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Prognostic Features of Sporadic Creutzfeldt-Jakob Disease: An Analysis of Taiwan's Nationwide Surveillance

  • Yu Sun
    Affiliations
    Department of Neurology, En Chu Kong Hospital, New Taipei City, Taiwan

    Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
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  • Ling-Yun Fan
    Affiliations
    Queensland Brain Institute, University of Queensland, St Lucia, Queensland, Australia
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  • Chung-Te Huang
    Affiliations
    Center for Research, Diagnostics and Vaccine Development, Taiwan Centers for Disease Control, Taipei, Taiwan
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  • Chih-Ching Liu
    Affiliations
    Department of Healthcare Administration, College of Medical and Health Science, Asia University, Taichung, Taiwan
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  • Ta-Fu Chen
    Affiliations
    Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
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  • Chien-Jung Lu
    Affiliations
    Department of Neurology, En Chu Kong Hospital, New Taipei City, Taiwan

    Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
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  • Wan-Yuo Guo
    Affiliations
    Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan

    School of Medicine, National Yang-Ming University, Taipei, Taiwan
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  • Yang-Chyuan Chang
    Correspondence
    Yang-Chyuan Chang, MD, MBA, Min-Sheng General Hospital, No. 168, Jingguo Rd, Taoyuan District, Taoyuan 330, Taiwan.
    Affiliations
    Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan

    Department of Neurology, Min-Sheng General Hospital, Taoyuan, Taiwan
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  • Ming-Jang Chiu
    Correspondence
    Address correspondence to Ming-Jang Chiu, MD, PhD, Department of Neurology, National Taiwan University Hospital, No. 7, Chung-Shang S Rd, Chung-Zheng District, Taipei 100, Taiwan.
    Affiliations
    Department of Neurology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan

    Graduate Institute of Brain and Mind Sciences, College of Medicine, National Taiwan University, Taipei, Taiwan

    Graduate Institute of Psychology, College of Science, National Taiwan University, Taipei, Taiwan

    Graduate Institute of Biomedical Electronics and Bioinformatics, National Taiwan University, Taipei, Taiwan
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Published:September 04, 2021DOI:https://doi.org/10.1016/j.jamda.2021.08.010

      Abstract

      Objectives

      To study the prognostic features of Creutzfeldt-Jakob disease (CJD) and shed light on its future therapy.

      Design

      Retrospective cohort study of a longitudinal national cohort of the Taiwan Centers for Disease Control.

      Setting and Participants

      All patients with suspected CJD are reported to the CJD surveillance unit of the Taiwan Centers for Disease Control. An expert committee discussed the reported cases and designated a consensus-based diagnosis. From 1996 to 2020, a total of 809 cases were referred to the CJD surveillance unit for confirmation; of these, 441 cases (women, n = 230) were determined to be sporadic CJD.

      Methods

      We investigated the clinical manifestations and laboratory findings for 400 patients diagnosed with definite or probable sporadic CJD. We used Kaplan-Meier analyses and Cox proportional hazards model to identify prognostic factors.

      Results

      The mean age of onset was 67 ± 9.9 years. The mean survival duration was 13.3 ± 14.2 (median 10) months. The leading clinical symptoms were myoclonus (73%) and akinetic mutism (54%). For PRNP polymorphism, 99% of patients (195/197) showed a methionine homozygous genotype at codon 129 (M129M). The sensitivity of periodic sharp wave complexes (PSWCs) on electroencephalograms (EEGs) was 59.7%. The sensitivity of cerebrospinal fluid 14-3-3 protein and total tau protein (>1200 pg/mL) were 69.7% and 75.6%, respectively. Younger patients lived longer than those aged ≥65 years [hazard ratio (HR) 0.466, P < .001]. Women had a better survival probability in the first 3 years than their male counterparts (HR 0.712, P = .005). PSWCs had a persistent negative effect on survival (HR 0.788, P < .05). Although uncommon, epileptic seizures were the only clinical prognostic factor for survival time (HR 0.768, P < .05). PSWCs can be used as an EEG biomarker for prognosis. Epileptic seizures, though not common, are the only clinical prognostic factor for a short survival.

      Conclusions and Implications

      We found that a lower age of onset and female gender favor the survival of patients with sCJD. PSWCs are EEG biomarkers unfavorable for survival, and so are epileptic seizures.

      Keywords

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