Measles Sclerosing Subacute PanEncephalitis (SSPE), an intriguing and ever-present disease: Data, assumptions and new perspectives - 27/06/21

Doi : 10.1016/j.neurol.2021.02.387

P. Lebon ^a,⁎ , A. Gelot ^b, S.-Y. Zhang ^c, J.-L. Casanova ^d,^e, J.-J. Hauw ^f
^a Faculté de médecine, Paris Descartes, université de Paris, 15, rue de l’école de Médecine, 75006 Paris, France
^b Service de neuropathologie, Hôpital Trousseau, 75012 Paris, France
^c Saint-Giles Laboratory of Human Genetics of Infectious Diseases, Rockefeller Branch, The Rockefeller University, 10065 New York, NY, USA
^d Inserm UMR 1163, Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Necker Hospital for Sick Children, 75015 Paris, France
^e Imagine Institute, Paris Descartes University, 75015 Paris, France
^f Académie nationale de médecine, 75006 Paris, France

^⁎Corresponding author.

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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.

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Keywords : Subacute encephalitis, Measles virus, Interferon, Genes, COVID-19