Subacute Sclerosing Panencephalitis (SSPE)

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NINDS: ⁵² 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, but persistent, viral infection caused by defective measles virus. 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. There is a higher incidence among males than females (male/female: 3/1). Most youngsters with SSPE have a history of measles infection at an early age, usually younger than 2 years, followed by a latent period of 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. The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the head, trunk or limbs called myoclonic jerks. Seizures may also occur. 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.

MalaCards based summary: Subacute Sclerosing Panencephalitis, also known as sspe, is related to measles and chorioretinitis. An important gene associated with Subacute Sclerosing Panencephalitis is CD46 (CD46 Molecule), and among its related pathways/superpathways are Innate Immune System and MIF Mediated Glucocorticoid Regulation. Affiliated tissues include brain, cortex and heart, and related phenotypes are infectious encephalitis and immune system

GARD: ¹⁹ Subacute sclerosing panencephalitis (SSPE) a rare condition that is caused by a measles infection acquired earlier in life. Affected people may initially experience behavioral changes, dementia, and disturbances in motor function. Ultimately, many people with SSPE succumb to fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system. It is unclear why some people develop SSPE after they have seemingly recovered from the measles while others do not.

OMIM®: ⁵⁷ Subacute sclerosing panencephalitis (SSPE) is a fatal neurodegenerative disease caused by persistent central nervous system infection with the measles virus (summary by Torisu et al., 2004). (260470) (Updated 08-Dec-2022)

Orphanet: ⁵⁸ A chronic progressive encephalitis that develops a few years after measles infection and presents with a demyelination of the cerebral cortex.

Disease Ontology: ¹¹ A viral infectious disease that results in infection located in brain and that has material basis in Measles virus which is immune resistant.

Wikipedia: ⁷⁵ Subacute sclerosing panencephalitis (SSPE)—also known as Dawson disease—is a rare form of chronic,... more...

Clinical features from OMIM®:

Search NIH Clinical Center for Subacute Sclerosing Panencephalitis