{"id":15206,"date":"2024-06-04T14:49:08","date_gmt":"2024-06-04T18:49:08","guid":{"rendered":"https:\/\/www.bu.edu\/research\/?page_id=15206"},"modified":"2025-09-03T12:14:51","modified_gmt":"2025-09-03T16:14:51","slug":"prions-agent-information-sheet","status":"publish","type":"page","link":"https:\/\/www.bu.edu\/research\/ethics-compliance\/safety\/rohp\/agent-information-sheets\/prions-agent-information-sheet\/","title":{"rendered":"Prions Agent Information Sheet"},"content":{"rendered":"<p><span>Boston University<br \/>\nResearch Occupational Health Program (ROHP)<br \/>\n617-358-7647<\/span><\/p>\n<h2>Agent<\/h2>\n<p><strong>Agent<\/strong><\/p>\n<p>Prion diseases are a variety of neurodegenerative diseases which affect humans, as well as both domestic and wild animal species.\u00a0 In prion diseases, a cellular prion protein (PrP<sup>c<\/sup>) normally found in humans is transformed into a pathogenic isoform (PrPSc) upon exposure to a misfolded prion protein. PrPSc<sup>\u00a0<\/sup>is derived from PrP<sup>c<\/sup> by a series of posttranslational processes whereby PrPSc acquires a high \u00ad<em>beta<\/em>-sheet content.\u00a0 Prions self-propagate and exponentially increase in number, rapidly progressing to kill the host.<\/p>\n<h3>Disease\/Infection<\/h3>\n<p>Five human prion diseases are currently recognized: kuru, Creutzfeldt-Jakob disease (CJD), variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Straussler-Scheinker syndrome (GSS), and fatal familial insomnia.\u00a0 The following are prion diseases that have been found in animals: scrapie, bovine spongiform encephalopathy (BSE), chronic wasting disease, exotic ungulate encephalopathy, feline spongiform encephalopathy, and transmissible mink encephalopathy.<\/p>\n<h3>Pathogenicity<\/h3>\n<p>No increased incidence of Creutzfeldt-Jakob disease (CJD) has been found among pathologists who encounter cases of the disease post-mortem.\u00a0 One occupational health exposure reported from France in 2020.<\/p>\n<ul>\n<li><strong>Special Populations at Risk<\/strong><br \/>\nLab personnel working with prions and sharps.<\/li>\n<\/ul>\n<h3>Biosafety Information<\/h3>\n<p><strong>Risk Group\/BSL<\/strong><br \/>\nRisk Group 2<br \/>\nBiosafety level: BSL2\/ABSL2<\/p>\n<p><strong>Modes of Transmission<\/strong><\/p>\n<p>Prion diseases are transmissible by inoculation by needle, scalpel or ingestion of infected tissues or homogenates.\u00a0 Infectivity is present at high levels in brain and other central nervous system tissues, and at a slightly lower level in lymphoid tissues, such as the spleen, lymph nodes, gut, bone marrow, and blood.\u00a0 Risk of infection from droplets, and exposure of intact skin is not known. There is no evidence of contact or aerosol transmission of prions from human to another human.\u00a0 \u00a0There is a case of CJ disease being transmitted to a lab worker 7 years after exposure.\u00a0 There has been no transmission human to human.<\/p>\n<div class=\"responsive-table\"><table>\n<tbody>\n<tr>\n<td><\/td>\n<td>Transmission<\/td>\n<\/tr>\n<tr>\n<td>Skin Exposure (Needlestick, animal bite, or scratch):<\/td>\n<td>Accidental parenteral inoculation, direct or indirect contact with broken skin<\/td>\n<\/tr>\n<tr>\n<td>Mucous Membrane Exposure Splash to Eye(s), Nose or Mouth:<\/td>\n<td>Unknown<\/td>\n<\/tr>\n<tr>\n<td>Inhalation:<\/td>\n<td>None<\/td>\n<\/tr>\n<tr>\n<td>Ingestion:<\/td>\n<td>Low risk<\/td>\n<\/tr>\n<\/tbody>\n<\/table><\/div>\n<p><strong>Host Range\/Reservoir<\/strong><\/p>\n<p>Prion diseases are found in humans, as well as a variety of domestic and wild animal species, including sheep, goats, cattle, monkeys, chimpanzees, guinea pigs, deer, mice, and cats. The reservoir for prions is the environment and infected animal tissue and carcasses.<\/p>\n<p><strong>Symptoms<\/strong><\/p>\n<p>Symptoms vary based on the particular pathogen, though are primarily neurological:\u00a0 tremors, ataxia, dementia, muscle weakness, postural instability, behavioral changes, sleep disturbances, and dysautonomia.\u00a0 People with prion disease may also have difficulties in concentration, memory, and judgment.<\/p>\n<p><strong>Incubation Period<\/strong><br \/>\n15 months to 2 years for CJD iatrogenic cases.