[Measles subacute sclerosing panencephalitis (SSPE): A still present and still mysterious fatal disease. History,

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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis

Published on: July 4, 2007

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Pierre Lebon1, Antoinette Gelot2, Shen-Ying Zhang3

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

Medecine Sciences : M/S
|June 29, 2022
PubMed

Insights

Subacute sclerosing panencephalitis (SSPE) persists due to low measles vaccination rates. Research explores SSPE

Area of Science:

  • Neurology
  • Immunology
  • Virology

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a severe, late neurological complication of measles infection.
  • Insufficient measles vaccination rates contribute to the continued presence of SSPE, particularly during outbreaks.
  • Understanding SSPE's pathophysiology is crucial for developing effective interventions.

Purpose of the Study:

  • To review the historical context, diagnostic criteria, and pathophysiology of SSPE.
  • To explore various clinical presentations and potential genetic influences on SSPE.
  • To identify genetic factors contributing to SSPE and other post-viral neurological disorders.

Main Methods:

  • Historical review of SSPE literature.
  • Analysis of innate immunity and interferon responses in SSPE patients.
  • Formulation of hypotheses based on diverse clinical forms of SSPE.
  • Retrospective and prospective exome analysis of patients grouped by latency period.

Main Results:

  • Studies indicate a decrease in cellular immunity and interferon responses in SSPE.
  • Multiple clinical forms of SSPE exist, including congenital, perinatal, rapidly evolving, and adult-onset.
  • Familial cases suggest a potential genetic predisposition.
  • Latency period duration differentiates patient groups for genetic analysis.

Conclusions:

  • SSPE remains a significant public health concern linked to measles vaccination coverage.
  • Genetic factors likely play a role in SSPE development and progression.
  • Identifying causative genes will advance understanding of SSPE and related neurological conditions.

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