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Subacute sclerosing panencephalitis
Abstract:
Subacute sclerosing panencephalitis (SSPE) is now considered to be caused by measles virus. There are four diagnostic criteria, namely the clinical picture, a characteristic EEG, serology of serum and CSF and brain histology. A register of cases in the U.K. has been kept since 1971, and up to September 1977, ninety-six patients have been reported. The male/female ratio is 2 : 1. The disease most commonly affects children between the ages of nine and eleven years who usually have had measles at a very early age. The average delay between the measles infection and onset of SSPE was 6.8 years and of the thirty-four patients known to have died the average survival times was 1.2 years. There are still many questions about the pathogenesis and epidemiology of SSPE that have yet to be answered.
Insights
Subacute sclerosing panencephalitis (SSPE) is a rare neurological disorder caused by measles virus. This study reports on 96 UK cases, noting a 6.8-year delay from measles infection to SSPE onset.
Area of Science:
- Neurology
- Virology
- Epidemiology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disease.
- SSPE is now understood to be caused by the measles virus.
Purpose of the Study:
- To report on cases of SSPE in the UK.
- To analyze the epidemiology and clinical characteristics of SSPE.
Main Methods:
- Case registration in the UK since 1971.
- Analysis of diagnostic criteria including clinical presentation, EEG, serology, and brain histology.
- Review of patient demographics, disease onset, and survival times.
Main Results:
- Ninety-six SSPE cases were reported in the UK up to September 1977.
- The male to female ratio was 2:1.
- The disease predominantly affected children aged 9-11, with an average delay of 6.8 years between measles infection and SSPE onset. Average survival post-diagnosis was 1.2 years for deceased patients.
Conclusions:
- SSPE is a significant neurological complication of measles infection.
- Further research is needed to understand the pathogenesis and epidemiology of SSPE.