Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, incurable, and fatal neurodegenerative disorder. The objective of this study was to describe the clinical features and survival time of Chinese sCJD patients, and to explore the associations between clinical data and survival. In this study, we analysed the clinical data of 21 sCJD patients in a tertiary care hospital and used all Chinese case material available from 152 patients with sCJD in literatures between 2008 and 2018. The mean age of onset of all 173 deceased patients was 61.44 year-olds (y), with the highest incidence in the population of 60 to 69 y. The most common manifestation at disease onset was progressive dementia. With the progression of the disease, the four main clinical symptoms and signs were developed, including myoclonus, visual or cerebella disturbance, pyramidal or extrapyramidal dysfunction, and akinetic mutism. Extrapyramidal symptoms were more frequently observed. The mean survival time was 7.34 months, and 82.10% of cases died within 1 year after disease onset. The follow-up showed that the survival time was longer and the myoclonus sign was more frequently presented in younger-onset sCJD patients. Patients with abnormalities only in cortical regions had a higher frequency of pyramidal dysfunction than patients having lesions in both cortex and basal ganglia. The findings of this study might provide some insight into the clinical characteristics of sCJD patients in China, but further studies could examine the presences of clinical features and survival time in patients with early age of onset in a prospective manner.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2020 |
|---|---|
| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:14 |
|---|---|
| Enthalten in: |
Prion - 14(2020), 1 vom: 18. Dez., Seite 137-142 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Yang, Jing [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
A tertiary care hospital |
|---|
| Anmerkungen: |
Date Completed 30.09.2021 Date Revised 07.12.2022 published: Print Citation Status MEDLINE |
|---|
| doi: |
10.1080/19336896.2020.1761515 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM309618045 |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM309618045 | ||
| 003 | DE-627 | ||
| 005 | 20231225134318.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231225s2020 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1080/19336896.2020.1761515 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1032.xml |
| 035 | |a (DE-627)NLM309618045 | ||
| 035 | |a (NLM)32378453 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Yang, Jing |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease |
| 264 | 1 | |c 2020 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 30.09.2021 | ||
| 500 | |a Date Revised 07.12.2022 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, incurable, and fatal neurodegenerative disorder. The objective of this study was to describe the clinical features and survival time of Chinese sCJD patients, and to explore the associations between clinical data and survival. In this study, we analysed the clinical data of 21 sCJD patients in a tertiary care hospital and used all Chinese case material available from 152 patients with sCJD in literatures between 2008 and 2018. The mean age of onset of all 173 deceased patients was 61.44 year-olds (y), with the highest incidence in the population of 60 to 69 y. The most common manifestation at disease onset was progressive dementia. With the progression of the disease, the four main clinical symptoms and signs were developed, including myoclonus, visual or cerebella disturbance, pyramidal or extrapyramidal dysfunction, and akinetic mutism. Extrapyramidal symptoms were more frequently observed. The mean survival time was 7.34 months, and 82.10% of cases died within 1 year after disease onset. The follow-up showed that the survival time was longer and the myoclonus sign was more frequently presented in younger-onset sCJD patients. Patients with abnormalities only in cortical regions had a higher frequency of pyramidal dysfunction than patients having lesions in both cortex and basal ganglia. The findings of this study might provide some insight into the clinical characteristics of sCJD patients in China, but further studies could examine the presences of clinical features and survival time in patients with early age of onset in a prospective manner | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Sporadic Creutzfeldt-Jakob disease | |
| 650 | 4 | |a a tertiary care hospital | |
| 650 | 4 | |a clinical manifestation | |
| 650 | 4 | |a literature review | |
| 650 | 4 | |a survival | |
| 700 | 1 | |a Kuang, Haiyan |e verfasserin |4 aut | |
| 700 | 1 | |a Wang, Qiong |e verfasserin |4 aut | |
| 700 | 1 | |a Liu, Jiao |e verfasserin |4 aut | |
| 700 | 1 | |a Chen, Xueping |e verfasserin |4 aut | |
| 700 | 1 | |a Shang, Huifang |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Prion |d 2007 |g 14(2020), 1 vom: 18. Dez., Seite 137-142 |w (DE-627)NLM185323758 |x 1933-690X |7 nnns |
| 773 | 1 | 8 | |g volume:14 |g year:2020 |g number:1 |g day:18 |g month:12 |g pages:137-142 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1080/19336896.2020.1761515 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 14 |j 2020 |e 1 |b 18 |c 12 |h 137-142 | ||