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Sonu Pawar
Sonu Pawar

US Maintains "Negligible Risk" Status for BSE Amidst Atypical Case Detection.

Spongiform Encephalopathy News

Recent news highlights ongoing vigilance and detection efforts related to Bovine Spongiform Encephalopathy (BSE), commonly known as "mad cow disease," both in the United States and internationally.

Low Risk Status Maintained in the US:

The United States Department of Agriculture (USDA) Animal and Plant Health Inspection Service (APHIS) maintains a "negligible risk" status for BSE, as classified by the World Organisation for Animal Health (WOAH). This classification is based on the country's history with the disease – only seven cases detected between 2003 and 2023, with the majority being atypical forms – robust control measures, and a strong surveillance system.

Atypical BSE Cases Detected:

While classical BSE, linked to contaminated feed, remains a concern, recent detections in the US have been classified as atypical BSE. These atypical cases, including one announced in February 2025 involving an older beef cow in South Carolina, are believed to occur spontaneously in older cattle populations at a very low rate and are not expected to impact the US's negligible risk status or cause trade issues. Importantly, the USDA emphasizes that these animals never entered the slaughter channels and posed no risk to the food supply or human health.

Robust Surveillance and Control Measures in the US:

The US has implemented stringent safeguards against BSE, including:

  • Prohibiting mammalian meat-and-bone meal in animal feed.

  • Banning specified risk materials (brain and spinal cord tissues) from the food supply and animal feed.

  • Destroying animals showing BSE signs and those associated with BSE-positive cattle.

  • Banning non-ambulatory cattle from the human food chain.

  • Maintaining a robust BSE surveillance program targeting high-risk cattle populations.

International Occurrences:

Globally, cases of BSE continue to be monitored. In December 2024, a single case of atypical BSE was confirmed on a farm in Scotland. Authorities implemented precautionary movement restrictions and investigations are underway to determine the origin. Similar to the US findings, the Scottish government and Food Standards Scotland confirmed that the affected animal did not enter the human food chain and posed no risk to public health. The rapid detection was attributed to the robust veterinary surveillance system in place.

Understanding Spongiform Encephalopathies:

Spongiform encephalopathies, also known as prion diseases, are a group of progressive, incurable, and fatal neurodegenerative conditions affecting the brain and nervous system of various animals, including humans. These diseases are associated with the prion hypothesis, involving abnormally folded proteins that cause a sponge-like degeneration in brain tissue.

Types of Spongiform Encephalopathies:

  • Human TSEs: Creutzfeldt-Jakob Disease (CJD), Variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann-Straussler-Scheinker Syndrome, Fatal Familial Insomnia, and Kuru. vCJD is linked to the consumption of beef from cattle infected with BSE.   

  • Animal TSEs: Bovine Spongiform Encephalopathy (BSE) in cattle, Scrapie in sheep and goats, Chronic Wasting Disease (CWD) in deer and elk, Transmissible Mink Encephalopathy, and Feline Spongiform Encephalopathy.   

Ongoing Research and Prevention:

Research continues to focus on understanding prion diseases, their transmission, and potential diagnostic and therapeutic approaches. Prevention efforts primarily revolve around stringent animal health regulations, feed controls, and surveillance programs to minimize the risk of BSE and other animal TSEs entering the food chain and potentially impacting human health.

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