SUBACUTE SCLEROSING PANENCEPHALITIS: AN UPDATE

Tuesday, 16th of July 2013

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

SSPE continues to be one of the most debilitating complications of measles virus infections, just as it is a fatal disease. Fortunately, the incidence of SSPE is decreasing as a result of large vaccination campaigns; however, it still inflicts its burden on thousands of people globally. If interested in epidemiology, pathogenesis, pathology, clinical features, diagnosis and treatment options, then read update available at:  http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2010.03717.x/full

 

 

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.