학술논문
Genetic Creutzfeldt-Jakob disease affected monozygotic twins: Analysis of survival time, age at death and possible exogenous risk factors.
Document Type
Academic Journal
Author
Mitrová E; Department of Prion Diseases, Slovak Medical University, Limbová 14, Bratislava, Slovakia. Electronic address: eva.mitrova@szu.sk.; Záková-Slivarichová D; Department of Prion Diseases, Slovak Medical University, Limbová 14, Bratislava, Slovakia.; Stelzer M; Department of Prion Diseases, Slovak Medical University, Limbová 14, Bratislava, Slovakia.; Belay G; Department of Prion Diseases, Slovak Medical University, Limbová 14, Bratislava, Slovakia.; Janáková A; Department of Prion Diseases, Slovak Medical University, Limbová 14, Bratislava, Slovakia.; Koscová S; Department of Prion Diseases, Slovak Medical University, Limbová 14, Bratislava, Slovakia.
Source
Publisher: Churchill Livingstone Country of Publication: Scotland NLM ID: 9433352 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-2653 (Electronic) Linking ISSN: 09675868 NLM ISO Abbreviation: J Clin Neurosci Subsets: MEDLINE
Subject
Language
English
Abstract
Three monozygotic twin pairs with the Creutzfeldt-Jakob diseases-specific mutation E200K are described. All three have been concordant for genetic CJD E200K and discordant for the age at death and the duration of the disease. Twin pairs have been compared with genetically non - identical sibling pairs also concordant for genetic CJD E200K and discordant for the age at death. The difference of the mean age at death in compared subgroups was not significant. Detailed analysis of twin pairs revealed considerable differences in the duration and quality of chronic stress, induced by the analysed exogenous factors. The stress was evidently of higher intensity in two of the three earlier affected twins. Clear correlation between the age at death and medical history of twins was not observed. The discordance of twins with genetic CJD E200K in the age at death and the striking correlation with the discordant intensity of analysed exogenous influence, draw attention to described potential risk factors (mainly to chronic social, economic and emotional stress) and support their role in accelerating the clinical onset of genetic CJD E200K .
(Copyright © 2019 Elsevier Ltd. All rights reserved.)
(Copyright © 2019 Elsevier Ltd. All rights reserved.)