Thursday, September 05, 2013

Possible Patient Exposure to Creutzfeldt-Jakob Disease Announced New Hampshire DHHS

Press Release


Possible Patient Exposure to Creutzfeldt-Jakob Disease Announced


Contact: Public Information Office (603) 271-9391


Publish Date: September 4, 2013


Concord, NH – The New Hampshire Department of Health and Human Services (DHHS), Catholic Medical Center (CMC), and the Manchester Health Department (MHD) announce that neurosurgery on a patient at CMC, who is now suspected to have had Creutzfeldt-Jakob Disease (CJD), may have resulted in the exposure of 8 other neurosurgery patients. The only way to 100% diagnose CJD is through autopsy, which is currently under way at the National Prion Disease Pathology Surveillance Center.


This patient is believed to have sporadic CJD, meaning it happens spontaneously with no known cause. Sporadic CJD is not variant CJD, also known by the nickname "mad cow disease," which is transmitted by eating contaminated beef.


The 8 patients were potentially exposed through neurosurgical equipment, because the prion that causes sporadic CJD is not eradicated by the standard sterilization process mandated at hospitals. CMC has notified all of these patients about their potential risk. The general public and any other patients at CMC and their employees are not at any risk.


"The risk to these individuals is considered extremely low," said Dr. José Montero, Director of Public Health at DHHS, "but after extensive expert discussion, we could not conclude that there was no risk, so we are taking the step of notifying the patients and providing them with as much information as we can. Our sympathies are with all of the patients and their families, as this may be a confusing and difficult situation."


Creutzfeldt-Jakob disease (CJD) is a rare and fatal disease that affects the nervous system and causes deterioration of the brain. It affects about one in a million people each year worldwide. In the United States, only about 200 people are diagnosed with CJD each year.


"Our concern is with the health and well-being of the eight patients who may have been exposed to CJD," says Dr. Joseph Pepe, M.D., President and CEO of CMC. "We will work closely with these families to help them in any way possible, even though the risk of infection is extremely low."


In the early stages of CJD, the patient may have rapidly failing memory and other cognitive difficulties. These symptoms become worse much faster than with other more common types of dementia, such as Alzheimer’s disease. Other symptoms can include personality changes including anxiety and depression, lack of coordination, and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements such as sudden jerky movements, blindness, weakness of extremities, and coma may occur. There is no treatment or cure.


For more information about CJD, visit the Centers for Disease Control and Prevention website at, the World Health Organization at, or the NH Department of Health and Human Services at For concerns or questions, contact the NH Department of Health and Human Services, Bureau of Infectious Disease Control at 603-271-4496, or Catholic Medical Center, Office of Infection Control at 603-663-6374.







Tuesday, July 31, 2012


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Thursday, January 17, 2013


TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)




Tuesday, May 28, 2013


Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance




Sunday, June 9, 2013


TSEAC March 14, 2013: Transmissible Spongiform Encephalopathies Advisory Committee Meeting Webcast




Friday, August 16, 2013


Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates




Sunday, August 11, 2013


Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013


Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010




Sunday, September 1, 2013


Evaluation of the Zoonotic Potential of Transmissible Mink Encephalopathy


We previously described the biochemical similarities between PrPres derived from L-BSE infected macaque and cortical MM2 sporadic CJD: those observations suggest a link between these two uncommon prion phenotypes in a primate model (it is to note that such a link has not been observed in other models less relevant from the human situation as hamsters or transgenic mice overexpressing ovine PrP [28]). We speculate that a group of related animal prion strains (L-BSE, c-BSE and TME) would have a zoonotic potential and lead to prion diseases in humans with a type 2 PrPres molecular signature (and more specifically type 2B for vCJD)




Together with previous experiments performed in ovinized and bovinized transgenic mice and hamsters [8,9] indicating similarities between TME and L-BSE, the data support the hypothesis that L-BSE could be the origin of the TME outbreaks in North America and Europe during the mid-1900s.







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