Epidemiological data on subacute sclerosing panencephalitis (SSPE) in Germany

Objectives: Subacute sclerosing panencephalitis (SSPE) is a rare, uniformly fatal complication of infection with the measles wildtype virus. It occurs several years after measles virus infection, with a frequency of about one in 10.000 to 100.000, and affects mainly children and young adults. Measles vaccine strains have never been shown to cause SSPE. Not much is known about the current SSPE situation in Germany

Material and Methods: In a retrospective study, SSPE cases were identified from the samples received by the virological diagnostic laboratory for the period from 1988 to 2004. Demographic data, results of the virological studies as well as the available clinical data of these patients were collected and evaluated.

Results: For the period from 1988 to 2004, SSPE was diagnosed in 120 patients from Germany and in 11 patients from Austria. The mean age of the patients at the time of diagnosis was 11.7 years (median 10 years; range 2–34 years). 83% of the patients were younger than 18 years. The male to female ratio was 1.6. For 53 cases, information about a previous wildtype measles infection was available. The mean age at the time of measles infection was 1.8 years (median 1 year; range 2 months –13 years). The main diagnostic test results were a strong intrathecal synthesis of total and measles virus specific IgG in the CSF.

Conclusions: In 34% of the SSPE patients in this study, infection with wildtype measles virus occurred during the first year of life. These cases would not have been prevented by a measles vaccination according to the current German recommendations. In a further 34% of the patients, measles infection occurred in the second year of life. Thus, both the individual vaccination at the earliest time point possible and a general increase of the measles vaccination rates are necessary to eliminate the deadly disease SSPE in Germany.