Subacute sclerosing panencephalitis

K. J. Lizarraga, J. Gutierrez, C. Singer

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Subacute sclerosing panencephalitis (SSPE) is a rare childhood disorder caused by persistent measles virus (MV) infection of the brain that slowly evolves into chronic demyelination and neurodegeneration. Mutant MVs able to spread from cell-to-cell while evading cell-mediated immunity in the brain lead to SSPE 6-8 years after the primary infection. Patients present with behavioral changes followed by progressive myoclonus, seizures, cognitive decline, altered consciousness and death within 1-3 years. Ancillary diagnostic findings include periodic complexes on electroencephalography and intrathecal anti-MV antibodies. SSPE remains fatal despite therapeutic trials. Yet, blockage of MV fusion mechanisms is a promising approach.

Original languageEnglish (US)
Title of host publicationThe Curated Reference Collection in Neuroscience and Biobehavioral Psychology
PublisherElsevier Science Ltd.
Pages187-189
Number of pages3
ISBN (Electronic)9780128093245
DOIs
StatePublished - Jan 1 2016
Externally publishedYes

Keywords

  • Childhood dementia
  • Childhood epilepsy
  • Childhood myoclonus
  • Chronic brain infection
  • Chronic infectious demyelination
  • Measles
  • Measles fusion blockers
  • Mutant measles virus
  • Periodic complexes
  • Progressive neurodegeneration
  • Subacute sclerosing panencephalitis

ASJC Scopus subject areas

  • Medicine(all)

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