\u00a0 4 to over 20 years for Kuru.<\/p>\n<p><strong>Viability<\/strong><\/p>\n<p>Prions are resistant to inactivation by normal disinfection processes, including irradiation, boiling, dry heat, and chemicals (formalin, betapropiolactone, alcohols). Animal carcasses and other tissue waste can be disposed by incineration with a minimum secondary temperature of 1000\u00b0C (1832\u00b0F).\u00a0 The alkaline hydrolysis process, using a pressurized vessel that exposes the carcass or tissues to 1 N NaOH or sodium hypochlorite 20,000 ppm for 1 hour can be used as an alternative to incineration for the disposal of carcasses and tissues.\u00a0 Sterilization of reusable instruments and decontamination of surfaces should be performed in accordance with recommendations described by the CDC (www.cdc.gov) and the WHO infection control guidelines (www.who.int\/en\/) and BMBL 6th Edition, VIII-H,, Table 4, p.362. These concentrations of disinfectants highly corrosive may cause severe burns to unprotected skin and eyes.<\/p>\n<p><strong>Survival Outside Host<\/strong><\/p>\n<p>Prion diseases can remain infectious after years in the environment.\u00a0 Studies have reported infectivity of prion diseases up to 16 years after improper decontamination.\u00a0 Contaminated electrodes stored in ethanol-formalin for several years were found to cause CJD in chimpanzees.<\/p>\n<h2>Information for Lab Workers<\/h2>\n<h3>Laboratory PPE<\/h3>\n<p>Disposable gowns which close in back, gloves (double nitrile gloves), and eye protection must be used when direct contact with infected materials or animals is unavoidable.\u00a0 Face shields must be used when there is a known or potential risk of exposure to splashes.\u00a0 Extreme care must be taken to avoid accidental autoinoculation or other parenteral inoculations of infectious tissues and fluids.<\/p>\n<h3>Containment<\/h3>\n<p>BSL-2 practices, containment equipment, and facilities are recommended for activities using clinical materials and diagnostic quantities of infectious material.<\/p>\n<h3>In Case of Exposure\/Disease<\/h3>\n<ul>\n<li>For injuries in the lab which are major medical emergencies (heart attacks, seizures, etc\u2026):<br \/>\n<strong>Medical Campus:<\/strong> call or have a coworker call the Control Center at 617-358\u20134144.<br \/>\n<strong>Charles River Campus:<\/strong> call or have a coworker call campus security at 617-353-2121.<br \/>\nYou will be referred to or transported to the appropriate health care location by the emergency response team.<\/li>\n<li>For lab exposures (needle sticks, bite, cut, splash, etc\u2026) involving animals or infectious agents, or for unexplained symptoms or illness call the ROHP 24\/7-hour number (1-617-358-ROHP (7647); or, 8-ROHP (7647) if calling from an on-campus location) to be connected with the BU Research Occupational Health Program (ROHP) medical officer. ROHP will refer you to the appropriate health care location.<\/li>\n<li>Under any of these scenarios, always inform the physician of your work in the laboratory and the agent(s) that you work with.<\/li>\n<li>Provide the wallet-size agent ID card to the physician.<\/li>\n<\/ul>\n<div class=\"important action\">To reach the ROHP directly use the 24\/7-hour number <strong>1-617-358-7647<\/strong> (ROHP).<\/div>\n<p>You will be connected with the BU Research Occupational Health Program (ROHP) medical officer.\u00a0 ROHP will refer you to the appropriate health care location.<\/p>\n<h3>Vaccination<\/h3>\n<p>No vaccine is currently available.<\/p>\n<h2>Information for First Responders\/Medical Personnel<\/h2>\n<h3>Public Health Issues<\/h3>\n<p>All evidence of infection due to human prion diseases are reportable to the Massachusetts Department of Public Health (phone: 617-983-6800 and ask for the Epidemiologist On-call).\u00a0 Health care providers should immediately report to the local board of health (BPHC) where the diagnosis was made.<\/p>\n<h3>Diagnosis\/Surveillance<\/h3>\n<p>Monitor for clinical signs. Prion diseases can be diagnosed with neuroimaging, cerebrospinal fluid analysis, electroencephalogram (EEG), and on post-mortem brain biopsy.<\/p>\n<h3>First Aid\/Post Exposure Prophylaxis<\/h3>\n<p>Any skin contact with infectious materials should be followed by washing with sodium hydroxide for 15 minutes. Rinse well.