Prion diseases reported in the “Annual of the Pathological Autopsy Cases in Japan”

For surveillance projects to be successful, it is important to accurately diagnose all patients, without overlooking any cases. Here, we investigated the present clinical diagnostic accuracy for prion diseases in Japan. We analyzed volumes of the “Annual of the Pathological Autopsy Cases in Japan”,...

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Veröffentlicht in:Journal of the neurological sciences 2021-01, Vol.420, p.117215-117215, Article 117215
Hauptverfasser: Horimoto, Yoshihiko, Sato, Chikako, Inagaki, Aki, Tajima, Toshihisa, Hibino, Hiroaki, Kabasawa, Hidehiro, Inagaki, Hiroshi
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container_end_page 117215
container_issue
container_start_page 117215
container_title Journal of the neurological sciences
container_volume 420
creator Horimoto, Yoshihiko
Sato, Chikako
Inagaki, Aki
Tajima, Toshihisa
Hibino, Hiroaki
Kabasawa, Hidehiro
Inagaki, Hiroshi
description For surveillance projects to be successful, it is important to accurately diagnose all patients, without overlooking any cases. Here, we investigated the present clinical diagnostic accuracy for prion diseases in Japan. We analyzed volumes of the “Annual of the Pathological Autopsy Cases in Japan”, which reported details on 130,105 autopsies conducted from 2007 to 2016 throughout Japan. The clinical diagnosis of patients with prion disease had a specificity of 91.3% and a sensitivity of 96.3%. The autopsy rates were estimated as 17.8% for patients with clinically suspected prion disease and as 1.8% for the entire population. Despite the good accuracy of clinical diagnoses of prion diseases, a calculated 78.4 patients with prion disease were expected to have gone undiagnosed during the 10-year study period. However, autopsy is estimated to reveal a maximum of only 13.8 of these clinically undiagnosed patients because of the low autopsy rate. The overall autopsy rate, irrespective of any specific disorder, must increase for effective surveillance projects of disease incidence to be conducted. •Clinical diagnostic specificity of prion disease was 91.3% and sensitivity was 96.3%.•The autopsy rate for suspected prion disease patients was estimated as 17.8%.•The autopsy rate for the entire population was estimated as 1.8%.•While 78.4 undiagnosed prion disease patients were expected, autopsy revealed 13.8.•It is recommended that the overall autopsy rate be increased.•ker syndrome; FFI: fatal familial insomnia
doi_str_mv 10.1016/j.jns.2020.117215
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source MEDLINE; ScienceDirect Journals (5 years ago - present)
subjects Autopsy
Creutzfeldt-Jakob disease (CJD)
Creutzfeldt-Jakob Syndrome
Gerstmann-Straussler-Scheinker Disease
Gerstmann-Sträussler-Scheinker syndrome (GSS)
Humans
Japan - epidemiology
Prion disease
Prion Diseases - epidemiology
title Prion diseases reported in the “Annual of the Pathological Autopsy Cases in Japan”
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