No abstract available.
Animals ; Cattle ; Encephalopathy, Bovine Spongiform/epidemiology ; Meat/*analysis ; United States

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.
14-3-3 Proteins ; Biopsy ; Brain ; Creutzfeldt-Jakob Syndrome ; Surveys and Questionnaires ; Electroencephalography ; Encephalopathy, Bovine Spongiform ; Humans ; Korea ; Prion Diseases ; Rare Diseases

Bovine spongiform encephalopathy (BSE) is one of the fatal neurodegenerative diseases known as transmissible spongiform encephalopathies (TSEs) caused by infectious prion proteins. Genetic variations correlated with susceptibility or resistance to TSE in humans and sheep have not been reported for bovine strains including those from Holstein, Jersey, and Japanese Black cattle. Here, we investigated bovine prion protein gene (PRNP) variations in Hanwoo cattle [Bos (B.) taurus coreanae], a native breed in Korea. We identified mutations and polymorphisms in the coding region of PRNP, determined their frequency, and evaluated their significance. We identified four synonymous polymorphisms and two non-synonymous mutations in PRNP, but found no novel polymorphisms. The sequence and number of octapeptide repeats were completely conserved, and the haplotype frequency of the coding region was similar to that of other B. taurus strains. When we examined the 23-bp and 12-bp insertion/deletion (indel) polymorphisms in the non-coding region of PRNP, Hanwoo cattle had a lower deletion allele and 23-bp del/12-bp del haplotype frequency than healthy and BSE-affected animals of other strains. Thus, Hanwoo are seemingly less susceptible to BSE than other strains due to the 23-bp and 12-bp indel polymorphisms.
Animals ; Base Sequence ; Cattle ; DNA/genetics ; Encephalopathy, Bovine Spongiform/*genetics ; *Genetic Variation ; Haplotypes ; Prions/*genetics ; Republic of Korea

Clinical manifestations of chronic exposure to organic mercury usually have a gradual onset. As the primary target is the nervous system, chronic mercury exposure can cause symptoms such as fatigue, weakness, headache, and poor recall and concentration. In severe cases chronic exposure leads to intellectual deterioration and neurologic abnormality. Recent outbreaks of bovine spongiform encephalopathy and pathogenic avian influenza have increased fish consumption in Korea. Methyl-mercury, a type of organic mercury, is present in higher than normal ranges in the general Korean population. When we examine a patient with chronic fatigue, we assess his/her methyl-mercury concentrations in the body if environmental exposure such as excessive fish consumption is suspected. In the current case, we learned the patient had consumed many slices of raw tuna and was initially diagnosed with chronic fatigue syndrome. Therefore, we suspected that he was exposured to methyl-mercury and that the mercury concentration in his hair would be below the poisoning level identified by World Health Organization but above the normal range according to hair toxic mineral assay. Our patient's toxic chronic fatigue symptoms improved after he was given mercury removal therapy, indicating that he was correctly diagnosed with chronic exposure to organic mercury.
Animals ; Cattle ; Disease Outbreaks ; Encephalopathy, Bovine Spongiform ; Environmental Exposure ; Fatigue ; Fatigue Syndrome, Chronic ; Hair ; Headache ; Humans ; Influenza in Birds ; Korea ; Nervous System ; Reference Values ; Tuna ; World Health Organization

Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder in which accumulation of the pathogenic prion protein induces neuronal damage and results in distinct pathologic features. This abnormal prion is an infectious protein and resistant to methods of sterilization currently being used. Therefore, management of definite, or suspected CJD patients requires additional precautions. We report our experience of a patient who had undergone brain biopsy for suspected of CJD. The patient was confirmed to have sporadic CJD.
Anesthesia ; Biopsy ; Brain ; Creutzfeldt-Jakob Syndrome ; Encephalopathy, Bovine Spongiform ; Humans ; Neurodegenerative Diseases ; Neurons ; Sterilization

It is one of the frequently utilized strategies for positive-negative selection to elevate the gene targeting efficiency in somatic cells by enriching targeted colonies. Knocking out prnp in animals by gene targeting can prevent it from expressing Prion protein (Pathogenic protein of transmissible spongiform encephalopathy), which enables it to resist infection of Prion. We constructed a bovine prnp biallelic targeting vector via the positive-negative selection strategy, and transfected the linearized vector into the bovine fetal fibroblasts through electroporation. Then, we selected cells in cell culture medium with G418 under a concentration of 600 microg/mL followed by Ganciclovir (GCV) under a concentration of 200 nmol/mL. In the end, we successfully obtained 176 cell clones. All these clones were identified by means of sequencing, immunofluorescence and western blotting, respectively, confirming that there existed 9 positive cell clones. The results showed that the bovine prnp gene was successfully knocked out. Conclusively, we provide an effective way to knockout bovine prnp gene, which could serve as the basis for producing prion protein gene knockout transgenic cloned cattle.
Animals ; Cattle ; Electroporation ; Encephalopathy, Bovine Spongiform ; genetics ; Fetus ; cytology ; Fibroblasts ; cytology ; metabolism ; Gene Knockout Techniques ; methods ; Gene Targeting ; Genetic Vectors ; genetics ; Prions ; genetics ; Transfection