\u00a0 There is no post exposure prophylaxis for prion diseases.<\/p>\n<p>For the following exposures, perform one of the following actions:<\/p>\n<div class=\"responsive-table\"><table>\n<tbody>\n<tr>\n<td>Skin Exposure (Needlestick or scratch):<\/td>\n<td>Immediately go to the sink and thoroughly wash the wound with soap and water for 15 minutes. Decontaminate any exposed skin surfaces with an antiseptic scrub solution.<\/td>\n<\/tr>\n<tr>\n<td>Mucous Membrane Splash to Eye(s), Nose or Mouth:<\/td>\n<td>Exposure should be irrigated vigorously.<\/td>\n<\/tr>\n<tr>\n<td>Splash Affecting Garments:<\/td>\n<td>Remove garments that may have become soiled or contaminated and place them in a double red plastic bag.<\/td>\n<\/tr>\n<\/tbody>\n<\/table><\/div>\n<h3>Treatment<\/h3>\n<p>No effective treatment has been identified for human prion diseases. Care is mainly supportive.\u00a0 Animal studies suggest that the dye Congo red, anthracyclines, DMSO, glycerol, polyene antibiotics, and copper chelation with penicillamine have shown efficacy in delaying PrPSc accumulation, but none have been tried in human disease.<\/p>\n<h3>References<\/h3>\n<p>Biosafety in Microbiological and Biomedical Laboratories (BMBL) 6<sup>th<\/sup> Edition. Center for Disease Control and Prevention. Last updated June 2020. Section VIII-H: Prion Diseases, p355. <a href=\"https:\/\/www.cdc.gov\/labs\/pdf\/SF__19_308133-A_BMBL6_00-BOOK-WEB-final-3.pdf\">Biosafety in Microbiological and Biomedical Laboratories\u20146th Edition, CDC\/NIH\/HHS<\/a>)<\/p>\n<p>Brown, Henry, and John M Lee. \u201cDiseases of the central nervous system caused by prions.\u201d 16 October 2014. UptoDate.com &lt; https:\/\/www.uptodate.com\/contents\/diseases-of-the-central-nervous-system-caused-by-prions&gt;.<\/p>\n<p>Massachusetts Department of Public Health, Bureau of Communicable Disease Control, Guide to Surveillance, Reporting and Control. \u201cList of Diseases Reportable by all Clinical Laboratories.\u201d 13 June 2018.<\/p>\n<p>\u201cPathogen Safety Data Sheet: Infectious Substances \u2013 Creutzfeldt-Jakob agent, Kuru agent.\u201d Public Health Agency of Canada. Last modified 09 July 2015. &lt;https:\/\/www.canada.ca\/en\/public-health\/services\/laboratory-biosafety-biosecurity\/pathogen-safety-data-sheets-risk-assessment\/creutzfeldt-jakob-agent-kuru-agent.html&gt;.<\/p>\n<p>Saunders, Samuel, et. al. \u201cPrions in the environment: Occurrence, fate, and mitigation.\u201d Prion. 2008 Oct-Dec; 2(4): 162-169. &lt;https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC2658766\/&gt;.<\/p>\n<p>Bandel, JP et al. Variant CJ disease diagnosed 7.5 years after occupational exposure. Correspondence, NEJM 383;1, July 2,2020<\/p>\n<p><strong>\u00a0<\/strong><a href=\"https:\/\/www.cdc.gov\/prions\/\">CDC Prion Diseases<\/a><\/p>\n<p>Revised 3\/4\/25<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Boston University Research Occupational Health Program (ROHP) 617-358-7647 Agent Agent Prion diseases are a variety of neurodegenerative diseases which affect humans, as well as both domestic and wild animal species.\u00a0 In prion diseases, a cellular prion protein (PrPc) normally found in humans is transformed into a pathogenic isoform (PrPSc) upon exposure to a misfolded prion [&hellip;]<\/p>\n","protected":false},"author":4202,"featured_media":0,"parent":5281,"menu_order":48,"comment_status":"closed","ping_status":"closed","template":"","meta":[],"_links":{"self":[{"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/pages\/15206"}],"collection":[{"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/users\/4202"}],"replies":[{"embeddable":true,"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/comments?post=15206"}],"version-history":[{"count":10,"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/pages\/15206\/revisions"}],"predecessor-version":[{"id":58628,"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/pages\/15206\/revisions\/58628"}],"up":[{"embeddable":true,"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/pages\/5281"}],"wp:attachment":[{"href":"https:\/\/www.bu.edu\/research\/wp-json\/wp\/v2\/media?parent=15206"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}