Creutzfeldt-Jakob Disease In Japan Research Articles

Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan

ObjectiveMore than 60% of patients worldwide with Creutzfeldt-Jakob disease (CJD) associated with dura mater graft (dCJD) have been diagnosed in Japan. The remarkable frequency of dura mater grafts in Japan...

Prospective 10-year surveillance of human prion diseases in Japan

We analysed the epidemiological data and clinical features of patients with prion diseases that had been registered by the Creutzfeldt-Jakob Disease Surveillance Committee, Japan, over the past 10 years, since 1999. We obtained information on 1685 Japanese patients suspected as having prion diseases and judged that 1222 patients had prion diseases, consisting of definite (n=180, 14.7%) and probable (n=1029, 84.2%) cases, except for dura mater graft-associated Creutzfeldt-Jakob disease which also included possible cases (n=13, 1.1%). They were classified into 922 (75.5%) with sporadic Creutzfeldt-Jakob disease, 216 (17.7%) with genetic prion diseases, 81 (6.6%) with acquired prion diseases, including 80 cases of dura mater graft-associated Creutzfeldt-Jakob disease and one case of variant Creutzfeldt-Jakob disease, and three cases of unclassified Creutzfeldt-Jakob disease (0.2%). The annual incidence rate of prion disease ranged from 0.65 in 1999 to 1.10 in 2006, with an average of 0.85, similar to European countries. Although methionine homozygosity at codon 129 polymorphism of the prion protein gene was reported to be very common (93%) in the general Japanese population, sporadic Creutzfeldt-Jakob disease in Japan was significantly associated with codon 129 homozygosity (97.5%), as reported in western countries. In sporadic Creutzfeldt-Jakob disease, MM1 type (Parchi's classification) is the most common, as in western countries. Among atypical sporadic Creutzfeldt-Jakob disease cases, the MM2 type appeared most common, probably related to the very high proportion of methionine allele in the Japanese population. As for iatrogenic Creutzfeldt-Jakob disease, only dura mater graft-associated Creutzfeldt-Jakob disease cases were reported in Japan and, combined with the data from previous surveillance systems, the total number of dura mater graft-associated Creutzfeldt-Jakob disease was 138, comprising the majority of worldwide dura mater graft-associated Creutzfeldt-Jakob disease patients. Regarding genetic prion diseases, the most common mutation of prion protein gene was V180I (41.2%), followed by P102L (18.1%), E200K (17.1%) and M232R (15.3%), and this distribution was quite different from that in Europe. In particular, V180I and M232R were quite rare mutations worldwide. Patients with V180I or M232R mutations rarely had a family history of prion diseases, indicating that a genetic test for sporadic cases is necessary to distinguish these from sporadic Creutzfeldt-Jakob disease. In conclusion, our prospective 10-year surveillance revealed a frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease, and unique phenotypes of sporadic Creutzfeldt-Jakob disease and genetic prion diseases related to the characteristic distribution of prion protein gene mutations and polymorphisms in Japan, compared with those in western countries.

Spatial Clusters of Creutzfeldt-Jakob Disease Mortality in Japan between 1995 and 2004

Background: There is suggested to be a geographical difference in Creutzfeldt-Jakob disease (CJD) mortality in Japan. We performed a study to detect localized clusters and hot-spot areas of deaths from CJD in Japan during the 10-year period from 1995 to 2004. Methods: The diagnosis of CJD was taken from the death certificate (coded as A81.0 in the ICD-10). A total number of 1,168 CJD deaths (500 males and 668 females) were used for analysis using empirical Bayes estimates of standardized mortality ratios and the flexible spatial scan statistic to detect clusters. To detect the most likely cluster, p values were obtained using Monte Carlo hypothesis testing (with p < 0.05 as statistical significance).Results: The most likely cluster of CJD mortality was located in the northwest region from the base of Mt. Fuji, stretching over the two neighboring prefectures of Yamanashi and Shizuoka (relative risk = 2.28, p = 0.021). Some other clusters were detected but were not significant. Conclusions: The present study supports the evidence of geographical clustering of deaths from CJD at a specific location in Japan.

