CJD CONFIRMED in patient at New Hampshire Department of Health and Human Services (DHHS), Catholic Medical Center (CMC), and the
Posted Sep 21 2013 11:40am
Test Results in Possible Creutzfeldt-Jakob Disease Case Announced
Contact: Public Information Office (603) 271-9391
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Publish Date: September 20, 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 the test results on a patient who was treated at CMC and who was believed to have had Creutzfeldt-Jakob Disease (CJD) have come back positive from the National Prion Disease Pathology Surveillance Center. The test results were reported to DHHS and CMC earlier today.
Earlier this month, CMC notified 8 neurosurgery patients who may have been exposed to CJD through neurosurgical equipment because the prion that causes sporadic CJD is not eradicated by the standard sterilization process mandated at hospitals. Five other patients in Massachusetts and Connecticut were also potentially exposed. The general public and any other patients at CMC and their employees are not at any risk.
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.
"Though we are not surprised by the test results,” said Dr. José Montero, Director of Public Health at DHHS, “we are saddened by the toll this disease takes on families and our sympathies go out to all those affected.”
"Our focus and concern continues to be with the patients who may have been exposed to CJD,” said Dr. Joseph Pepe, M.D., President & CEO of CMC. This afternoon we have reached out to our 8 patients to let them know about the autopsy results. We let them know we will continue to help and support them and to monitor their health going forward even though the risk is extremely low that any of these patients was infected.”
For more information about CJD, visit the Centers for Disease Control and Prevention website at www.cdc.gov/ncidod/dvrd/cjd/ , the World Health Organization at www.who.int/mediacentre/factsheets/fs180/en/ , or the NH Department of Health and Human Services at www.dhhs.nh.gov . 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.
Oral.05: Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates
Emmanuel Corney,1 Nina Jaffre,1 Jacqueline Mikol,1 Valerie Durand,1 Christelle Jas-Duval,1,2 Sophie Luccantoni-Freire,1 Evelyne Correia,1 Nathalie Lescoutra-Etcheqaray,3 Nathalie Streichenberqer,4 Stephane Haik,5 Chryslain Sumian,3 Paul Brown1 and Jean-Philippe Deslys1
1Commissariat a l'Energie Atomique; Institute of Emerging Diseases and Innovative Therapies (iMETI); Division of Prions and Related Diseases (SEPIA); Fontenay-aux- Roses, France; 2EFS·Nord de France; Lille, France; 3MacoPharm; Tourcoing, France; 4Hospices Civils de Lyon; Prion Unit; Neurobiology Department; Bron, France; 5Inserm; U 975·CNRS; UMR 7225 - Universite Pierre et Marie Curie; Paris, France
Background, Concerns about the blood-borne risk of prion infection have been confirmed by the occurrence in the UK of four transfusion-related infections of vCJD and an apparently silent infection in an hemophiliac patient. Asymptomatic incubation periods in prion diseases can extend over decades in humans. We present here unexpected results of experiments evaluating blood transmission risk in a non-human primate model.
Material and Methods, Cynomolgus macaques were inoculated with brain or blood specimens from vCJD infected humans or monkeys. Neuropathological and biochemical findings were obtained using current methods used for human patients.
Results, Thirteen out of 23 primates exposed to various human or macaque blood products exhibited a previously undescribed myelopathic syndrome, devoid of the classical features of prion disease, notably abnormal prion protein (PrPres) deposition, whereas the 14 corresponding brain-inoculated donor animals and 1 transfused animal exhibited the classical vCJD pattern. In passage experiments, plasma transfusion induced the same atypical phenotype after two years (again, with no detectable PrPres), whereas the intracerebral inoculation of spinal cord led to a typical prion disease with cerebral spongiosis and PrPres accumulation in the brain of the primate recipient. Interestingly, passage experiments in transgenic mice were largely unsuccessful.
In another experiment designed to test the efficacy of antiprion filters, three recipients of filtered red blood cells suspended in plasma are still healthy 4.5 y after transfusion whereas the recipients of unfiltered inocula died after 2.5 y with the atypical neurological profile.
Conclusion. We describe a new fatal neurological myelopathic syndrome in monkeys exposed to various vCJD/BSE-infected blood components.
Secondary transmission in primates confirms
(I) the transmissibility of this myelopathy, and
(2) its prion origin which could not be diagnosed as such in the first recipients.
This myelopathy might be compared in some respects to certain forms of human lower motor neuron disease, including neuromyelitis optica, the flail arm syndrome of amyotrophic lateral sclerosis (ALS), and the recently described FOSMN (facial onset sensory and motor neuronopathy) syndrome.
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 ). 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.
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.