Two elderly patients presented with rapidly progressive dementia and their electroencephalograms showed periodic discharges. Creutzfeldt-Jakob disease was pathologically confirmed in one of the patients. Striking clinical features and characteristic electroencephalograms should prompt the clinical diagnosis in most patients.

A 45 year old woman is reported who initially presented with a cerebellar syndrome, severe ataxia, and dysarthria. She rapidly deteriorated to coma vigile with bilateral myoclonic jerks, flexion rigidity, and immobility necessitating complete nursing. Her EEG showed generalised slow activity and periodic biphasic and triphasic waves. The CSF concentration of neuron specific enolase was very hig...

The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) was established by the Commonwealth Government in October 1993 in response to the recognition of four probable human pituitary hormone related Creutzfeldt-Jakob disease (CJD) deaths. An inquiry into CJD in Australia and the use of human pituitary hormones under the Australian Human Pituitary Hormone Program suggested the expans...

Nation-wide surveillance for transmissible spongiform encephalopathies including Creutzfeldt-Jakob disease (CJD) is undertaken by the Australian National Creutzfeldt-Jakob disease Registry (ANCJDR), based at the University of Melbourne. Surveillance has been undertaken since 1993. During this period the unit has evolved and adapted to changes in surveillance practices and requirements, the emer...

The manufacture of Prolastin®-C (alpha1-proteinase inhibitor [human]) uses a modified process for Prolastin® that incorporates two new dedicated virus reduction steps: a unique solvent/detergent treatment and a small pore nanofiltration. The Prolastin-C manufacturing process was investigated for its capacity to remove or inactivate relevant viruses or models of relevant viruses by evaluating in...

Creutzfeldt-Jakob disease may have many atypical presentations before the development of classic progressive dementia and startle myoclonus. In two patients with pathologically established disease association with a progressive alien hand syndrome was the sole initial manifestation of the disease.

The authors present two biopsy-proved cases of Creutzfeldt-Jakob disease. MR appears to be more sensitive than CT in detecting pathologic changes; signal abnormalities, when found, are predominantly within gray matter and may involve only peripheral cortex.

Bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob Disease (CJD) belong to a group of degenerative neurological disorders collectively known as the transmissible spongiform encephalopathies (TSEs). The group also includes scrapie of sheep and goats, kuru of humans, chronic wasting disease of mule deer and elk and transmissible encephalopathy of mink. These fatal diseases cause behavio...

Human prion diseases can be caused by mutations in the prion protein gene PRNP. Prion disease with mutations at codon 188 has been reported in 6 cases, but only 1 had the T188R mutation and it was not pathologically confirmed. We report the clinical, neuropsychologic, imaging, genetic, and neuropathologic features of a patient with familial Creutzfeldt-Jakob disease, associated with a very rare...