Geographic Difference of Mortality of Creutzfeldt-Jakob Disease in Japan

BACKGROUNDThe geographic mortality difference of Creutzfeldt-Jakob disease is still unclear in Japan.METHODSUsing vital statistics of Japan for 6 year period between 1999 and 2004 officially published by the government, we observed the mortality from Creutzfeldt-Jakob disease (ICD-10th: A81.0 and A81.8) by prefecture. Standardized mortality ratios were calculated for the 47 prefectures.RESULTSFor the observed 6 years, a total of 792 deaths from Creutzfeldt-Jakob disease were observed whole in Japan. Two prefectures, Akita and Yamanashi, presented significantly high standardized mortality ratios. In addition, Tochigi, Kochi, and Nagasaki showed standardized mortality rates higher than 1.5 without significance. No prefecture had significantly low standardized mortality ratios.CONCLUSIONSome prefectures with high mortality rate from Creutzfeldt-Jakob disease existed in Japan. Some of them had high incidence rate in a survey conducted in 1996 as well.

A case-control study of Creutzfeldt-Jakob disease in Japan: transplantation of cadaveric dura mater was a risk factor.

A case-control study was conducted to reveal the relative risk of cadaveric dura mater graft transplantation for Creutzfeldt-Jakob disease. Fifty-two cases with Creutzfeldt-Jakob disease that were reported to the surveillance of the disease, and 102 age- and sex-matched hospital controls were selected. Information on family history, occupations, and medical history was collected. Eight cases and no control had a history of cadaveric dura mater graft transplantation. Surgical operations without the dura mater graft, blood transfusion, and acupuncture did not elevate the risk.

Incidence rate of Creutzfeldt-Jakob disease in Japan.

The objective of this study is to clarify the incidence rate of Creutzfeldt-Jakob disease (CJD) during the last decade in Japan. A nationwide mail survey was conducted in all hospitals with a minimum bed capacity of 100 and having at least one of three departments: neurology, psychiatry, and neuropathology. The survey required the patient's sex, date of birth, date of diagnosis, diagnostic criteria, medical history and CJD incidence in the family. From 493 hospitals throughout the country, 821 patients with CJD were reported from January 1985 through March 1996. The annual incidence rate was 0.49 per million population for males and 0.68 for females. The age-specific incidence rate was highest among those 70-79 years of age, followed by the 60-69, and 50-59 age groups. The incidence and mortality increased during the observed period; however, the incidence rate among younger generations did not rise. A nationwide incidence survey of CJD in Japan revealed the incidence and distribution of the disease over the recent decade. It was found that the incidence and mortality rates had increased during the observed period.

Chronological trend of the incidence rate of Creutzfeldt-Jakob disease in Japan

The purpose of this study was to find whether the number of incidents of Creutzfeldt-Jakob disease has increased during the last decade in Japan. The chronological trend was observed by examining the data from a nationwide epidemiologic survey conducted by a special committee sponsored by the Ministry of Health and Welfare of Japan in May 1996. The subjects of the survey were patients with Creutzfeldt-Jakob disease who had been diagnosed between January 1985 and May 1996. Although the number of patients reported each year increased continually during the 11-year 5-month period, there were two issues that remained to cast doubt on the accuracy of this apparently obvious chronological trend: (1) the existence of hospitals that did not respond to the survey, and (2) the existence of hospitals where no information was available before a specific time during the observation period. To make up for the incomplete data, the following two methods were proposed: (1) for those hospitals where the precise annual incidence was not known, the average annual incidence for those years when the information was available should be used to make up for the missing data and (2) when a hospital did not have the information for certain years, it would be considered to be a nonrespondent for those years; the response rate should be calculated year-by-year; then the total number of patients should be obtained by the reported number of patients divided by the response rate. The first method underestimates the annual trend if a trend does exist. To evaluate the trend mathematically, linear regression and quadratic regression models were used, in which the independent variable was the calendar year, the dependent variable, the number of patients. With either method, a trend for increases in incidence was noted. Statistical significance was obtained for the increasing trend in each model. It was found that the quadratic regression model was a better fit than the linear model. The incidence of Creutzfeldt-Jakob disease in Japan has increased during the last decade.

