What is subacute sclerosing panencephalitis?
Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of children and young adults that affects the central nervous system (CNS). It is a slow and persistent viral infection related to measles.
SSPE has been reported in all parts of the world, but it is considered a rare disease in more developed countries.
Most children with SSPE have a history of measles infection at an early age, usually younger than age 2, followed by a latent period of six to eight 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.
The initial symptoms of SSPE are subtle and include the following:
- Mild cognitive decline (such as memory loss)
- Changes in behavior (such as irritability)
- Disturbances in motor function, including uncontrollable jerking movements of the head, trunk or limbs called myoclonic jerks
- Seizures may occur
- Some people may go 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.
Currently, there is no cure for SSPE. Prevention in the form of the measles vaccination is the only real "cure" for SSPE.
Good nursing care is the most important aspect of treatment for SSPE, along with anticonvulsant and antispasmodic drugs when needed.
Most individuals with SSPE will die within one to three years of diagnosis. In a small percentage, the disease will progress rapidly, leading to death within three months of diagnosis. Another small group will have a chronic, slowly progressive form, some with relapses and remissions. A very small number may experience spontaneous long-term improvement and regain lost function.
How can I or my loved one help improve care for people with subacute sclerosing panencephalitis?
Consider participating in a clinical trial so clinicians and scientists can learn more about SSPE and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.
All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.
For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with SSPE at Clinicaltrials.gov.
Where can I find more information about subacute sclerosing panencephalitis?
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