OBJECTIVES: This study was aimed to examine whether participants of a Korean candlelight rally had correct medical information about human mad cow disease and rational attitudes about imported U.S. beef in relation to human mad cow disease. METHODS: A total of 393 face-to-face interviews were conducted, and subjects completed questions about prevalence of senile dementia and human mad cow disease in U.S. and whether they will eat U.S. beef even if no cases of human mad cow disease occurred in the U.S. or if the chance of being affected with human mad cow disease was lower than dying in a plane crash. RESULTS: Correct answer rates to the questions about prevalence of senile dementia and human mad cow disease were 28.2% and 36.1%, respectively. A majority of respondents answered that they would not eat U.S. beef even if there were no reported cases of human mad cow disease in the U.S. or if their chance of being affected with human mad cow disease was lower than dying in a plane crash (75.6% and 86.0%, respectively). CONCLUSION: At least 64.4% of participants had incorrect medical information about human mad cow disease, and their attitudes about imported U.S. beef may be rooted in emotion rather than fact.
Adenine Nucleotides ; Alzheimer Disease ; Animals ; Cattle ; Surveys and Questionnaires ; Encephalopathy, Bovine Spongiform ; Humans ; Korea ; Mycophenolic Acid ; Prevalence

The effect of intravenous recombinant tissue plasminogen activator for patients with Creutzfeldt-Jakob disease (CJD) has yet to be determined. A woman with rapidly progressive cognitive decline, cerebellar dysfunction, and extrapyramidal symptoms, diagnosed as probable sporadic CJD, was complicated with pulmonary thromboembolism and systemic hypotension. Her vital signs were restored without brain parenchymal hemorrhage after intravenous thrombolysis. The presence of diffuse brain lesions in CJD did not increase the hemorrhagic complications associated with intravenous thrombolysis, which differs from an ischemic vascular event.
Brain ; Cerebellar Diseases ; Creutzfeldt-Jakob Syndrome ; Encephalopathy, Bovine Spongiform ; Female ; Hemorrhage ; Humans ; Hypotension ; Pulmonary Embolism ; Thrombolytic Therapy ; Tissue Plasminogen Activator ; Vital Signs

Transmissible Spongiform Encephalopathy (TSE) or prion diseases are fatal neurodegenerative diseases, which are caused by transmissible abnormal prion proteins, converting the endogenous normal prion in the body to the infectious abnormal prions. The most common form of human prion diseases is Creutzfeldt - Jakob disease (CJD). Most of CJD are sporadic with unknown cause. Some familial or iatrogenic CJDs are reported in many countries, but there have been no formally reported case in Korea. Variant CJD (vCJD) is a new form of human prion disease, which revealed differentiated clinical presentations and laboratory diagnostic results. vCJD was thought to be originated from eating the beefs or other parts of bovine spongiform encephalopathy (BSE) infected cattle. The unpredictable species barriers, the underestimated distribution of prion infected tissues, the variable clinical courses, and uncertain disease progressions of many prion diseases, all made the prion related risk assessment very difficult. Korea needs our own surveillance system for various prion diseases of human and animals and to make plans for the risk assessment of the various prion disease transmissions for the minimal spread by maximizing the research capacities.
Animals ; Cattle ; Disease Progression ; Eating ; Encephalopathy, Bovine Spongiform ; Humans ; Korea ; Neurodegenerative Diseases ; Prion Diseases ; Prions ; Risk Assessment

PURPOSE: To report a case of the Heidenhain variant of sporadic Creutzfeldt-Jakob disease (CJD), predominantly characterized by visual impairment at onset. CASE SUMMARY: History-taking, ophthalmologic examination, neurologic examination, cerebrospinal fluid examination including 14-3-3 protein analysis, and brain MRI were performed in a 48-year-old man with progressive visual loss and a visual field defect. These symptoms were accompanied by visual illusion and macropsia. Neurologic examination revealed relatively rapidly progressing cognitive impairment, ataxia, aphasia, and myoclonus. The 14-3-3 protein was detectable in otherwise normal CSF samples. The diffusion weighted brain MRI showed increased signal intensity in both occipital lobes, the basal ganglia, the temporal and frontal lobes. He was clinically diagnosed as having a Heidenhain variant of sporadic CJD. CONCLUSIONS: In a patient with a rapidly progressive visual loss, visual field defects, visual illusion, and neurologic abnormalities including progressive dementia, ataxia, aphasia, and myoclonus, the Heidenhain variant of CJD should be considered. Because prions, a cause of CJD, exhibit unusual resistance to conventional chemical and physical decontamination methods, it is necessary to have an appropriate management scheme to prevent the spread of infection.
14-3-3 Proteins ; Aphasia ; Ataxia ; Basal Ganglia ; Brain ; Creutzfeldt-Jakob Syndrome ; Decontamination ; Dementia ; Diffusion ; Encephalopathy, Bovine Spongiform ; Frontal Lobe ; Humans ; Illusions ; Middle Aged ; Myoclonus ; Neurologic Examination ; Occipital Lobe ; Prions ; Vision Disorders ; Visual Fields