Abstract
Creutzfeldt-Jakob disease (CJD) is a rare prion disorder that has been the subject of both professional and public interest following the identification of variant CJD as a zoonotic disorder. There have been recent advances in diagnostic techniques, including real-time quaking-induced conversion and magnetic resonance imaging brain scan, that have allowed more accurate case recognition in all forms of CJD. Although the epidemic of variant CJD is clearly in decline, prevalence studies suggest that it may be premature to be complacent about concerns for public health.
Highlights
Creutzfeldt-Jakob disease (CJD) belongs to a family of fatal degenerative disorders of the nervous system known as transmissible spongiform encephalopathies or prion diseases, which affect both animals and humans[1]
The mechanism for triggering this conformational change is not known, but the accumulation of this abnormal prion protein leads to neuronal degeneration, astrocytic gliosis, and spongiform change, resulting in a uniformly fatal neurological disorder[3]
Positive samples were identified in 19 out of 33,115 appendices, leading to an estimated prevalence of 1 in 4,000 in the general population[27]. This is a matter of concern, as there is no clinical evidence of infection prior to neuro-invasion in prion diseases and the mean incubation period in Variant CJD (vCJD) has been estimated to be 15 years
Summary
Creutzfeldt-Jakob disease (CJD) belongs to a family of fatal degenerative disorders of the nervous system known as transmissible spongiform encephalopathies or prion diseases, which affect both animals and humans[1]. Real-time quaking-induced conversion A number of cerebrospinal fluid (CSF) biomarkers, including 14-3-3, S100b, and tau, have been found to be elevated in patients with CJD These proteins have limited specificity but can be diagnostically useful in the appropriate clinical context[13]. A recipient of an implicated transfusion who died of a non-neurological disorder was found to have PrPSc positivity in the spleen, suggesting pre-clinical infection[26] This individual was a codon 129 heterozygote, and laboratory transmission studies demonstrated prion infectivity in the spleen with characteristics similar to those of primary cases. Positive samples were identified in 19 out of 33,115 appendices, leading to an estimated prevalence of 1 in 4,000 in the general population[27] This is a matter of concern, as there is no clinical evidence of infection prior to neuro-invasion in prion diseases and the mean incubation period in vCJD has been estimated to be 15 years. For example, has shown no evidence of transfusion transmission of a number of neurodegenerative disorders, including Alzheimer’s disease[35]
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