RT Journal Article SR Electronic T1 Brains with sporadic Creutzfeldt-Jakob disease and copathology showed a prolonged end-stage of disease JF Journal of Clinical Pathology JO J Clin Pathol FD BMJ Publishing Group Ltd and Association of Clinical Pathologists SP jclinpath-2017-204794 DO 10.1136/jclinpath-2017-204794 A1 Aitzol Miguelez-Rodriguez A1 Jorge Santos-Juanes A1 Ikerne Vicente-Etxenausia A1 Katty Perez de Heredia-Goñi A1 Beatriz Garcia A1 Luis M Quiros A1 Laura Lorente-Gea A1 Isabel Guerra-Merino A1 Jose J Aguirre A1 Ivan Fernandez-Vega YR 2017 UL http://jcp.bmj.com/content/early/2017/11/02/jclinpath-2017-204794.abstract AB 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.