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[Human transmissible spongiform encephalopathies]
Ilan Leibovitz1, Doron Zamir, Ilia Polychuck
1Department of Medicine, Barzilai Medical Center, Ashkelon, Ben Gurion University of the Negev.
Abstract:
A new variant of Creutzfeldt-Jakob disease was discovered in 1996. This new variant was found to be related to encephalopathy. There are 5 known human transmissible spongiform encephalopathies, all created by prions. The prion, a pure protein, smaller than a virus is derived from a normal glycoprotein of human cells wall. The function of this protein is unknown but supposed to be connected with copper incorporation into superoxide dismutase. Creutzfeldt-Jakob disease is an early dementive disease with loss of neurons, accumulation of amyloid and spongiform degeneration of the brain. We discuss the epidemiology, diagnosis and therapy of Creutzfeldt-Jakob and its variant. The increased life expectancy will certainly cause these diseases to be more common, thereby having a definite affect on the economy in both developing and developed countries. Future therapy will concentrate on either preventing the creation of pathologic prions or extracting intracellular prions.
Insights
A new variant of Creutzfeldt-Jakob disease (vCJD) linked to encephalopathy emerged in 1996. This prion disease, affecting the brain, is becoming more common due to increased life expectancy, impacting global economies.
Area of Science:
- Neurology
- Prion Science
- Infectious Diseases
Context:
- Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder.
- A novel variant, variant Creutzfeldt-Jakob disease (vCJD), emerged in 1996, linked to bovine spongiform encephalopathy.
- Prions, misfolded proteins, are the causative agents of transmissible spongiform encephalopathies (TSEs).
Purpose:
- To review the epidemiology, diagnosis, and therapy of CJD and vCJD.
- To discuss the nature of prions and their role in human TSEs.
- To explore the potential economic impact and future therapeutic strategies for prion diseases.
Summary:
- vCJD, a new variant of Creutzfeldt-Jakob disease, was identified in 1996 and is associated with encephalopathy.
- Prions, infectious proteins smaller than viruses, cause the 5 known human transmissible spongiform encephalopathies.
- CJD involves neuronal loss, amyloid accumulation, and brain degeneration; its increasing prevalence due to longer lifespans necessitates economic consideration.
Impact:
- Rising incidence of CJD and vCJD due to increased life expectancy will impact global economies.
- Future therapies aim to prevent pathological prion formation or facilitate intracellular prion clearance.
- Understanding prion diseases is crucial for public health and economic planning.