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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Article info
Publication history
Published online: September 04, 2021
Footnotes
Ling-Yun Fan and Yu Sun contributed equally to this study.
This report was supported by a grant (MOHW108-CDC-C- 114-123502) from the Ministry of Health and Welfare, Taiwan.
The authors declare no conflicts of interest.
Identification
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© 2021 AMDA - The Society for Post-Acute and Long-Term Care Medicine.
