Creutzfeldt-Jakob disease accounts for more than 90% of all sporadic prion disease cases. The molecular MM2 genotype has been divided into cortical and thalamic subtypes based on structures involved and is characterized clinically by progressive dementia without ataxia or typical electroencephalography changes. Proposed diagnostic criteria for MM2 cortical type sporadic Creutzfeldt-Jakob diseas...

Molecular typing of the abnormal form of the prion protein (PrP(Sc)) has come to be regarded as a powerful tool in the investigation of the prion diseases. All evidence thus far presented indicates a single PrP(Sc) molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resulting from infection with a single strain of the agent (bovine spongiform encephalopathy). Here w...

Creutzfeldt-Jakob disease is a rare progressive neurological disorder which is eventually fatal. Attention has recently been focused upon the iatrogenic transmission of this disease by four published reports of patients developing Creutzfeldt-Jakob disease associated with the administration of human growth hormone preparations originally prepared from human cadaver pituitaries. Characterized cl...

A periodic EEG pattern very similar to the changes in Creutzfeldt-Jakob disease was seen in a case of anoxic encephalopathy. Necropsy revealed status spongiosus of the cerebral cortex. Generalised repetitive sharp transients in anoxic encephalopathy occur when the patient is comatose and carry a poor prognosis. They are not specific for Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease is a fatal spongiform encephalopathy, which typically presents with a rapidly progressing dementia and additional neurological findings that can be quite variable and diverse. Here we report the unusual case of a patient who presented with left alien limb sign without overt cognitive impairment and was ultimately diagnosed with pathologically confirmed Creutzfeldt-Jako...

Current evidence indicates that variant Creutzfeldt-Jakob disease is caused by the transmission of bovine spongiform encephalopathy to humans. The clinical and investigative features of variant CJD are relatively distinct from sporadic CJD and the neuropathological appearances are novel. The number of cases of vCJD in the UK may have peaked, but the total future number of cases of vCJD is uncer...