We serially examined a patient with Creutzfeldt-Jakob disease using fludeoxyglucose F 18 and positron emission tomography. Marked cerebral hypometabolism reflected clinical deterioration in the early stages of Creutzfeldt-Jakob disease when no parenchymal abnormalities were present on MR imaging.

OBJECTIVE To report the clinical, neuropsychological, linguistic, imaging, and neuropathological features of a unique case of sporadic Jakob-Creutzfeldt disease in which the patient presented with a logopenic variant of primary progressive aphasia. DESIGN Case report. SETTING Large referral center for atypical memory and aging disorders, particularly Jakob-Creutzfeldt disease. PATIENT Pat...

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. ...

Four members of a kindred wi th Creutzfeldt-Jakob disease are reported, i n whom myoclonus d id not develop and in whom serial electroencephalograms performed late in their illness failed to show periodic sharp wave complexes. Otherwise, the patients’ disease duration, clinical features, and neuropathological findings were similar to those described in sporadic cases of Creutzfeldt-Jakob diseas...

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 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...

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...

BACKGROUND In patients with sporadic Creutzfeldt-Jakob disease, pathologic disease-associated prion protein (PrPSc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrPSc in Creutzfeldt-Jakob disease is important for classification and diagnosis and perhaps even for prevention. METHODS We used a highly sensitive method of det...