Subacute sclerosing panencephalitis: a very deadly result of measles virus

Subacute sclerosing panencephalitis is an emerging infectious disease, according to the CDC. It is a progressive neurological disorder of children and young adults that affects the central nervous system. It is a slow, but persistent, viral infection caused by defective measles virus.

It is also known as Panencephalitis, Subacute Sclerosing, SSPE, Dawson’s Disease, and Dawson’s Encephalitis.

SSPE has been reported from all parts of the world, but it is considered a rare disease in developed countries, with fewer than 10 cases per year reported in the United States. The incidence of SSPE declined by at least 90 percent in countries that have practiced widespread immunization with measles vaccine. The incidence of SSPE is still high in developing countries such as India and Eastern Europe.

It is reported that 1 in 100,000 people infected with measles will develop SSPE. There is a higher incidence among males than females, with a ratio of 3-1. Most children with SSPE have a history of measles infection at an early age, usually younger than 2 years, followed by a period of at least 6 to 8 years before neurological symptoms begin.

Despite the long interval between the measles infection and the onset of SSPE, researchers think that the infection of the brain occurs soon after the primary bout with measles and progresses slowly. Why it persists and progresses still isn’t clear.

Initial symptoms may include memory loss, irritability, seizures, involuntary muscle movements, and/or behavioral changes, leading to neurological deterioration. Some people may become blind.

In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Death is usually the result of fever, heart failure, or the brain’s inability to continue controlling the autonomic nervous system.

Most individuals with SSPE will die within 1 to 3 years of diagnosis. In a small percentage of people, the disease will progress rapidly, leading to death over a short course within three months of diagnosis. Another small group will have a chronic, slowly progressive form, some with relapses and remissions. Approximately 5% may experience spontaneous long term improvement and regain lost function.  Prevention, in the form of measles vaccination, is the only real “cure” for SSPE.

Clinical trials of antiviral (isoprinosine and ribavirin) and immunomodulatory (interferon alpha) drugs have suggested that these types of therapies given alone or in combination halt the progression of the disease and can prolong life, but their long-term effects on individuals, and eventual outcome, are unknown. Good nursing care is the most important aspect of treatment for SSPE, along with anticonvulsant and antispasmodic drugs when needed.

On the CDC website you can read a case study of a healthy 13-year-old boy who had spent the first 4.5 years of his life in an orphanage in Thailand before adoption by an American couple. He later became ill with subacute sclerosing panencephalitis and died several months later. The boy had most likely contracted wild-type measles in Thailand. Measles complications are a risk in international adoptions.

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