Human prion diseases like Creutzfeldt-Jakob disease are infectious, inherited, or sporadic neurodegenerative disorders, characterized by the accumulation of an abnormal isoform of the host-encoded prion protein. This affects nervous tissue in sporadic Creutzfeldt-Jakob disease and, additionally, in lymphoid tissue in bovine spongiform encephalopathy-linked variant Creutzfeldt-Jakob disease. Exp...

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...

This CHMP position statement replaces the CHMP position statement on Creutzfeldt-Jakob disease and plasma-derived and urine-derived medicinal products (EMEA/CPMP/BWP/2879/02/rev 1). CHMP position statement on Creutzfeldt-Jakob disease and plasma-derived and urine-derived medicinal products

The correlation between the appearance of the characteristic electroencephalographic abnormality, cardinal clinical manifestations, and neuropathological features was studied in four cases of Creutzfeldt-Jakob syndrome consisting of subacute spongiform encephalopathy and classical Creutzfeldt-Jakob disease. Consideration is given to the differences in the electroencephalographic findings betwee...

We report the presence of infectivity in erythrocytes, leukocytes, and plasma of 1 person with variant Creutzfeldt-Jakob disease and in the plasma of 2 in 4 persons whose tests were positive for sporadic Creutzfeldt-Jakob disease. The measured infectivity levels were comparable to those reported in various animals with transmissible spongiform encephalopathies.

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...

BACKGROUND Involvement of the peripheral nervous system in the pathogenesis of prion diseases is becoming increasingly evident. However, pathologic protease-resistant prion protein deposition in the peripheral nerves of patients with Creutzfeldt-Jakob disease has never been demonstrated, to our knowledge. OBJECTIVE To determine whether mutated prion protein accumulation could be shown in the ...

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...

OBJECTIVE To describe clinical features and diagnostic tests of the MM2 cortical subtype in sporadic Creutzfeldt-Jakob disease. METHODS Clinical symptoms, magnetic resonance imaging studies, electroencephalograms, and cerebrospinal fluid markers were studied in 12 patients with genetically and neuropathologically verified sporadic Creutzfeldt-Jakob disease. Histological findings were semiquan...