia. People may become blind.
• In the final phases, the body temperature may rise, and the blood pressure and pulse become abnormal.

Subacute sclerosing panencephalitis Treatment

• High mortality rates are associated with SSPE, and there is no cure for SSPE.
• Treatment is generally aimed at regulating symptoms. 
• Certain antiviral medicines and medicines that boost the immune system may be tried to slow the progression of the disease.

Source: HT