Brains with sporadic Creutzfeldt-Jakob disease and copathology showed a prolonged end-stage of disease
| Autor: | Isabel Guerra-Merino, Aitzol Miguelez-Rodriguez, Ikerne Vicente-Etxenausia, Katty Perez de Heredia-Goñi, Jorge Santos-Juanes, Luis M. Quirós, Laura Lorente-Gea, José Javier Aguirre, Beatriz García, Iván Fernández-Vega |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Male Pathology medicine.medical_specialty Amyloid beta Biopsy Nerve Tissue Proteins tau Proteins Neuropathology Disease Kaplan-Meier Estimate Creutzfeldt-Jakob Syndrome Pathology and Forensic Medicine 03 medical and health sciences 0302 clinical medicine mental disorders medicine Humans Stage (cooking) Phosphorylation Survival analysis Aged Biological Specimen Banks Retrospective Studies Univariate analysis Amyloid beta-Peptides biology business.industry Neurodegeneration Brain General Medicine Middle Aged medicine.disease Immunohistochemistry nervous system diseases DNA-Binding Proteins 030104 developmental biology Spain biology.protein Disease Progression alpha-Synuclein Dementia Female Autopsy business 030217 neurology & neurosurgery |
| Zdroj: | Journal of clinical pathology. 71(5) |
| ISSN: | 1472-4146 |
| Popis: | AimsTo investigate the expression of major proteins related to primary neurodegenerative diseases and their prognostic significance in brains with Creutzfeldt-Jakob disease (CJD).Materials and methodsThirty 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.ResultsCopathology 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).ConclusionThe 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. |
| Databáze: | OpenAIRE |
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