Incidence rate of Creutzfeldt-Jakob disease in Japan

Incidence rate of Creutzfeldt-Jakob disease in Japan

Creutzfeldt-Jakob disease with florid plaques after cadaveric dural graft in a Japanese woman

The Japanese National Committee for Creutzfeldt-Jakob disease (CJD) reported there are 43 patients with CJD in Japan after implantation of cadaveric dura mater, and that no case of new-variant CJD (nvCJD) has been detected. We report a case with similar features to nvCJD. At the age of 38 years, a Japanese woman received a cadaveric dural graft (Lyodura, B Braun Melsungen AG, Germany) after a clipping procedure of the right middle cerebral artery aneurysm in September, 1985. In November, 1994, 110 months after the procedure, she became unsteady and had blurred vision. She was admitted to hospital because of progressing ataxia. Examination disclosed dementia, visual disturbance, hyperreflexia, and cerebellar ataxia. Blood chemistry and cerebrospinal fluid were normal. Brain computed tomography (CT) showed no abnormal lesions. There was slow activities without periodic discharge on her electroencephalogram (EEG). gradually decreased and could not be detected after 40 months. In the second patient, the IgA antibody value remained low. None of the remaining eight patients developed detectable IgA antibodies. Thus far, a total of 1707 infusions have been given and only six (0·4%) mild systemic adverse reactions (headache, nausea, and/or light dizziness) have been noted. Our preliminary experience indicates that prophylactic IgG replacement therapy can safely be given to patients with selective IgA deficiency including those with IgA antibodies by subcutaneous administration. The treatment seems to be effective as the frequency of bacterial respiratory tract infections was reduced. One possible explanation why administration of IgG is effective in IgA deficiency could be that the patients are given additional specific antibodies that they themselves are unable to produce in sufficient amounts. According to the estimated prevalence, the number of IgA deficient individuals is 20 000 in Sweden, 120 000 in UK, and at least 250 000 in the USA. Given that 30% of these have an increased susceptibility to infections, further studies are needed to decide whether to broaden the indications for administration of subcutaneous IgG.

Allelic variation of apolipoprotein E in Japanese sporadic Creutzfeldt-Jakob disease patients

We analyzed apolipoprotein E (apo E) genotypes in 53 Japanese sporadic Creutzfeldt-Jakob disease (CJD) patients and 100 normal controls using the polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) methods. The apo E allelic frequencies in Japanese sporadic CJD patients and our control population were as follows: ϵ2, 6.6% versus 7.5%; ϵ3, 82.1% versus 81.5%; ϵ4, 11.3% versus 11.0%. The mean ages at onset in Japanese sporadic CJD patients were as follows: ϵ3/ϵ3, 66.8 years; ϵ3/ϵ4, 66.3 years; ϵ2/ϵ3, 68.6 years. These results indicate that there is no association between apo E genotype and sporadic CJD in Japan.

Creutzfeldt-Jakob Disease in Japan: An Epidemiological Study Done in a Select Prefecture between 1976 and 1986

An epidemiological study was performed with respect to Creutzfeldt-Jakob disease in a designated area in Japan. The subjects were observed in a small rural prefecture where the population generally remains within a limited radius throughout their lives. The patients' life-styles in each area were investigated in detail. Nine cases appeared in 11 years; 6 were definitive and 3 probable. They were all of the subacute type; there were no noteworthy sexual differences, age of onset, course and/or past histories. Three of the nine cases came from two families; the relationship between familial and isolated cases was examined. Revealed facts and time-space clustering were investigated statistically, but no indication of natural transmission was observable.

Familial Creutzfeldt-Jakob disease in Japan: Three cases in a family with white matter involvement

Three cases of Creutzfeldt-Jakob disease occurring in one family have been clinicopathologically examined. Although the age at onset, duration, and age at death differed for each case, pathological findings, including diffuse neuronal loss, astrocytosis, spongiform changes and patchy and/or diffuse white matter involvement were similar. Life histories and inheritance patterns of the present 3 cases and 2 other families previously reported in Japan are compared with the general findings for familial cases in western countries.

Creutzfeldt-Jakob disease in Japan.

In a nationwide survey of Creutzfeldt-Jakob disease (CJD) in Japan, the point prevalence rate on June 1, 1978 in Fukuoka Prefecture and the estimated national prevalence rate were approximately one per one million population. The minimal period prevalence rate was 0.45 per one million population. The minimal annual incidence and the minimal annual mortality rate were 0.19 and 0.15 per one million population, respectively. The geographic distribution of CJD in Japan was uniform. Clinically, CJD affected the central nervous system diffusely and was rapidly fatal. No specific features were found in family, social, and past histories.