10.5061/DRYAD.GP47D91
Hermann, Peter
University of Göttingen
Laux, Mareike
University of Göttingen
Glatzel, Markus
University Medical Center Hamburg-Eppendorf
Matschke, Jakob
University Medical Center Hamburg-Eppendorf
Knipper, Tobias
University of Göttingen
Goebel, Stefan
University of Göttingen
Treig, Johannes
University of Göttingen
Schulz-Schaeffer, Walter
Saarland University
Cramm, Maria
University of Göttingen
Schmitz, Matthias
University of Göttingen
Zerr, Inga
University of Göttingen
Data from: Validation and utilization of amended diagnostic criteria in
Creutzfeldt-Jakob Disease surveillance
Dryad
dataset
2019
Prion disease
Cerebrospinal fluid
Incidence studies
Prion
Diagnostic test assessment
2019-05-02T00:00:00Z
2019-05-02T00:00:00Z
en
https://doi.org/10.1212/wnl.0000000000005860
22665 bytes
1
CC0 1.0 Universal (CC0 1.0) Public Domain Dedication
Objective: To validate an amended protocol for clinical diagnosis of
sporadic Creutzfeldt-Jakob Disease (sCJD) including Real-Time
Quaking-induced Conversion (RT-QuIC) and to observe its utilization in CJD
surveillance. Methods: In the framework of a prospective epidemiological
study, all neuropathological confirmed sCJD cases that received CSF
RT-QuIC analysis during diagnostic work up (n=65) and a control group of
non- CJD cases (n=118) were selected to investigate the accuracy of an
amended diagnostic protocol. The patients had been referred to the German
National Reference Center for Transmissible Spongiform Encephalopathies.
The influence of the amended protocol on incidence figures was evaluated
in the context of three years of surveillance activity (screened cases
using 14-3-3 test: n=18.789, highly suspicious cases of CJD: n=704).
Annual incidences were calculated using current criteria and the amended
protocol. Results: The amended protocol showed a sensitivity of 97% and a
specificity of 99%. When it was applied to all suspected cases that were
referred to the reference center, the assessed incidence of CJD increased
from 1.7 to 2.2 per million in 2016. Conclusion: CJD surveillance remains
challenging as information from external healthcare institutions can be
limited. RT-QuIC shows excellent diagnostic accuracy when applied in the
clinical setting to symptomatic patients. Data for RT-QuIC alone, when
applied as a general screening test, are not available yet. We propose an
amended research protocol which improves early and accurate clinical
diagnosis of sCJD during surveillance activities. The utilization of this
protocol will probably lead to a significant increase of the incidence
rate.
table e-1Performed CSF tests and surveillance dataID# NEUROLOGY2017869404
table e-1.docx