A 68 year old woman with sporadic Creutzfeldt-Jakob disease is described, who neither showed characteristic EEG abnormalities nor a positive test of the neuronal protein 14-3-3 or neuron specific enolase (NSE) in CSF, despite a clinical presentation with ataxia of cerebellar type, rapidly progressive dementia, myoclonus, and marked hyperintense signal abnormalities in the deep cortical layers a...

New variant Creutzfeldt-Jakob disease may be associated with exposure to the causative agent of bovine spongiform encephalopathy. Currently, a reliable diagnosis is possible only after neuropathological examination of the brain, which is risky for patients and diagnosticians. The sensitivity and specificity of recently developed techniques are not known for new variant Creutzfeldt-Jakob disease...

The case of a young man who had previously received pituitary derived growth hormone for treatment of radiation induced growth hormone deficiency is reported. He underwent neurosurgery for presumed recurrence of a posterior fossa tumour but was subsequently shown to have Creutzfeldt-Jakob disease, confirmed on necropsy. The risk of transmission of Creutzfeldt-Jakob disease by neurosurgical inst...