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Brains with sporadic Creutzfeldt-Jakob disease and copathology showed a prolonged end-stage of disease
  1. Aitzol Miguelez-Rodriguez1,
  2. Jorge Santos-Juanes2,
  3. Ikerne Vicente-Etxenausia3,
  4. Katty Perez de Heredia-Goñi3,
  5. Beatriz Garcia4,
  6. Luis M Quiros4,5,
  7. Laura Lorente-Gea6,
  8. Isabel Guerra-Merino1,3,6,
  9. Jose J Aguirre6,
  10. Ivan Fernandez-Vega2,3,4,6
  1. 1 Faculty of Medicine, University of Basque Country, Vitoria, Spain
  2. 2 Department of Pathology, Hospital Universitario Central de Asturias, Oviedo, Spain
  3. 3 Biobanco Vasco para la Investigación (O+eHun), Brain Bank, Hospital Universitario Araba, Vitoria-Gasteiz, Spain
  4. 4 Scientific Department, Instituto Universitario Fernández-Vega, Oviedo, Spain
  5. 5 Department of Functional Biology, University of Oviedo, Oviedo, Spain
  6. 6 Department of Pathology, Hospital Universitario de Araba-Txagorritxu, Vitoria-Gasteiz, Spain
  1. Correspondence to Dr Ivan Fernandez-Vega, Service of Anatomic Pathology, Hospital Universitario de Araba-Txagorritxu, C/Jose Atxotegui s/n, E-01009, Vitoria-Gasteiz, Alava, Spain; ivan_fernandez_vega{at}


Aims To investigate the expression of major proteins related to primary neurodegenerative diseases and their prognostic significance in brains with Creutzfeldt-Jakob disease (CJD).

Materials and methods Thirty consecutive cases of confirmed CJD during the period 2010–2015 at Basque Brain bank were retrospectively reviewed. Moreover, major neurodegenerative-associated proteins (phosphorylated Tau, 4R tau, 3R tau, alpha-synuclein, TDP43, amyloid beta) were tested. Clinical data were reviewed. Cases were divided according to the presence or absence of copathology. Survival curves were also determined.

Results Copathology was significantly associated with survival in brains with CJD (4.2±1.2 vs 9.2±1.9; P=0.019) and in brains with MM1/MV1 CJD (2.1±1.0 vs 6.7±2.8; P=0.012). Besides, the presence of more than one major neurodegenerative-associated protein was significantly associated with survival (4.2±1.2 vs 10.7±2.6; P=0.017). Thus, univariate analyses further pointed out variables significantly associated with better survival: copathology in CJD (HR=0.430; P=0.033); more than one neurodegenerative-associated protein in CJD (HR=0.369; P=0.036) and copathology in MM1/MV1 CJD (HR=0.525; P=0.032).

Conclusion The existence of copathology significantly prolongs survival in patients with rapidly progressive dementia due to CJD. The study of major neurodegenerative-associated proteins in brains with CJD could allow us to further understand the molecular mechanisms behind prion diseases.

  • neurodegeneration
  • brain
  • immunohistochemistry
  • neuropathology
  • proteins

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  • Handling editor Runjan Chetty.

  • Contributors IF-V reviewed the neuropathological findings in every brain and performed the coordination of the study. Immunohistochemistry and molecular analysis were done by IV-E and KPdH-G. Data analyses were developed by AM-R, JS-J and JJA. IF-V contributed with the data analyses and drafted the manuscript. AM-R, LL-G, BG, IG-M and LMQ provided technical support and critically reviewed the manuscript. All authors read and approved the final manuscript.

  • Funding This work was fully supported by Pathology Department of Hospital Universitario de Araba.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.