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Prions and Diseases pp 103–119Cite as

The Spectrum of Tau Pathology in Human Prion Disease

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Abstract

Intracellular deposition of hyperphosphorylated tau characterizes tauopathies: there is a spectrum from neuron-predominant through mixed neuronal and glial, to glia-predominant forms. However, tau pathology appears in practically all forms of human prion disease. In addition to the rare cooccurrence of a primary tauopathy with prion disease, tau pathology may associate with prion diseases in distinct patterns: (1) small neuritic profiles correlating with tissue lesioning can be observed in all prion diseases; (2) larger dystrophic neurites may be observed around PrP amyloid plaques; and (3) neurofibrillary degeneration may follow the distribution described by Braak and Braak as Alzheimer-related pathology but might show atypical locations. It may be associated with prominent neuropil threads in subcortical regions in certain mutations with Creutzfeldt–Jakob disease (i.e., E200K mutation). Furthermore, widespread neurofibrillary degeneration in several subcortical, allocortical, and neocortical regions is consistently associated with certain PRNP mutations in Gerstmann–Sträussler–Scheinker disease or PrP cerebral amyloid angiopathy. Other types of tau pathologies include the rare presence of glial tau immunoreactivity. In summary, widespread application of phospho-tau immunostaining has revealed a previously underrecognized spectrum of tau pathologies in human prion diseases. The relation between tau pathology and PrP deposition, and factors influencing its appearance in prion diseases merit further studies.

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Abbreviations

AD:

Alzheimer disease

AGD:

Argyrophilic grain disease

CAA:

Cerebral amyloid angiopathy

CBD:

Corticobasal degeneration

CJD:

Creutzfeldt–Jakob disease

DLB:

Dementia with Lewy bodies

FFI:

Fatal familial insomnia

gCJD:

Genetic CJD

GSK3β:

Glycogen synthase kinase 3β

GSS:

Gerstmann–Sträussler–Scheinker disease

iCJD:

iatrogenic CJD

NDD:

Neurodegenerative disease

NFT:

Neurofibrillary tangle

PD:

Parkinson’s disease

PK:

Proteinase K

PRNP:

Prion protein gene

PrP:

Prion protein

PSP:

Progressive supranuclear palsy

sCJD:

Sporadic CJD

vCJD:

Variant CJD

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Authors and Affiliations

  1. Institute of Neurology, Medical University of Vienna, Austrian Reference Centre for Human Prion Diseases, AKH 4J Währinger Gürtel 18-20, 1097, Vienna, Austria

    Gabor G. Kovacs M.D., Ph.D. & Herbert Budka M.D., M.Sc., Dhc

Authors
  1. Gabor G. Kovacs M.D., Ph.D.
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  2. Herbert Budka M.D., M.Sc., Dhc
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Correspondence to Gabor G. Kovacs M.D., Ph.D. .

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Editors and Affiliations

  1. , Department of Pathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Wen-Quan Zou

  2. , Department of Neuropathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Pierluigi Gambetti

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Kovacs, G.G., Budka, H. (2013). The Spectrum of Tau Pathology in Human Prion Disease. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5338-3_7

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