Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review
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 initia...
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Frontiers Media S.A.
2024-04-01
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| Series: | Frontiers in Aging Neuroscience |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fnagi.2024.1379011/full |
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| author | 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 |
| author_facet | 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 |
| author_sort | Jiangfeng Liao |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-58926469b5aa4d1688ab4f1063d1c880 |
| institution | Kabale University |
| issn | 1663-4365 |
| language | English |
| publishDate | 2024-04-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Aging Neuroscience |
| spelling | doaj-art-58926469b5aa4d1688ab4f1063d1c8802025-02-06T13:14:23ZengFrontiers Media S.A.Frontiers in Aging Neuroscience1663-43652024-04-011610.3389/fnagi.2024.13790111379011Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic reviewJiangfeng Liao0Jiangfeng Liao1Wenming Hu2Wenming Hu3Shiheng Chen4Chunyu Huang5Chunyu Huang6Senwei Dong7Senwei Dong8Wanjin Chen9Wanjin Chen10Xiaochun Chen11Longfei Chen12Longfei Chen13Department 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, ChinaDepartment of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, ChinaDepartment 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, ChinaDepartment of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, ChinaDepartment of Neurology, Fuzhou Changle District People’s Hospital, Fuzhou, ChinaDepartment 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, ChinaDepartment of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, ChinaDepartment 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, ChinaDepartment of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, ChinaDepartment 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, ChinaDepartment of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, ChinaDepartment of Neurology, Fujian Medical University Union Hospital, Fuzhou, ChinaDepartment 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, ChinaDepartment of Neurology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou, ChinaBackgroundAs 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.https://www.frontiersin.org/articles/10.3389/fnagi.2024.1379011/fullsporadic Creutzfeldt-Jakob diseaseelderlyclinical featuresdiagnosis biomarkersneuropathology hallmarks |
| spellingShingle | 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 Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review Frontiers in Aging Neuroscience sporadic Creutzfeldt-Jakob disease elderly clinical features diagnosis biomarkers neuropathology hallmarks |
| title | Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review |
| title_full | Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review |
| title_fullStr | Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review |
| title_full_unstemmed | Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review |
| title_short | Multidimensional features of sporadic Creutzfeldt-Jakob disease in the elderly: a case report and systematic review |
| title_sort | multidimensional features of sporadic creutzfeldt jakob disease in the elderly a case report and systematic review |
| topic | sporadic Creutzfeldt-Jakob disease elderly clinical features diagnosis biomarkers neuropathology hallmarks |
| url | https://www.frontiersin.org/articles/10.3389/fnagi.2024.1379011/full |
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