Neurologists’ Awareness and Preparedness on Prion Diseases in Korea
2
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Background: Creutzfeldt-Jakob disease (CJD) is very rare human prion disease. But, neurologists take a key role in diagnosis, surveillance and management of the cases because of its complexity and difficulty in diagnosis of the disease. The aim of this study is to investigate the level of awareness and preparedness of Korean neurologists on this rare disease. Methods: Survey sheets of self-administered questionnaire were given to Korean neurologists who participated in the 31st Annual Meeting of the Koran Neurological Association. Data from 133 respondents were conducted by descriptive analysis. Results: Their answers were as follows: About 62% of neurologists have experienced patients of CJD. Forty-four percent of the patients were confirmed by brain biopsy. Most of neurologists (44%) were not confident to diagnose CJD and the reason why they felt hard to diagnose was due to the variable initial clinical manifestations (45.1%) and the lack of clinical experience (51.9%). Heidenheim variant CJD, proteinase sensitive prionopathy, molecular subtypes of sporadic CJD, diagnostic criteria was not familiar term to Korean neurologists (76.7%, 53.4%,58.6% and 62.4% respectively). Opinion for the most useful diagnostic tool was brain MRI (45.1%), CSF 14-3-3 protein (30.1%), typical EEG finding (36.8%) and gene (PRNP)test (42.9%). And they consider none of them are specific for the diagnosis of CJD (89.5%, 73.7%, 83.5%,91.7%, respectively). Most of the neurologist in this survey answered that the opportunity for education of CJD should be increased (67.7%). Conclusions: Most of neurologists have encountered CJD patients although it is very rare disease. Some of the important and fundamental concepts of CJD were not correctly recognized to Korean neurologists, necessitating a persistent support for updating knowledge and information.
Abstract
Background: Creutzfeldt-Jakob disease (CJD) is very rare human prion disease. But, neurologists take a key role in diagnosis, surveillance and management of the cases because of its complexity and difficulty in diagnosis of the disease. The aim of this study is to investigate the level of awareness and preparedness of Korean neurologists on this rare disease. Methods: Survey sheets of self-administered questionnaire were given to Korean neurologists who participated in the 31st Annual Meeting of the Koran Neurological Association. Data from 133 respondents were conducted by descriptive analysis. Results: Their answers were as follows: About 62% of neurologists have experienced patients of CJD. Forty-four percent of the patients were confirmed by brain biopsy. Most of neurologists (44%) were not confident to diagnose CJD and the reason why they felt hard to diagnose was due to the variable initial clinical manifestations (45.1%) and the lack of clinical experience (51.9%). Heidenheim variant CJD, proteinase sensitive prionopathy, molecular subtypes of sporadic CJD, diagnostic criteria was not familiar term to Korean neurologists (76.7%, 53.4%,58.6% and 62.4% respectively). Opinion for the most useful diagnostic tool was brain MRI (45.1%), CSF 14-3-3 protein (30.1%), typical EEG finding (36.8%) and gene (PRNP)test (42.9%). And they consider none of them are specific for the diagnosis of CJD (89.5%, 73.7%, 83.5%,91.7%, respectively). Most of the neurologist in this survey answered that the opportunity for education of CJD should be increased (67.7%). Conclusions: Most of neurologists have encountered CJD patients although it is very rare disease. Some of the important and fundamental concepts of CJD were not correctly recognized to Korean neurologists, necessitating a persistent support for updating knowledge and information.
참고문헌 (16)
- Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: report of a WHO consultation, , ~ (1998)
- 김혜림, "Dura Mater Graft-Associated Creutzfeldt-Jakob Disease: The First Case in Korea", Journal of Korean Medical Science, 26(11), pp.1515~1517 (2011), 대한의학회
- and introduction of the active surveillance system for CJD patients, , Public Health, 73~80 (2011)
- Haywood AM, "Transmissible spongiform encephalopathies", The New England Journal of Medicine, 337, pp.1821~1828 (1997)
- "National Creutzfeldt-Jakob disease Surveillance Diagnositic Criteria" (2010)[URL]
- Zerr I, "Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease", Brain : a journal of neurology, 132(Pt 10), pp.2659~2668 (2009)
- Vitali P, "Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias", Neurology, 76, pp.1711~1719 (2011)
- Zerr I, "Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease", Neurology, 55, pp.811~815 (2000)
- Steinhoff BJ, "Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease", Annals of neurology, 56, pp.702~708 (2004)
- Sanchez-Juan P, "CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease", Neurology, 67, pp.637~643 (2006)
- Satoh K, "14-3- 3 protein, total tau and phosphorylated tau in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease and neurodegenerative disease in Japan", Cellular and Molecular Neurobiology, 26, pp.45~52 (2006)
- Satoh J, "The 14-3-3 protein detectable in the cerebrospinal fluid of patients with prion-unrelated neurological diseases is expressed constitutively in neurons and glial cells in culture", European Neurology, 41, pp.216~225 (1999)
- Rabinovici GD, "First symptom in sporadic Creutzfeldt-Jakob disease", Neurology, 66, pp.286~287 (2006)
- Will RG, "Acquired prion disease: iatrogenic CJD, variant CJD, kuru", British Medical Bulletin, 66, pp.255~265 (2003)
- Boesenberg C, "Clinical course in young patients with sporadic Creutzfeldt- Jakob disease", Annals of Neurology, 58, pp.533~543 (2005)
- 김상윤, "사람에서 발생하는 프리온 질환들", 대한의사협회지, 51(12), pp.1125~1138 (2008), 대한의사협회