Accuracy of administrative diagnostic data for pathologically confirmed cases of Creutzfeldt-Jakob disease in Massachusetts, 2000-2008.
BACKGROUND
Creutzfeldt-Jakob disease (CJD) is a transmissible disorder that is monitored by public health authorities at the state and national levels in the United States. Little is known about the current accuracy and concurrence of CJD diagnoses across national and state sources of surveillance data.


METHODS
Using multiple sources, including the National Prion Disease Pathology Surveillance Center (NPDPSC) registry, we sought to identify all deceased Massachusetts patients with pathologically diagnosed CJD between 2000 and 2008. Pathologically verified CJD cases were then matched to their respective records in the Massachusetts hospital discharge and death certificate datasets. Using these data, we also aimed to estimate the sensitivity and specificity of death certificate diagnoses.


RESULTS
Death certificate and hospital discharge dataset diagnoses of CJD combined accounted for 80% (35 of 44) of pathologically confirmed cases. The estimated sensitivity and specificity for death certificate diagnoses alone were 71% (27 of 38) and 75% (9 of 12), respectively.


CONCLUSIONS
Death certificate diagnoses were less sensitive for pathologically confirmed CJD than reported previously. Increasing reliance on autopsy over biopsy and an expanding spectrum of health care delivery may be responsible for this discrepancy. The findings reported here underscore the value of using multiple mechanisms in national CJD surveillance.
Department of Medicine, Yale University School of Medicine, Robert Wood Johnson Foundation Clinical Scholars Program, New Haven, CT; Department of Neurology, Veterans Affairs Medical Center, West Haven, CT. Electronic address: jed.a.barash@lahey.org.
Jed A
JA
Barash
Bureau of Infectious Diseases, Massachusetts Department of Public Health, Boston, MA.
James K
JK
West
Bureau of Health Statistics, Research, and Evaluation, Massachusetts Department of Public Health, Jamaica Plain, MA.
Alfred
A
DeMaria
D000368:Aged; D000369:Aged, 80 and over; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000453:epidemiology*; D066264:Datasets as Topic / Q000592:standards*; D003644:Death Certificates; D005260:Female; D006801:Humans; D038801:International Classification of Diseases; D008297:Male; D008404:Massachusetts / Q000453:epidemiology; D008875:Middle Aged; D063886:Patient Discharge Summaries / Q000592:standards*; D011159:Population Surveillance; D012042:Registries / Q000592:standards*; D012680:Sensitivity and Specificity
Death certificate; Prion diseases; Surveillance