Detecting Creutzfeldt-Jakob disease in the United States: An analysis of CDC surveillance data, 1994-2006 Public

Abstract

Detecting Creutzfeldt-Jakob disease in the United States: An analysis of CDC surveillance data, 1994-2006
By Caitlin Mertzlufft

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive fatal neurodegenerative disease associated with infectious prions. The Centers for Disease Control and Prevention (CDC) have monitored potential variant Creutzfeldt-Jakob disease (vCJD) cases in the United States since 1996 in individuals diagnosed with prion-related disease who were less than age 55 at time of death, following the novel identification of this disease in the UK; two years of
retrospective data were also collected from 1994 and 1995. Using data collected from this surveillance effort, this paper seeks to examine the clinical presentation and associated demographic characteristics of this < 55 cohort, especially in comparison with the typical clinical presentation of known CJD cases from around the world and a cohort of older (age > 55) prion disease cases from the United States. World Health Organization (WHO) and CDC diagnostic criteria were used to sort the 309 individuals who fit study criteria into CJD subtype by clinical presentation, and a logistic regression model was used to evaluate the association of the typical electroencephalogram (EEG) signal seen in sporadic CJD patients with symptoms of interest. Typical EEG characteristic of sporadic CJD was found to be significantly associated with myoclonus and visual or cerebellar signs. The majority of cases were sporadic CJD at 70.55%, and no cases of variant CJD were identified. Analyses of clinical features of the CJD cases emphasize the importance of robust neuropathologic testing to confirm diagnoses.

Detecting Creutzfeldt-Jakob disease in the United States: An analysis of CDC surveillance data, 1994-2006
By
Caitlin Mertzlufft
Bachelor of Science, University of Georgia, 2008
Faculty Thesis Advisor: Brad Pearce, PhD
Thesis Field Advisor: Ryan Maddox, MPH
A thesis submitted to the Faculty of the
Rollins School of Public Health of Emory University
in partial fulfillment of the requirements for the degree of
Master of Public Health
in Global Epidemiology
2011

Table of Contents

Table of Contents
Introduction...1

Human Prion Disease...2
Animal Prion Disease...4
BSE and vCJD...7
Present Day vCJD...8
vCJD Surveillance in the United States...11

Results...22
Discussion...28
Conclusion...33
References...34
Tables and Figures...39

Figure 1. Distribution of Creutzfeldt-Jakob disease cases by year among those individuals <55 and >54 identified through the national Creutzfeldt-Jakob disease surveillance system, 1994-2006...39
Figure 2. Distribution of CJD cases by age among those individuals <55 identified through the national Creutzfeldt-Jakob disease surveillance system, 1994-2006...40
Figure 3. Distriution of CJD cases by age among those individuals > 54 identified through the national Creutzfeldt-Jakob disease surveillance system, 1994-2006...41
Table 1. Demographic characteristics of individuals aged < 55 at time of death identified through the national Creutzfeldt-Jakob disease surveillance system, 1994-2006...42
Table 2. Comparison of demographic characteristics between individuals < 55 years of age and > 55 years of age at time of death identified through the national Creutzfeldt-Jakob disease surveillance system, United States, 1994-2006...43
Table 3. A comparison of demographic characteristics between individuals < 55 years of age who had an autopsy or biopsy performed and those who did not among those identified through the national Creutzfeldt-Jakob disease surveillance system 1994-2006...44
Table 4. Classification by CJD type among individuals < 55 years of age identified through the national Creutzfeldt-Jakob disease surveillance system, 1994-2006...45
Table 5. Occurrence of symptoms associated with sporadic Creutzfeldt-Jakob disease among individuals < 55 years of age classified as having definite or probable sporadic Creutzfeldt-Jakob disease (N=176) and possible sCJD (N=42)...46
Table 6. Occurrence of variant CJD diagnostic criteria among decedents < 55 years of age at time of death with neuropathologic confirmation of sporadic CJD, 1994-2006 (N=135)...48
Table 7. Logistic regression model to determine the association between typical electroencephalogram (EEG) and symptoms of interest among CJD patients < 55 years of age identified through the national Creutzfeldt-Jakob disease surveillance system, 1994-2006...49

Appendix A - Short abstraction form...50
Appendix B - Long abstraction form...52
Appendix C - CDC Diagnostic Criteria for variant CJD...60
Appendix D - WHO Diagnostic Criteria for CJD...61
Appendix E - IRB clearance...62

About this Master's Thesis

Rights statement

• Permission granted by the author to include this thesis or dissertation in this repository. All rights reserved by the author. Please contact the author for information regarding the reproduction and use of this thesis or dissertation.

School	Rollins School of Public Health
Department	Epidemiology
Subfield / Discipline	Global Epidemiology - MPH & MSPH
Degree	MPH
Submission	Master's Thesis
Language	English
Research Field	Health Sciences, Public Health
Mot-clé	Creutzfeldt-Jakob disease transmissible spongiform encephalopathy prion variant Creutzfelt-Jakob disease bovine spongiform encephalopathy neurodegenerative
Committee Chair / Thesis Advisor	Pearce, Brad, Emory University
Committee Members	Maddox, Ryan, Centers for Disease Control and Prevention (CDC)
Partnering Agencies	CDC

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