Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review

Jiangfeng Liao; Jiangfeng Liao; Wenming Hu; Wenming Hu; Shiheng Chen; Chunyu Huang; Chunyu Huang; Senwei Dong; Senwei Dong; Wanjin Chen; Wanjin Chen; Xiaochun Chen; Longfei Chen; Longfei Chen

doi:10.3389/fnagi.2024.1379011

Frontiers in Aging Neuroscience (Apr 2024)

Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review

Jiangfeng Liao,
Jiangfeng Liao,
Wenming Hu,
Wenming Hu,
Shiheng Chen,
Chunyu Huang,
Chunyu Huang,
Senwei Dong,
Senwei Dong,
Wanjin Chen,
Wanjin Chen,
Xiaochun Chen,
Longfei Chen,
Longfei Chen

Affiliations

Jiangfeng Liao: Department of Neurology and Institute of Neurology of the First Affiliated Hospital, Institute of Neuroscience, and Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, Fuzhou, China
Jiangfeng Liao: Department of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, China
Wenming Hu: Department of Neurology and Institute of Neurology of the First Affiliated Hospital, Institute of Neuroscience, and Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, Fuzhou, China
Wenming Hu: Department of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, China
Shiheng Chen: Department of Neurology, Fuzhou Changle District People’s Hospital, Fuzhou, China
Chunyu Huang: Department of Neurology and Institute of Neurology of the First Affiliated Hospital, Institute of Neuroscience, and Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, Fuzhou, China
Chunyu Huang: Department of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, China
Senwei Dong: Department of Neurology and Institute of Neurology of the First Affiliated Hospital, Institute of Neuroscience, and Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, Fuzhou, China
Senwei Dong: Department of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, China
Wanjin Chen: Department of Neurology and Institute of Neurology of the First Affiliated Hospital, Institute of Neuroscience, and Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, Fuzhou, China
Wanjin Chen: Department of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, China
Xiaochun Chen: Department of Neurology, Fujian Medical University Union Hospital, Fuzhou, China
Longfei Chen: Department of Neurology and Institute of Neurology of the First Affiliated Hospital, Institute of Neuroscience, and Fujian Key Laboratory of Molecular Neurology, Fujian Medical University, Fuzhou, China
Longfei Chen: Department of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, China

DOI: https://doi.org/10.3389/fnagi.2024.1379011
Journal volume & issue: Vol. 16

Abstract

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BackgroundAs a rare neurodegenerative disease, sporadic Creutzfeldt-Jakob disease (sCJD) is poorly understood in the elderly populace. This study aims to enunciate the multidimensional features of sCJD in this group.MethodsA case of probable sCJD was reported in a 90-year-old Chinese man with initial dizziness. Then, available English literature of the elderly sCJD cases (aged 80 years and over) was reviewed and analyzed. Patients (15 cases) were subdivided and compared geographically.ResultsIn the elderly sCJD cohort, the onset age was 84.9 ± 4.5 years and the median disease duration was 6.8 months, with respiratory infection/failure as the commonest death cause. Various clinical symptoms were identified, with cognitive disorder (86.7%) as the commonest typical symptom and speech impairment (66.7%) as the most atypical one. Restricted hyperintensities were reported in 60.0% cases on DWI, periodic sharp wave complexes in 73.3% cases on electroencephalogram, and cerebral hypoperfusion/hypometabolism in 26.7% cases on molecular imaging. The sensitive cerebrospinal fluid biomarkers were total tau (83.3%), 14-3-3 protein (75.0%), and PrP RT-QuIC (75.0%). Neuropathological profiles in the cerebral cortex revealed vacuolar spongiosis, neuronal loss, gliosis, and aging-related markers, with synaptic deposit as the commonest PrP pattern (60.0%). The polymorphic PRNP analysis at codon 129 was M/M (90.9%), with MM1 and MM2C as the primary molecular phenotypes. Latency to first clinic visit, hyperintense signals on DWI, and disease duration were significantly different between the patient subgroups.ConclusionThe characteristics of sCJD are multidimensional in the elderly, deepening our understanding of the disease and facilitating an earlier recognition and better care for this group.

Published in Frontiers in Aging Neuroscience

ISSN: 1663-4365 (Online)
Publisher: Frontiers Media S.A.
Country of publisher: Switzerland
LCC subjects: Medicine: Internal medicine: Neurosciences. Biological psychiatry. Neuropsychiatry
Website: https://www.frontiersin.org/journals/aging-neuroscience

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