spongiform encephalopathy

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spongiform encephalopathy

disease

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July 14, 2026

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Spongiform encephalopathies are a group of fatal neurodegenerative diseases affecting the brain, characterized by the development of porous, spongelike lesions. These diseases are caused by prions, which are abnormal forms of normally harmless proteins found in the brain. Prions replicate by converting normal proteins into their abnormal shape, leading to the accumulation and damage of nerve cells.123

One example is Bovine Spongiform Encephalopathy (BSE), or "mad cow disease," which affects cattle and was first recognized in the United Kingdom in 1986. Another is scrapie, a similar disease affecting sheep and goats. In humans, Creutzfeldt-Jakob disease (CJD) is a rare and fatal spongiform encephalopathy. A variant form of CJD (vCJD) has been linked to the consumption of tissues contaminated with the prion that causes BSE. There is no known cure for these diseases, and treatment is supportive.423

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Creutzfeldt-Jakob disease
…diseases are types of transmissible spongiform encephalopathies, so called because of the characteristic spongelike pattern of neuronal destruction that leaves brain tissue filled with holes.
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…diseases are types of transmissible spongiform encephalopathies, so called because of the characteristic spongelike pattern of neuronal destruction that leaves brain tissue filled with holes.
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kuru
…diseases sometimes referred to as spongiform encephalopathies because the brains of those with the disease become filled with holes. Spongiform encephalopathies include diseases of humans, such as Creutzfeldt-Jakob disease, and those that affect animals, such as bovine spongiform encephalopathy, also known as mad cow disease.
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…diseases sometimes referred to as spongiform encephalopathies because the brains of those with the disease become filled with holes. Spongiform encephalopathies include diseases of humans, such as Creutzfeldt-Jakob disease, and those that affect animals, such as bovine spongiform encephalopathy, also known as mad cow disease.
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Stanley B. Prusiner
…little-known class of neurodegenerative disorders—the spongiform encephalopathies—that caused progressive dementia and death in humans and animals. In 1974 he set up a laboratory to study scrapie, a related disorder of sheep, and in 1982 he claimed to have isolated the scrapie-causing agent. He claimed that this pathogenic agent, which he…
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…little-known class of neurodegenerative disorders—the spongiform encephalopathies—that caused progressive dementia and death in humans and animals. In 1974 he set up a laboratory to study scrapie, a related disorder of sheep, and in 1982 he claimed to have isolated the scrapie-causing agent. He claimed that this pathogenic agent, which he…
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scrapie
…group of diseases classified as spongiform encephalopathies, so named because the resultant deterioration of neurons causes a spongelike pattern to develop in the brain tissue. The agent responsible for those diseases is an abnormal prion, a deviant form of a benign protein normally found in the brain. In susceptible animals,…
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…group of diseases classified as spongiform encephalopathies, so named because the resultant deterioration of neurons causes a spongelike pattern to develop in the brain tissue. The agent responsible for those diseases is an abnormal prion, a deviant form of a benign protein normally found in the brain. In susceptible animals,…
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