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. 2015;9(2):136-43.
doi: 10.1080/19336896.2015.1022020.

Characteristics of Korean patients with suspected Creutzfeldt-Jakob disease with 14-3-3 protein in cerebrospinal fluid: Preliminary study of the Korean Creutzfeldt-Jakob disease active surveillance program

Jae-Sung Lim  1 , Hyung-Min Kwon, Jae-Won Jang, Young-Ran Ju, SuYeon Kim, Young Ho Park, So Young Park, SangYun Kim
Affiliations

Characteristics of Korean patients with suspected Creutzfeldt-Jakob disease with 14-3-3 protein in cerebrospinal fluid: Preliminary study of the Korean Creutzfeldt-Jakob disease active surveillance program

Jae-Sung Lim et al. Prion. 2015.

Abstract

Although Korea had a national surveillance system for Creutzfeldt-Jakob disease (CJD), it was mainly dependent on attending physician's reports. Thus, little prospective data about the epidemiology, characteristics, and final diagnoses of suspected patients were available. We have established a nationwide network for the active surveillance of patients with suspected CJD. When the requested cerebrospinal fluid (CSF) samples tested positive for 14-3-3 protein, we investigated the clinical characteristics of the corresponding patients and followed them until their final diagnoses were confirmed. A total of 218 samples were requested for CSF assays from May 2010 to August 2012, and 106 (48.6%) were positive for 14-3-3 protein. In 89 patients with complete clinical data, 38 (42.7%) were diagnosed with probable CJD and the estimated annual occurrence of CJD was 16.3 persons-per-year. The most common diagnoses of the remainder were central nervous system infection and any-cause encephalopathy. Non-CJD subjects showed worse initial consciousness levels than CJD patients. This preliminary study showed that the number of reported cases of CJD and the true positivity rates of CSF 14-3-3 protein assays were both low in Korea. An active surveillance system is urgently needed to provide the latest nationwide epidemiological data of CJD.

Keywords: 14-3-3 proteins; CJD, Creutzfeldt-Jakob disease; CSF, cerebrospinal fluid; Creutzfeldt-Jakob disease; K-CDC, Korean Center for Disease Control; WHO, World Health Organization; cerebrospinal fluid; surveillance.

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Figures

Figure 1.
Figure 1.
Enrollment flow chart. CSF indicates cerebrospinal fluid; CJD, Creutzfeldt-Jakob disease.
See this image and copyright information in PMC

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