Validation and utilization of amended diagnostic criteria in Creutzfeldt-Jakob disease surveillance
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Validation and utilization of amended diagnostic criteria in Creutzfeldt-Jakob disease surveillance. / Hermann, Peter; Laux, Mareike; Glatzel, Markus; Matschke, Jakob; Knipper, Tobias; Goebel, Stefan; Treig, Johannes; Schulz-Schaeffer, Walter; Cramm, Maria; Schmitz, Matthias; Zerr, Inga.
In: NEUROLOGY, Vol. 91, No. 4, 24.07.2018, p. e331-e338.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Validation and utilization of amended diagnostic criteria in Creutzfeldt-Jakob disease surveillance
AU - Hermann, Peter
AU - Laux, Mareike
AU - Glatzel, Markus
AU - Matschke, Jakob
AU - Knipper, Tobias
AU - Goebel, Stefan
AU - Treig, Johannes
AU - Schulz-Schaeffer, Walter
AU - Cramm, Maria
AU - Schmitz, Matthias
AU - Zerr, Inga
N1 - © 2018 American Academy of Neurology.
PY - 2018/7/24
Y1 - 2018/7/24
N2 - 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 use in CJD surveillance.METHODS: In the framework of a prospective epidemiologic study, all neuropathologically confirmed cases with sCJD who received CSF RT-QuIC analysis during diagnostic workup (n = 65) and a control group of individuals without CJD (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 3 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 with 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 who 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 because information from external health care 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 that improves early and accurate clinical diagnosis of sCJD during surveillance activities. The use of this protocol will probably lead to a significant increase of the incidence rate.CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that for patients with suspected sCJD, criteria for clinical diagnosis plus the CSF RT-QuIC accurately identifies patients with sCJD (sensitivity 97%, specificity 99%).
AB - 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 use in CJD surveillance.METHODS: In the framework of a prospective epidemiologic study, all neuropathologically confirmed cases with sCJD who received CSF RT-QuIC analysis during diagnostic workup (n = 65) and a control group of individuals without CJD (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 3 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 with 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 who 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 because information from external health care 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 that improves early and accurate clinical diagnosis of sCJD during surveillance activities. The use of this protocol will probably lead to a significant increase of the incidence rate.CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that for patients with suspected sCJD, criteria for clinical diagnosis plus the CSF RT-QuIC accurately identifies patients with sCJD (sensitivity 97%, specificity 99%).
KW - Journal Article
U2 - 10.1212/WNL.0000000000005860
DO - 10.1212/WNL.0000000000005860
M3 - SCORING: Journal article
C2 - 29934424
VL - 91
SP - e331-e338
JO - NEUROLOGY
JF - NEUROLOGY
SN - 0028-3878
IS - 4
ER -