BACKGROUND The geographic mortality difference of Creutzfeldt-Jakob disease is still unclear in Japan. METHODS Using 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. ...

A typical case of Creutzfeldt-Jakob disease is described. Two unusual morphological features-namely, `kuru-like' plaques in the cerebellum and coarsely vacuolated neurones in the striatum-are further similarities between Creutzfeldt-Jakob disease and kuru.

BACKGROUND Prions, the infectious agents responsible for transmissible spongiform encephalopathies, consist mainly of the misfolded prion protein (PrP(Sc)). The unique mechanism of transmission and the appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to consumption of prion-contaminated cattle meat, have raised concerns about public health. Evidence suggests that...

Nation-wide surveillance of transmissible spongiform encephalopathies including Creutzfeldt-Jakob disease, is performed by the Australian National Creutzfeldt-Jakob Disease Registry, based at the University of Melbourne. Surveillance has been undertaken since 1993. Over this dynamic period in transmissible spongiform encephalopathy research and understanding, the unit has evolved and adapted to...

BACKGROUND Nonvasculitic autoimmune inflammatory meningoencephalitis and Creutzfeldt-Jakob disease can present as rapidly progressive encephalopathies with similar clinical features. Slowing of background rhythm is an electroencephalographic characteristic shown by both, but persistent periodic sharp waves are more specific for Creutzfeldt-Jakob disease and have not been reported in nonvasculit...

OBJECTIVE To analyse serum concentrations of brain specific S100 protein in patients with Creutzfeldt-Jakob disease and in controls. DESIGN Prospective case-control study. SETTING National Creutzfeldt-Jakob disease surveillance unit. SUBJECTS 224 patients referred to the surveillance unit with suspected Creutzfeldt-Jakob disease and 35 control patients without dementia. MAIN OUTCOME MEA...

BACKGROUND The value of magnetic resonance imaging of the brain in the diagnosis of iatrogenic cases of Creutzfeldt-Jakob disease has been questioned. OBJECTIVE To illustrate the value of magnetic resonance imaging of the brain in the diagnosis of iatrogenic Creutzfeldt-Jakob disease. METHODS Case report. RESULTS A patient with a history of 3 corneal transplantations exhibited the alien h...

There is now good evidence that the new variant of Creutzfeldt-Jakob disease first identified in 1996 is caused by exposure to bovine spongiform encephalopathy (BSE). Because Creutzfeldt-Jakob disease can be difficult to diagnose, it is possible that cases of sporadic and new variant Creutzfeldt-Jakob disease have occurred that were not correctly diagnosed because postmortem or neuropathologica...

Blood transfusion has been identified as a source of human-to-human transmission of variant Creutzfeldt-Jakob disease. Three cases of variant Creutzfeldt-Jakob disease have been identified following red cell transfusions from donors who subsequently developed variant Creutzfeldt-Jakob disease and an asymptomatic red cell transfusion recipient, who did not die of variant Creutzfeldt-Jakob diseas...