Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.

<h4>Objective</h4>Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal and transmissible neurodegenerative disorder. However, no studies have reported Chinese specific characteristics of sCJD. We aimed to identify differences in sCJD between Chinese patients and patients from other count...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Wei Zhao, Jia-Tang Zhang, Xiao-Wei Xing, De-Hui Huang, Cheng-Lin Tian, Wei-Quan Jia, Xu-Sheng Huang, Wei-Ping Wu, Chuan-Qiang Pu, Sen-Yang Lang, Sheng-Yuan Yu
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2013
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/010f4af5ede546b6bb59d59cc65533a9
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:010f4af5ede546b6bb59d59cc65533a9
record_format	dspace
spelling	oai:doaj.org-article:010f4af5ede546b6bb59d59cc65533a92021-11-18T07:53:28ZChinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.1932-620310.1371/journal.pone.0058442https://doaj.org/article/010f4af5ede546b6bb59d59cc65533a92013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/23516482/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Objective</h4>Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal and transmissible neurodegenerative disorder. However, no studies have reported Chinese specific characteristics of sCJD. We aimed to identify differences in sCJD between Chinese patients and patients from other countries.<h4>Methods</h4>The data from 57 Chinese sCJD patients were retrospectively analyzed, including demographic data, clinical manifestations, laboratory examinations, electroencephalograms (EEGs), diffusion-weighted imaging (DWI) scans, positron emission tomography (PET) scans, and pathological results.<h4>Result</h4>The disease was pathologically confirmed in 11 patients. 39 cases were diagnosed as probable sCJD, and 7 were possible. Of the total cases, 33 were male, and 24 were female. The onset age ranged from 36 to 75 years (mean: 55.5, median: 57). Disease onset before the age of 60 occurred in 57.9% of patients. The disease duration from onset to death ranged 5-22 months (mean: 11.6, median: 11), and 51.9% of patients died 7 to 12 months after disease onset. The majority of patients presented with sub-acute onset with progressive dementia. 3 of the 9 patients who took 14-3-3 protein analysis had positive results (33.3%). The sensitivity of EEG was 79.6% (43/54). For DWI and PET examinations, the sensitivities were 94% (47/50) and 94.1% (16/17), respectively. In seven patients who did not show typical hyper-intensities on the first DWI examination, abnormalities of hypo-metabolism in the cerebral cortex were clearly detected by PET. In 13 out of the 17 patients, PET detected extra abnormal regions in addition to the hyper-intense areas observed in DWI.<h4>Conclusion</h4>This is the first study to indicate that Chinese sCJD patients have a much earlier onset age and a longer disease duration than other populations, which is most likely related to racial differences. The longer disease duration may also be a probable characteristic of Asian populations. PET had high sensitivity for the diagnosis of sCJD.Wei ZhaoJia-Tang ZhangXiao-Wei XingDe-Hui HuangCheng-Lin TianWei-Quan JiaXu-Sheng HuangWei-Ping WuChuan-Qiang PuSen-Yang LangSheng-Yuan YuPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 3, p e58442 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Wei Zhao Jia-Tang Zhang Xiao-Wei Xing De-Hui Huang Cheng-Lin Tian Wei-Quan Jia Xu-Sheng Huang Wei-Ping Wu Chuan-Qiang Pu Sen-Yang Lang Sheng-Yuan Yu Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.
description	<h4>Objective</h4>Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal and transmissible neurodegenerative disorder. However, no studies have reported Chinese specific characteristics of sCJD. We aimed to identify differences in sCJD between Chinese patients and patients from other countries.<h4>Methods</h4>The data from 57 Chinese sCJD patients were retrospectively analyzed, including demographic data, clinical manifestations, laboratory examinations, electroencephalograms (EEGs), diffusion-weighted imaging (DWI) scans, positron emission tomography (PET) scans, and pathological results.<h4>Result</h4>The disease was pathologically confirmed in 11 patients. 39 cases were diagnosed as probable sCJD, and 7 were possible. Of the total cases, 33 were male, and 24 were female. The onset age ranged from 36 to 75 years (mean: 55.5, median: 57). Disease onset before the age of 60 occurred in 57.9% of patients. The disease duration from onset to death ranged 5-22 months (mean: 11.6, median: 11), and 51.9% of patients died 7 to 12 months after disease onset. The majority of patients presented with sub-acute onset with progressive dementia. 3 of the 9 patients who took 14-3-3 protein analysis had positive results (33.3%). The sensitivity of EEG was 79.6% (43/54). For DWI and PET examinations, the sensitivities were 94% (47/50) and 94.1% (16/17), respectively. In seven patients who did not show typical hyper-intensities on the first DWI examination, abnormalities of hypo-metabolism in the cerebral cortex were clearly detected by PET. In 13 out of the 17 patients, PET detected extra abnormal regions in addition to the hyper-intense areas observed in DWI.<h4>Conclusion</h4>This is the first study to indicate that Chinese sCJD patients have a much earlier onset age and a longer disease duration than other populations, which is most likely related to racial differences. The longer disease duration may also be a probable characteristic of Asian populations. PET had high sensitivity for the diagnosis of sCJD.
format	article
author	Wei Zhao Jia-Tang Zhang Xiao-Wei Xing De-Hui Huang Cheng-Lin Tian Wei-Quan Jia Xu-Sheng Huang Wei-Ping Wu Chuan-Qiang Pu Sen-Yang Lang Sheng-Yuan Yu
author_facet	Wei Zhao Jia-Tang Zhang Xiao-Wei Xing De-Hui Huang Cheng-Lin Tian Wei-Quan Jia Xu-Sheng Huang Wei-Ping Wu Chuan-Qiang Pu Sen-Yang Lang Sheng-Yuan Yu
author_sort	Wei Zhao
title	Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.
title_short	Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.
title_full	Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.
title_fullStr	Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.
title_full_unstemmed	Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.
title_sort	chinese specific characteristics of sporadic creutzfeldt-jakob disease: a retrospective analysis of 57 cases.
publisher	Public Library of Science (PLoS)
publishDate	2013
url	https://doaj.org/article/010f4af5ede546b6bb59d59cc65533a9
work_keys_str_mv	AT weizhao chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT jiatangzhang chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT xiaoweixing chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT dehuihuang chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT chenglintian chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT weiquanjia chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT xushenghuang chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT weipingwu chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT chuanqiangpu chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT senyanglang chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases AT shengyuanyu chinesespecificcharacteristicsofsporadiccreutzfeldtjakobdiseasearetrospectiveanalysisof57cases
_version_	1718422805043740672

Chinese specific characteristics of sporadic Creutzfeldt-Jakob disease: a retrospective analysis of 57 cases.

Ejemplares similares