No abstract available.
Prion Diseases*

BACKGROUND: Reusable Proseal(TM) laryngeal mask airways (PLMAs) can act as a vector for the transmission of prion diseases such as variant Creutzfeldt-Jacob disease. This study tested the hypothesis that supplementary ultrasonic cleaning facilitates the removal of protein deposits on PLMAs after anesthesia. METHODS: After clinical use, 40 PLMAs were randomly allocated into two groups. In the first group, the PLMAs were washed by hand and were then subsequently placed in an autoclave at 134degrees C for 40 min (Group 1, n = 20). In the second group, the PLMAs were washed by hand and ultrasonic cleaning using an enzymatic solution for 5 min, and were then subsequently placed in an autoclave (Group 2, n = 20). In both groups, protein deposits were detected on PLMAs by erythrosin staining. A staining score designated as none (0%), mild (0-20%), moderate (20-80%) and severe (80-100%), was assigned to each site (outer surface, inner surface and edges of the cuff, airway and drain tube, finger strap) according to the percentage of the stained surface area. RESULTS: Despite the cleaning of the masks, residual protein was found on the outer surface, inner surface and edge of the cuff, airway and drain tube, and finger strap of the PLMAs in both groups. Similar scores were observed for each part of the cleaned PLMAs in both groups, except for the outer surface of the PLMAs in Group 2 (P < 0.05). CONCLUSIONS: We conclude that the use of an ultrasonic cleaner with an enzymatic solution may be effective to cleanse the outer surface of the PLMAs, but there were no differences in the total scores for both groups.
Erythrosine ; Fingers ; Hand ; Laryngeal Masks ; Masks ; Prion Diseases ; Proteins ; Ultrasonics

Animals ; PrPSc Proteins ; metabolism ; pathogenicity ; Prion Diseases ; transmission ; Prions ; physiology

Creutzfeldt-Jakob disease (CJD) is one of the transmissible spongiform encephalopathies, which is mediated by what has been known as "prion". It is a rare and fatal progressive neurodegenerative disease that affects the middle and old aged. There are a number of subtypes of CJD, one of which is the sporadic type characterized by rapidly progressing clinical symptoms, including progressive dementia, myoclonic jerk, and pyramidal or extrapyramidal syndrome. Patients usually end up dying within 1 to 2 years of contacting the disease. We report an autopsy-proven case of sporadic CJD with clinical symptoms that progressed within several days, along with dramatic changes on diffusion weighted magnetic resonance images.
Brain ; Creutzfeldt-Jakob Syndrome ; Dementia ; Diffusion ; Humans ; Myoclonus ; Neurodegenerative Diseases ; Prion Diseases

Human genetic prion diseases (gPrDs) are directly associated with mutations and insertions in the PRNP (Prion Protein) gene. We collected and analyzed the data of 218 Chinese gPrD patients identified between Jan 2006 and June 2020. Nineteen different subtypes were identified and gPrDs accounted for 10.9% of all diagnosed PrDs within the same period. Some subtypes of gPrDs showed a degree of geographic association. The age at onset of Chinese gPrDs peaked in the 50-59 year group. Gerstmann-Sträussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI) cases usually displayed clinical symptoms earlier than genetic Creutzfeldt-Jakob disease (gCJD) patients with point mutations. A family history was more frequently recalled in P105L GSS and D178N FFI patients than T188K and E200K patients. None of the E196A gCJD patients reported a family history. The gCJD cases with point mutations always developed clinical manifestations typical of sporadic CJD (sCJD). EEG examination was not sensitive for gPrDs. sCJD-associated abnormalities on MRI were found in high proportions of GSS and gCJD patients. CSF 14-3-3 positivity was frequently detected in gCJD patients. Increased CSF tau was found in more than half of FFI and T188K gCJD cases, and an even higher proportion of E196A and E200K gCJD patients. 63.6% of P105L GSS cases showed a positive reaction in cerebrospinal fluid RT-QuIC. GSS and FFI cases had longer durations than most subtypes of gCJD. This is one of the largest studies of gPrDs in East Asians, and the illness profile of Chinese gPrDs is clearly distinct. Extremely high proportions of T188K and E196A occur among Chinese gPrDs; these mutations are rarely reported in Caucasians and Japanese.
14-3-3 Proteins/cerebrospinal fluid* ; China ; Creutzfeldt-Jakob Syndrome/genetics* ; Humans ; Mutation/genetics* ; Prion Diseases/genetics* ; Prion Proteins/genetics* ; Prions/genetics* ; tau Proteins/cerebrospinal fluid*

