The photomicrograph shown below is most commonly seen in cases of ?
A. Creutzfeldt-Jakob Disease.
B. Fetal CMV infection.
C. Brain abscess.
D. All of the above.
Ans:A. Creutzfeldt-Jakob Disease.
The photomicrograph shown above represents characteristic spongy change seen in cases of Prions disease.
- They are transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals.
- They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.
- Initially, intracytoplasmic vacuoles appear in neurons. As the disease progresses, vacuolization becomes more pronounced and the cortical neuropil develops a spongy appearance, hence the term spongiform encephalopathy.
- The causative agents of TSEs are believed to be prions.
- The term “prions” refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain.
- Prion diseases are usually rapidly progressive and always fatal.
- Human Prion Diseases:
- Creutzfeldt-Jakob Disease (CJD)
- Variant Creutzfeldt-Jakob Disease (vCJD)
- Gerstmann-Straussler-Scheinker Syndrome
- Fatal Familial Insomnia