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Measles Sclerosing Subacute PanEncephalitis (SSPE), an intriguing and ever-present disease: Data, assumptions and new perspectives

Authors :
A. Gelot
Pierre Lebon
Jean-Laurent Casanova
Shen-Ying Zhang
J.-J. Hauw
Source :
Revue Neurologique. 177:1059-1068
Publication Year :
2021
Publisher :
Elsevier BV, 2021.

Abstract

Background Subacute sclerosing panencephalitis (SSPE) is a rare, non-treatable and fatal neurological complication of measles , still present due to the return of the epidemic linked to the loosening of vaccination policies. Its mechanism remains unexplained. Objective The main objective was to investigate explanatory variables relating to the risk of developing SSPE and its pathophysiology . Methods Literature analysis was focused on different varieties of SSPE: perinatal forms, short-incubation forms similar to acute measles inclusion body encephalitis (MIBE), rapidly evolving forms, forms occurring in the immunosuppressed, adult forms, and family forms. In addition, several studies on the parameters of innate immunity and interferon responses of patients were analyzed. Results Two main data were highlighted: a relationship between the so-called fulminant forms and the prescription of corticosteroids was established. In familial SSPE, two groups were individualized according to the duration of the latency period, prompting an analysis of patient exomes . Conclusion Treatment with corticosteroids should be banned. Knowledge of the genes involved and epigenetics should be useful for understanding the pathophysiology of SSPE and other late-onset neurological infections with RNA viruses .

Details

ISSN :
00353787
Volume :
177
Database :
OpenAIRE
Journal :
Revue Neurologique
Accession number :
edsair.doi.dedup.....6966082ca09eab7c29ada36c81b770a3
Full Text :
https://doi.org/10.1016/j.neurol.2021.02.387