BACKGROUND: The reusable ProSeal(TM) laryngeal mask airways (PLMA's) have the potential to act as a vector for the transmission of prion diseases such as variant Creutzveldt-Jacob disease. This study tested the hypothesis that supplementary compressed air jet cleaning facilitates the removal of protein deposits on PLMA's after surgery. METHODS: After clinical use, thirty PLMA's were randomly allocated to be washed by hand and with an autoclave (134 degrees C for 40 min) (group 1, n = 15), or by hand, autoclave and compressed air jet cleaning (1 min) (group 2, n = 15). In both groups, protein deposits were detected on PLMA's by erythrosine staining. A staining score designated as nil, mild, moderate, and severe was given to each site (outer, inner surface and edges of the cuff, airway and drain tube, finger strap) according to the percentage of stained surface area. The severity of staining was compared for masks prior to use and after cleaning the mask. RESULTS: Despite the cleaning of masks, the staining score worsened on the outer, inner surface and edge of PLMA's in both groups (P < 0.05); however, a similar pattern was observed on each part of a cleaned PLMA for both groups. CONCLUSIONS: We conclude that compressed air jet cleaning for 1 min did not improve the removal of protein deposits on PLMA's after surgery.
Compressed Air* ; Equipment Contamination ; Erythrosine ; Fingers ; Hand ; Laryngeal Masks* ; Masks ; Prion Diseases

Animals ; China ; epidemiology ; Humans ; Prevalence ; Prion Diseases ; epidemiology ; metabolism ; prevention & control ; Prions ; metabolism

Human prion diseases are fatal neurodegenerative disorders that are characterized by spongiform changes, astrogliosis, and the accumulation of an abnormal prion protein (PrP(Sc)). Approximately 10%-15% of human prion diseases are familial variants that are caused by pathogenic mutations in the prion protein gene (PRNP). Point mutations or the insertions of one or more copies of a 24 bp repeat are associated with familial human prion diseases including familial Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia. These mutations vary significantly in frequency between countries. Here, we compare the frequency of PRNP mutations between European countries and East Asians. Associations between single nucleotide polymorphisms (SNPs) of several candidate genes including PRNP and CJD have been reported. The SNP of PRNP at codon 129 has been shown to be associated with sporadic, iatrogenic, and variant CJD. The SNPs of several genes other than PRNP have been showed contradictory results. Case-control studies and genome-wide association studies have also been performed to identify candidate genes correlated with variant and/or sporadic CJD. This review provides a general overview of the genetic mutations and polymorphisms that have been analyzed in association with human prion diseases to date.
Europe ; Far East ; Humans ; Mutation ; Polymorphism, Single Nucleotide ; Prion Diseases/epidemiology/*genetics ; Prions/*genetics

PURPOSE: Through the understanding of the current status of transmissible spongiform encephalopathy(TSE), this study was conducted to contribute to the development of policy and strategy for the control of TSE in Korea in order to keep Korea as a bovine spongiform encephalopathy(BSE)- and variant Creutzfeldt-Jakob disease(vCJD)-free country. BSE and vCJD cases have not been found in Korea. During 2001-2004, the number of patients who have been diagnosed as a definite or probable CJD was 121, which are consisted of 62 male and 59 female(average age: 63 years old). The occurrence of the patients was 5-59 people per year until 2003 and has been gradually increasing due to the recent increase in the diagnostic rate rather than the increase of the incidence. In 2004, the annual occurrence of sporadic CJD(sCJD) in Korea was 1 people per million, which is similar to the average occurrence rate of the world. Two cases of chronic wasting disease(CWD) in deer were found in Chungcheongbuk-do, one in August 2001 and one in October 2001. After that, 4 more CWD-affected deer have been reported in Kyungsangnam-do area in November 2004. We have also examined the possibility that Korean CJD occurred as a result of dietary exposure to BSE. Fortunately, all of Korean CJD patients were not vCJD cases. However, if BSE occurs in Korea, there is a great potential for most of the Korean population to be easily infected with BSE due to their highly susceptible genotype to BSE infection as well as their traditional food habit. In 2003, the total number of people who left Korea was almost identical with the total number of people who entered Korea. However, we could not analyze the number of people who visited or stayed in the UK and Europe during 1980s~1990s, in which BSE was prevalent in Europe, because there was no statistical data available.
Chungcheongbuk-do ; Creutzfeldt-Jakob Syndrome* ; Deer ; Europe ; Food Habits ; Genotype ; Humans ; Incidence ; Korea* ; Male ; Prion Diseases

Genetic prion diseases account for about 10-15% of all cases of human prion disease and are caused by mutations in the prion protein gene. Gerstmann-Sträussler-Scheinker (GSS) disease is a rare genetic prion disease, which is characterized by slowly progressive cerebellar ataxia and the occurrence of cognitive decline in the later stage. P102L is the most common mutation in GSS. We report a patient with a P102L mutation that initially manifested as rapidly progressive dementia without cerebellar symptoms.
Cerebellar Ataxia ; Creutzfeldt-Jakob Syndrome ; Dementia ; Gerstmann-Straussler-Scheinker Disease ; Humans ; Prion Diseases ; Prions