Prion disease

What is Prion disease?

Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of progressive conditions (encephalopathies) that affect the brain and nervous system of many animals, including humans. According to the most widespread hypothesis, they are transmitted by prions, though some other data suggest an involvement of a Spiroplasma infection. Mental and physical abilities deteriorate and myriad tiny holes appear in the cortex causing it to appear like a sponge (hence spongiform) when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function, including memory changes, personality changes and problems with movement that worsen over time. Prion diseases of humans include classic Creutzfeldt–Jakob disease, new variant Creutzfeldt–Jakob disease (nvCJD, a human disorder related to Bovine spongiform encephalopathy), Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, kuru, and the recently discovered Variably protease-sensitive prionopathy. These conditions form a spectrum of diseases with overlapping signs and symptoms.

Unlike other kinds of infectious disease, which are spread by microbes, the infectious agent in TSEs is believed to be a type of protein, called the prion protein. Misshapen prion proteins carry the disease between individuals and cause deterioration of the brain. TSEs are unique diseases in that their aetiology may be genetic, sporadic, or infectious via ingestion of infected foodstuffs and via iatrogenic means (e.g., blood transfusion). Most TSEs are sporadic and occur in an animal with no prion protein mutation. Inherited TSE occurs in animals carrying a rare mutant prion allele, which expresses prion proteins that contort by themselves into the disease-causing conformation. Transmission occurs when healthy animals consume tainted tissues from others with the disease. In recent times, a type of TSE called bovine spongiform encephalopathy (BSE) spread in cattle in an epidemic fashion. This occurred because cattle were fed the processed remains of other cattle, a practice now banned in many countries.

Prions cannot be transmitted through the air or through touching or most other forms of casual contact. However, they may be transmitted through contact with infected tissue, body fluids, or contaminated medical instruments. Normal sterilization procedures such as boiling or irradiating materials fail to render prions non-infective.

Questions and answers on "Prion disease"

I know this might be a little detailed, but my husband has been having a problem within the last 6-8 months now. A little background, he has Crohn...

doctor1 MD

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Your husband needs to be fully evaluated

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Good morning. Thank you for your detailed accounting of things that are not...

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My 12 year old daughter has been having a severe headache that never goes away, nausea and dizziness and sleeping 18-20 per day. The symptoms have...

doctor1 MD

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Could be a migraine or an ADEM

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Hi...by what you quote I feel this could be a migraine or an ADEM - Acute disseminated encephalomyelitis...

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What can you tell me about CJD disease?

doctor1 MD

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Please see details for Creutzfeldt-Jakob Disease (CJD)

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Thank You for contacting HCM. My name is Dr Muhammad...

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What can you tell me about CJD disease?

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I know this might be a little detailed, but my husband has been having a problem within the last 6-8 months now. A little background, he has Crohn...

doctor1 MD

My 12 year old daughter has been having a severe headache that never goes away, nausea and dizziness and sleeping 18-20 per day. The symptoms have...

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