PT  - JOURNAL ARTICLE
AU  - Aitzol Miguelez-Rodriguez
AU  - Jorge Santos-Juanes
AU  - Ikerne Vicente-Etxenausia
AU  - Katty Perez de Heredia-Goñi
AU  - Beatriz Garcia
AU  - Luis M Quiros
AU  - Laura Lorente-Gea
AU  - Isabel Guerra-Merino
AU  - Jose J Aguirre
AU  - Ivan Fernandez-Vega
TI  - Brains with sporadic Creutzfeldt-Jakob disease and copathology showed a prolonged end-stage of disease
AID  - 10.1136/jclinpath-2017-204794
DP  - 2017 Nov 02
TA  - Journal of Clinical Pathology
PG  - jclinpath-2017-204794
4099  - http://jcp.bmj.com/content/early/2017/11/02/jclinpath-2017-204794.short
4100  - http://jcp.bmj.com/content/early/2017/11/02/jclinpath-2017-204794.full
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.