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

P Lebon1, A Gelot2, S-Y Zhang3

  • 1Faculté de médecine, Paris Descartes, université de Paris, 15, rue de l'école de Médecine, 75006 Paris, France.

Revue Neurologique
|June 30, 2021
PubMed
Abstract

Insights

Corticosteroid treatment should be avoided for subacute sclerosing panencephalitis (SSPE), a fatal measles complication. Understanding SSPE genetics and epigenetics is crucial for explaining its pathophysiology and other viral neurological diseases.

Area of Science:

  • Neurology
  • Immunology
  • Virology
  • Genetics

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurological sequela of measles infection.
  • SSPE incidence is increasing due to declining measles vaccination rates.
  • The underlying mechanisms of SSPE pathogenesis remain poorly understood.

Purpose of the Study:

  • To investigate risk factors associated with SSPE development.
  • To explore the pathophysiology of SSPE.
  • To analyze different clinical presentations and genetic factors in SSPE.

Main Methods:

  • Literature review encompassing diverse SSPE forms (perinatal, acute, rapidly evolving, immunosuppressed, adult, familial).
  • Analysis of studies on innate immunity and interferon responses in SSPE patients.
  • Exome analysis in familial SSPE cases to identify genetic factors.

Main Results:

  • A correlation was identified between fulminant SSPE and corticosteroid administration.
  • Familial SSPE cases were categorized into two groups based on latency period duration.
  • Genetic analysis was prompted by distinct familial SSPE presentations.

Conclusions:

  • Corticosteroid treatment for SSPE should be discontinued.
  • Investigating genetic factors and epigenetics is essential for elucidating SSPE pathophysiology.
  • This knowledge may aid in understanding other late-onset neurological infections caused by RNA viruses.
Keywords:
COVID-19GenesInterferonMeasles virusSubacute encephalitis

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