Infectious Diseases, Microbiology & Beneficial Microbes

Prion Diseases

Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of rare, progressive neurodegenerative disorders caused by abnormal forms of proteins called prions. Unlike viruses, bacteria, fungi, or parasites, prions do not contain DNA or RNA Creutzfeldt-Jakob Disease (CJD): The most common prion disease in humans, CJD can occur in sporadic, familial, or acquired forms. Symptoms include rapidly progressive dementia, muscle stiffness, involuntary movements, and eventually, coma and death.Variant Creutzfeldt-Jakob Disease (vCJD): This form of CJD is believed to be caused by consumption of products contaminated with prions, particularly beef contaminated with the agent responsible for bovine spongiform encephalopathy (BSE), also known as "mad cow disease." vCJD has a younger age of onset compared to classical CJD and may present with psychiatric symptoms early in the disease course.Gerstmann-Sträussler-Scheinker Syndrome (GSS): This rare inherited prion disease typically presents with ataxia, dementia, and other neurological symptoms. It is caused by mutations in the prion protein gene (PRNP).Fatal Familial Insomnia (FFI): Another rare inherited prion disease, FFI is characterized by progressive insomnia, autonomic dysfunction, and dementia. It is also caused by mutations in the PRNP gene.There is currently no cure for prion diseases, and they are invariably fatal. Treatment is primarily supportive and focused on managing symptoms and providing comfort care. Prevention of acquired forms of prion diseases involves measures such as avoiding consumption of contaminated meat products and implementing strict infection control measures in healthcare settings to prevent iatrogenic transmission.