Subacute sclerosing panencephalitis: an update

Tuesday, 19th of November 2013

[source]Development Medicine and Child Neurology[|source]

SSPE was described first by Dawson in 1934, in an individual with rapidly progressive encephalitis. Later, in 1945, van Bogaert described another individual with the same clinical presentation but in whom the disease exhibited a more gradual course. The disease is so called because it typically develops over a period of less than 9 months (subacute), because of the nature of the pathological lesions (sclerosis), and from the fact that the whole brain is affected (panencephalitis). 

In this article, the authors provide an update that concludes that SSPE continues to be a fatal disease. Fortunately, the article cites declines in incidence as a result of large vaccination campaigns; BUT also argue that the disease still inflicts its burden on thousands of people globally. The paper ends by re-asserting that the best approach to relieve the suffering of SSPE is by measles vaccination, and that there are no data to suggest any significant side-effects of vaccination. More details on this technical update are available at:  http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2010.03717.x/pdf

 Abstract

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.