Related Experiment Video
Updated: Jun 11, 2026
06:01
Prion Safety Laboratory Swipe Test
Published on: February 14, 2025
Variant Creutzfeldt-Jakob disease.
1National Creutzfeldt-Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK. james.ironside@ed.ac.uk
Summary
Variant Creutzfeldt-Jakob disease (vCJD), linked to bovine spongiform encephalopathy, shows widespread infectivity, including blood. Surveillance is crucial for secondary infections in patients receiving pooled plasma products.
Area of Science:
- Neuroscience
- Infectious Diseases
- Genetics
Background:
- Variant Creutzfeldt-Jakob disease (vCJD) is a human prion disease linked to bovine spongiform encephalopathy.
- Most vCJD cases are in the UK, with a specific genetic profile (methionine homozygosity at codon 129).
- vCJD exhibits widespread infectivity, including lymphoid tissues, raising concerns about bloodborne transmission.
Purpose of the Study:
- To investigate the potential for vCJD transmission through blood products.
- To assess the risk of secondary vCJD infections in patients treated with pooled plasma.
Main Methods:
- Surveillance of vCJD cases and their exposure history.
- Analysis of transfusion and treatment records for patients with bleeding disorders.
- Postmortem examination for evidence of vCJD infection.
Main Results:
- Four probable vCJD infections linked to packed red blood cell transfusions.
- One likely transmission of vCJD via UK factor VIII (FVIII) concentrates in a haemophilic patient.
- Evidence of vCJD infection found in the spleen of an asymptomatic recipient of FVIII concentrates.
Conclusions:
- vCJD can be transmitted through blood products, including packed red blood cells and pooled plasma derivatives like FVIII.
- Widespread infectivity in vCJD supports the risk of secondary transmission.
- Ongoing surveillance is essential to determine the prevalence of vCJD in patients treated with UK-sourced pooled plasma products.
Related Concept Videos
01:27Cryptococcal Meningitis
Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...
01:21Huntington Disease l: Introduction
Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
01:27Cytomegalovirus Disease
Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
01:23Alzheimer Disease ll: Pathophysiology
Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
01:25Arboviral Encephalitis
Arboviral encephalitis refers to brain inflammation caused by arthropod-borne viruses, particularly those transmitted through mosquito vectors. Among these, West Nile virus (WNV), a member of the Flaviviridae family, is a significant public health concern. WNV is an enveloped, positive-sense, single-stranded RNA virus. Human infection typically begins when an infected mosquito introduces the virus into the dermis during feeding. The primary transmission cycle involves birds as amplifying hosts...
01:26Encephalitis ll: Pathophysiology
Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...