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Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases

Lilla Reiniger1, Ilaria Mirabile, Ana Lukic

  • 1Division of Neuropathology, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK. s.brandner@ucl.ac.uk.

Acta Neuropathologica Communications
|November 21, 2013
PubMed
Abstract

Insights

Inherited prion diseases show unique filamentous prion protein deposits in the subcortical white matter. This finding suggests a distinct disease pathway for inherited prion conditions compared to sporadic and acquired forms.

Area of Science:

  • Neuroscience
  • Pathology
  • Genetics

Background:

  • Prion diseases, including sporadic, inherited, and acquired forms, exhibit distinct histological patterns of abnormal prion protein (PrP) deposition.
  • Inherited prion diseases often display unique histological patterns linked to specific mutations.
  • Previous research primarily focused on PrP deposition in the grey matter of the cortex and cerebellum.

Purpose of the Study:

  • To investigate the histological patterns of abnormal prion protein (PrP) deposition in the subcortical white matter of inherited prion diseases.
  • To characterize the morphology and distribution of PrP deposits in inherited prion diseases.

Main Methods:

  • Analysis of 35 cases of inherited prion disease from the UK National Prion Clinic.
  • Utilized immunohistochemistry, immunofluorescence, and electron microscopy techniques.
  • Examined PrP deposition in the subcortical white matter.

Main Results:

  • Filamentous depositions of abnormal PrP were frequently observed in the subcortical white matter of inherited prion diseases.
  • Abundant filamentous PrP deposition was found in myelinated fibres, particularly in cases with N-terminal mutations.
  • Histological findings revealed distinct PrP deposition patterns in the white matter.

Conclusions:

  • The presence of filamentous PrP in the subcortical white matter is a characteristic feature of inherited prion diseases.
  • This distinct deposition pattern suggests a unique pathogenesis for inherited prion diseases compared to sporadic and acquired forms.
  • Further research into white matter PrP deposition may elucidate disease mechanisms.

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