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[Human prion diseases: current issues].

N N Zavadenko1, G Sh Khondkaryan1, R Ts Bembeeva1

  • 1Pirogov Russian National Research Medical University of the Russian Federation Ministry of Health, Moscow, Russia.

Zhurnal Nevrologii I Psikhiatrii Imeni S.S. Korsakova
|July 25, 2018
PubMed
Summary
This summary is machine-generated.

Prion diseases, a group of neurodegenerative conditions, cause dementia and movement issues. Prevention is key due to a lack of effective treatments for these transmissible spongiform encephalopathies.

Keywords:
biopreparationsdiagnosispathogenesispreventionprion diseasessafetysymptoms

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Area of Science:

  • Neurology
  • Pathology
  • Infectious Diseases

Background:

  • Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative disorders.
  • They are characterized by progressive dementia and movement disorders.
  • Pathogenesis occurs through direct contamination, genetic, or sporadic forms.

Purpose of the Study:

  • To discuss the clinical characteristics of various prion diseases.
  • To explore the molecular-genetic aspects of prion disease pathogenesis.
  • To review current diagnostic approaches for prion diseases.

Main Methods:

  • Literature review of clinical presentations.
  • Analysis of molecular-genetic factors in pathogenesis.
  • Evaluation of current diagnostic strategies.

Main Results:

  • Identified clinical forms include Creutzfeldt-Jakob disease, variant CJD, Gerstmann-Straussler-Scheinker disease, fatal insomnia, kuru, and others.
  • Discussed pathogenesis variants: direct contamination, genetic, and sporadic.
  • Highlighted the importance of diagnostic approaches.

Conclusions:

  • Effective treatments for prion diseases are currently lacking.
  • Prevention of alimentary (contaminated meat) and iatrogenic (biopreparations) infections is crucial.
  • Ensuring biopreparation safety through modern manufacturing and international standards is vital.