Acta Neuropathologica

, Volume 120, Issue 6, pp 821–823 | Cite as

Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, α synuclein and Aβ pathology

  • Mark W. Head
  • Suzanne Lowrie
  • Gurjit Chohan
  • Richard Knight
  • David J. Scoones
  • James W. Ironside
Correspondence

Keywords

Protease-sensitive prionopathy Creutzfeldt-Jakob disease Prion protein PRNP codon 129 Alzheimer’s disease Lewy body disease 

References

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    Gambetti P, Dong Z, Yuan J et al (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 63:697–708CrossRefPubMedGoogle Scholar
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    Head MW, Knight R, Zeidler M, Yull H, Barlow A, Ironside JW (2009) A case of protease-sensitive prionopathy in a patient in the United Kingdom. Neuropathol Appl Neurobiol 35:628–632CrossRefPubMedGoogle Scholar
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    Jansen C, Head MW, van Gool WA et al (2010) The first case of protease-sensitive prionopathy (PSPr) in the Netherlands: a patient with an unusual GSS-like clinical phenotype. J Neurol Neurosurg Psychiatry 81:1052–1055CrossRefPubMedGoogle Scholar
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    Zou W-Q, Puoti G, Xiao X et al (2010) Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol 68:162–172CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Mark W. Head
    • 1
  • Suzanne Lowrie
    • 1
  • Gurjit Chohan
    • 1
  • Richard Knight
    • 1
  • David J. Scoones
    • 2
  • James W. Ironside
    • 1
    • 3
  1. 1.National CJD Surveillance Unit, School of Molecular and Clinical MedicineThe University of EdinburghEdinburghUK
  2. 2.Neuropathology DepartmentThe James Cook University HospitalMiddlesbroughUK
  3. 3.National CJD Surveillance Unit, Bryan Matthews Building, Western General HospitalThe University of EdinburghEdinburghUK

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