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Methods for Acute and Subacute Murine Hindlimb Ischemia
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Subacute sclerosing panencephalitis.

Ravindra Kumar Garg1, Anita Mahadevan2, Hardeep Singh Malhotra2

  • 1Department of Neurology, King George Medical University, Lucknow, India.

Reviews in Medical Virology
|June 26, 2019
PubMed
Summary
This summary is machine-generated.

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive brain disease caused by mutant measles virus, primarily affecting children. Measles vaccination significantly reduces SSPE incidence by preventing measles infection.

Keywords:
chorioretinitisencephalopathymeaslesmyoclonus

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Area of Science:

  • Neurology
  • Virology
  • Immunology

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a devastating neurological sequela of measles virus infection.
  • SSPE incidence has dramatically decreased in high-income nations due to widespread measles vaccination programs.
  • The SSPE-associated measles virus exhibits unique genomic mutations, particularly in the M gene, affecting viral protein structure and function.

Purpose of the Study:

  • To elucidate the pathogenesis of SSPE, focusing on viral mutations and neuronal spread.
  • To review the clinical manifestations, diagnostic methods, and current therapeutic limitations for SSPE.
  • To emphasize the critical role of measles vaccination in SSPE prevention.

Main Methods:

  • Analysis of measles virus genomes from brain tissues of SSPE patients.
  • Review of in vitro studies investigating viral entry and spread mechanisms.
  • Consolidation of clinical, electrophysiological, and neuroimaging findings in SSPE.
  • Evaluation of diagnostic criteria, including cerebrospinal fluid (CSF) measles antibody titers.

Main Results:

  • Mutations in the SSPE measles virus genome, especially in the M gene, impair protein function.
  • Mutations in the F protein may enhance viral fusion and facilitate transneuronal spread.
  • Clinical presentation includes cognitive decline, myoclonus, gait disturbances, vision loss, and chorioretinitis.
  • Characteristic EEG and neuroimaging findings support the diagnosis, with elevated CSF measles antibodies being definitive.

Conclusions:

  • SSPE pathogenesis involves a mutated measles virus with altered protein functions leading to neuronal damage and inflammation.
  • Current treatments lack robust clinical trial evidence, highlighting the need for novel therapeutic strategies like fusion inhibitors.
  • Universal measles vaccination remains the most effective strategy for preventing SSPE.