"Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances."
Caused by misfolded proteins, such as Creutzfeldt-Jakob disease.
Prion Biology: This includes the nature of prions, their structure, replication, and transmission.
Prion Diseases: A comprehensive overview of the different types of prion diseases, their symptoms, and pathology.
Contextualizing Prion diseases: Various ways of understanding prion diseases in the context of emerging infectious diseases, zoonosis, and public health implications.
Prion Protein Misfolding & Aggregation: Understanding the mechanisms behind PrPD misfolding and subsequent aggregation leading to toxic disease proteins.
Pathogenesis and Neuropathology: The progression of specific diseases and changes in neuropathology associated with prion diseases.
Epidemiology and Risk Factors: The distribution and prevalence of prion diseases, potential infectious agents, and risk factors.
Diagnostic Methods: Exploration of a wide range of diagnostic methods for identifying and tracking prion diseases, including biochemical assays and imaging studies.
Treatment and Prevention: Available and emerging approaches to treating prion diseases including pharmacological interventions, immunotherapies, and antiviral strategies.
Emerging Related Diseases: Discussion of prion-related diseases that arise from convergent evolution and detection of similar molecular mechanisms i.e., Alzheimer’s diseases.
Future Research Direction and Implications: The likely future research priorities and challenges related to prion diseases, along with broader implications for future global public health policies.
Creutzfeldt-Jakob Disease (CJD): This is the most common human prion disease and affects the brain and nervous system, causing dementia, loss of motor function, and eventually death.
Variant Creutzfeldt-Jakob Disease (vCJD): This rare form of CJD is caused by consuming contaminated beef products and has symptoms similar to CJD.
Gerstmann-Sträussler-Scheinker Syndrome (GSS): This prion disease causes progressive nerve degeneration and dementia, usually starting in mid-life.
Kuru: This now-rare prion disease was once prevalent among the Fore people of Papua New Guinea and was caused by ritualistic cannibalism.
Fatal Familial Insomnia (FFI): This inherited prion disease causes sleeplessness, hallucinations, and eventually death.
Bovine Spongiform Encephalopathy (BSE): Also known as "mad cow disease," this prion disease affects cattle and can be transmitted to humans who consume contaminated beef products.
Scrapie: This prion disease affects sheep and goats and is characterized by weight loss, tremors, and eventually death.
Chronic Wasting Disease (CWD): This prion disease affects deer, elk, and moose and causes weight loss, lack of coordination, and eventually death.
Transmissible mink encephalopathy (TME): This prion disease affects mink and is similar to scrapie in sheep and goats.
Transmissible spongiform encephalopathy of cats (TSE-Cat): This prion disease affects cats and is characterized by behavioral changes, tremors, and eventually death.
Feline Spongiform Encephalopathy (FSE): This prion disease affects cats and is similar to BSE in cattle.
"Later symptoms include dementia, involuntary movements, blindness, weakness, and coma."
"About 70% of people die within a year of diagnosis."
"The name Creutzfeldt–Jakob disease was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob."
"CJD is caused by a type of abnormal protein known as a prion."
"Infectious prions are misfolded proteins that can cause normally folded proteins to also become misfolded."
"About 85% of cases of CJD occur for unknown reasons."
"Inherited CJD accounts for about 10% of prion disease cases."
"Exposure to brain or spinal tissue from an infected person may result in spread."
"There is no evidence that sporadic CJD can spread among people via normal contact or blood transfusions."
"There is no specific treatment for CJD."
"Opioids may be used to help with pain."
"Clonazepam or sodium valproate may help with involuntary movements."
"CJD affects about one person per million people per year."
"The condition was first described in 1920."
"It is classified as a type of transmissible spongiform encephalopathy."
"Sporadic CJD is different from bovine spongiform encephalopathy (mad cow disease) and variant Creutzfeldt–Jakob disease (vCJD)."
"Magnetic resonance imaging may support the diagnosis."
"An electroencephalogram, spinal tap, or magnetic resonance imaging may support the diagnosis."
"Onset is typically around 60 years of age."