title: 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.
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.
authors.affiliation: a Department of Neurology ; Seoul National University Boramae Hospital ; Seoul , South Korea ;
authors.forename: Jae-Sung
authors.initials: JS
authors.lastname: Lim
authors.forename: Hyung-Min
authors.initials: HM
authors.lastname: Kwon
authors.forename: Jae-Won
authors.initials: JW
authors.lastname: Jang
authors.forename: Young-Ran
authors.initials: YR
authors.lastname: Ju
authors.forename: SuYeon
authors.initials: S
authors.lastname: Kim
authors.forename: Young Ho
authors.initials: YH
authors.lastname: Park
authors.forename: So Young
authors.initials: SY
authors.lastname: Park
authors.forename: SangYun
authors.initials: S
authors.lastname: Kim
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid*; D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000453:epidemiology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D062486:Public Health Surveillance; D056910:Republic of Korea / Q000453:epidemiology
chemical_list: D048948:14-3-3 Proteins
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