Subacute sclerosing panencephalitis: an update
Version of Record online: 15 JUN 2010
© The Authors. Journal compilation © Mac Keith Press 2010
Developmental Medicine & Child Neurology
Volume 52, Issue 10, pages 901–907, October 2010
How to Cite
GUTIERREZ, J., ISSACSON, R. S. and KOPPEL, B. S. (2010), Subacute sclerosing panencephalitis: an update. Developmental Medicine & Child Neurology, 52: 901–907. doi: 10.1111/j.1469-8749.2010.03717.x
- Issue online: 6 SEP 2010
- Version of Record online: 15 JUN 2010
- Accepted for publication 22nd April 2010. Published online 15th June 2010.
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.