aCJD

aCJD, or acquired Creutzfeldt-Jakob disease, is a rare and fatal neurodegenerative disorder belonging to the family of transmissible spongiform encephalopathies (TSEs). Unlike its genetic counterpart, sporadic Creutzfeldt-Jakob disease (sCJD), aCJD arises from exposure to prion proteins, which are misfolded versions of a normal cellular protein. These abnormal prions accumulate in the brain, leading to progressive brain damage and characteristic spongiform changes—hence the term "spongiform encephalopathy."

The transmission of aCJD typically occurs through exposure to contaminated medical instruments, tissues, or blood products. Historically, cases were linked to human growth hormone derived from cadaveric pituitary glands or dura mater grafts. More recently, concerns have emerged regarding potential transmission through blood transfusions or surgical procedures involving contaminated instruments. While rare, documented cases have also involved consumption of contaminated beef products, particularly in the context of variant Creutzfeldt-Jakob disease (vCJD), a subtype associated with bovine spongiform encephalopathy (BSE).

Symptoms of aCJD usually develop gradually and may include rapid cognitive decline, memory loss, behavioral changes, and motor dysfunction. Neurological signs such as myoclonus (involuntary muscle jerks), ataxia (loss of coordination), and visual disturbances are common. Diagnosis relies on clinical evaluation, brain imaging, and cerebrospinal fluid analysis, though definitive confirmation often requires brain biopsy or autopsy.

There is currently no cure for aCJD, and the disease progresses rapidly, typically resulting in death within months of symptom onset. Prevention focuses on strict infection control measures in healthcare settings, screening of blood and tissue donors, and adherence to guidelines for handling potentially contaminated materials. Research into therapeutic interventions remains ongoing, though no effective treatments have been established to date.