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A Practical Primer on Prion Pathology.

Brian S Appleby1,2, Daniel D Rhoads1,3, Karin Mente4

  • 1National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio.

Journal of Neuropathology and Experimental Neurology
|April 3, 2018
PubMed
Summary
This summary is machine-generated.

Prion diseases are rare, transmissible neurodegenerative disorders caused by misfolded proteins. This primer aids diagnosis and surveillance for prion diseases, including zoonotic transmission, to protect public health.

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Area of Science:

  • Neuropathology
  • Infectious Diseases
  • Rare Diseases

Background:

  • Prion diseases are transmissible neurodegenerative disorders.
  • Misfolded proteins propagate, causing cellular damage.
  • Rare disease status limits clinical and pathological experience.

Purpose of the Study:

  • To provide a primer on prion disease diagnosis and pathology.
  • To aid the Centers for Disease Control and Prevention (CDC) in surveillance.
  • To facilitate early detection and prevent zoonotic transmission.

Main Methods:

  • Assembled accumulated experience from a national surveillance center.
  • Focused on clinical and pathological manifestations.
  • Emphasized disease transmissibility within and between species.

Main Results:

  • The primer aims to improve neuropathological community's familiarity with prion diseases.
  • Enhanced diagnostic capabilities are expected.
  • Improved surveillance for zoonotic transmission is anticipated.

Conclusions:

  • This primer will enhance the neuropathological community's ability to diagnose and manage prion diseases.
  • It supports the CDC's mission to "save lives and protect people."
  • Increased awareness and expertise are crucial for controlling prion disease spread.