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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Subacute sclerosing panencephalitis: an update
Jose Gutierrez1, Richard S Issacson, Barbara S Koppel
1Department of Neurology, Miller School of Medicine, University of Miami, Miami, FL 33136, USA. drjosegc@hotmail.com
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.
Insights
Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurological condition following measles infection. It disproportionately affects unvaccinated populations, leading to progressive disability and death within five years for most patients.
Area of Science:
- Neurology
- Virology
- Immunology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a chronic, progressive encephalitis linked to measles virus infection.
- Disease prevalence is higher in areas with low measles vaccination rates.
- The exact pathophysiology involves a dysregulated immune response allowing persistent viral infection of neurons.
Purpose of the Study:
- To summarize the current understanding of SSPE's epidemiology, pathophysiology, clinical presentation, diagnosis, and management.
- To highlight the disease's relentless progression and poor prognosis despite treatment efforts.
Main Methods:
- Clinical case reviews and analysis of existing literature on SSPE.
- Diagnostic criteria including clinical presentation, EEG findings, brain imaging, and serological evidence of measles infection.
- Review of treatment trials and outcomes for SSPE.
Main Results:
- SSPE manifests years after initial measles infection, with prominent psychiatric and neurological symptoms.
- Diagnosis relies on a combination of clinical, electrophysiological, imaging, and immunological findings.
- While treatments aim to manage seizures and complications, SSPE shows a grim prognosis, with 95% mortality within 5 years.
Conclusions:
- SSPE remains a devastating complication of measles infection, particularly in under-vaccinated communities.
- Effective prevention through vaccination is crucial.
- Further research into pathophysiology and novel treatments is warranted, though current options offer limited impact on the disease's fatal course.
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