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Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study
The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts. To investigate t...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5759292/ https://www.ncbi.nlm.nih.gov/pubmed/29310723 http://dx.doi.org/10.1186/s40478-017-0503-z |
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author | Cali, Ignazio Cohen, Mark L. Haїk, Stéphane Parchi, Piero Giaccone, Giorgio Collins, Steven J. Kofskey, Diane Wang, Han McLean, Catriona A. Brandel, Jean-Philippe Privat, Nicolas Sazdovitch, Véronique Duyckaerts, Charles Kitamoto, Tetsuyuki Belay, Ermias D. Maddox, Ryan A. Tagliavini, Fabrizio Pocchiari, Maurizio Leschek, Ellen Appleby, Brian S. Safar, Jiri G. Schonberger, Lawrence B. Gambetti, Pierluigi |
author_facet | Cali, Ignazio Cohen, Mark L. Haїk, Stéphane Parchi, Piero Giaccone, Giorgio Collins, Steven J. Kofskey, Diane Wang, Han McLean, Catriona A. Brandel, Jean-Philippe Privat, Nicolas Sazdovitch, Véronique Duyckaerts, Charles Kitamoto, Tetsuyuki Belay, Ermias D. Maddox, Ryan A. Tagliavini, Fabrizio Pocchiari, Maurizio Leschek, Ellen Appleby, Brian S. Safar, Jiri G. Schonberger, Lawrence B. Gambetti, Pierluigi |
author_sort | Cali, Ignazio |
collection | PubMed |
description | The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts. To investigate this phenomenon further, a cohort of 27 iCJD cases – 21 with adequate number of histopathological sections – originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrP(Sc)), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND). Cases of iCJD and sCJD shared similar profiles of proteinase K-resistant PrP(Sc) with the exception of iCJD harboring the “MMi” phenotype. Cerebral amyloid angiopathy (CAA), either associated with, or free of, Thioflavin S-positive amyloid core plaques (CP), was observed in 52% of 21 cases of iCJD, which comprised 37.5% and 61.5% of the cases of GH- and DM-iCJD, respectively. If only cases younger than 54 years were considered, Aβ pathology affected 41%, 2% and 0% of iCJD, sCJD and non-ND, respectively. Despite the patients’ younger age CAA was more severe in iCJD than sCJD, while Aβ diffuse plaques, in absence of Aβ CP, populated one third of sCJD. Aβ pathology was by far most severe in AD. Tau pathology was scanty in iCJD and sCJD. In conclusion, (i) despite the divergences in the use of cadaveric GH and DM products, our cases combined with previous studies showed remarkably similar iCJD and Aβ phenotypes indicating that the occurrence of Aβ pathology in iCJD is a widespread phenomenon, (ii) CAA emerges as the hallmark of the Aβ phenotype in iCJD since it is observed in nearly 90% of all iCJD with Aβ pathology reported to date including ours, and it is shared by GH- and DM-iCJD, (iii) although the contributions to Aβ pathology of other factors, including GH deficiency, cannot be discounted, our findings increase the mounting evidence that this pathology is acquired by a mechanism resembling that of prion diseases. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s40478-017-0503-z) contains supplementary material, which is available to authorized users. |
format | Online Article Text |
id | pubmed-5759292 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-57592922018-01-10 Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study Cali, Ignazio Cohen, Mark L. Haїk, Stéphane Parchi, Piero Giaccone, Giorgio Collins, Steven J. Kofskey, Diane Wang, Han McLean, Catriona A. Brandel, Jean-Philippe Privat, Nicolas Sazdovitch, Véronique Duyckaerts, Charles Kitamoto, Tetsuyuki Belay, Ermias D. Maddox, Ryan A. Tagliavini, Fabrizio Pocchiari, Maurizio Leschek, Ellen Appleby, Brian S. Safar, Jiri G. Schonberger, Lawrence B. Gambetti, Pierluigi Acta Neuropathol Commun Research The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts. To investigate this phenomenon further, a cohort of 27 iCJD cases – 21 with adequate number of histopathological sections – originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrP(Sc)), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND). Cases of iCJD and sCJD shared similar profiles of proteinase K-resistant PrP(Sc) with the exception of iCJD harboring the “MMi” phenotype. Cerebral amyloid angiopathy (CAA), either associated with, or free of, Thioflavin S-positive amyloid core plaques (CP), was observed in 52% of 21 cases of iCJD, which comprised 37.5% and 61.5% of the cases of GH- and DM-iCJD, respectively. If only cases younger than 54 years were considered, Aβ pathology affected 41%, 2% and 0% of iCJD, sCJD and non-ND, respectively. Despite the patients’ younger age CAA was more severe in iCJD than sCJD, while Aβ diffuse plaques, in absence of Aβ CP, populated one third of sCJD. Aβ pathology was by far most severe in AD. Tau pathology was scanty in iCJD and sCJD. In conclusion, (i) despite the divergences in the use of cadaveric GH and DM products, our cases combined with previous studies showed remarkably similar iCJD and Aβ phenotypes indicating that the occurrence of Aβ pathology in iCJD is a widespread phenomenon, (ii) CAA emerges as the hallmark of the Aβ phenotype in iCJD since it is observed in nearly 90% of all iCJD with Aβ pathology reported to date including ours, and it is shared by GH- and DM-iCJD, (iii) although the contributions to Aβ pathology of other factors, including GH deficiency, cannot be discounted, our findings increase the mounting evidence that this pathology is acquired by a mechanism resembling that of prion diseases. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s40478-017-0503-z) contains supplementary material, which is available to authorized users. BioMed Central 2018-01-08 /pmc/articles/PMC5759292/ /pubmed/29310723 http://dx.doi.org/10.1186/s40478-017-0503-z Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Cali, Ignazio Cohen, Mark L. Haїk, Stéphane Parchi, Piero Giaccone, Giorgio Collins, Steven J. Kofskey, Diane Wang, Han McLean, Catriona A. Brandel, Jean-Philippe Privat, Nicolas Sazdovitch, Véronique Duyckaerts, Charles Kitamoto, Tetsuyuki Belay, Ermias D. Maddox, Ryan A. Tagliavini, Fabrizio Pocchiari, Maurizio Leschek, Ellen Appleby, Brian S. Safar, Jiri G. Schonberger, Lawrence B. Gambetti, Pierluigi Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study |
title | Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study |
title_full | Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study |
title_fullStr | Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study |
title_full_unstemmed | Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study |
title_short | Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study |
title_sort | iatrogenic creutzfeldt-jakob disease with amyloid-β pathology: an international study |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5759292/ https://www.ncbi.nlm.nih.gov/pubmed/29310723 http://dx.doi.org/10.1186/s40478-017-0503-z |
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