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Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of rare and lethal neurodegenerative diseases that affect a great number of mammal species, including humans and animals belonging to [...]

Human prion diseases, also called transmissible spongi-form encephalopathies, are a group of rare and inevitably fatal neurodegenerative diseases. Prion diseases are unique in that they occur as idiopathic (sporadic), familial, [...]

Variant Creutzfeldt-Jakob disease (vCJD) (1) is most likely caused by dietary exposure to bovine spongiform encephalopathy (BSE) prions (2-4). In the United Kingdom, risk for infection with BSE has been [...]

To authenticate to the full-text of this article, please visit this link: http://dx.doi.org/10.1111/j.1552-6569.2008.00294.x Byline: Jason R. Cornelius (1), Christopher J. Boes (1), Gena Ghearing (1), Jacqueline A. Leavitt (1), Neeraj Kumar (1) Keywords: HvCJD; visual symptoms Abstract: ABSTRACT BACKGROUND AND PURPOSE The distinguishing feature in Heidenhain variant Creutzfeldt-Jakob disease (HvCJD) is the presence of visual symptoms preceding the appearance of other clinical manifestations. The purpose of this report is to describe the broad range of visual symptomatology in a patient with HvCJD. METHODS The authors describe the clinical, neuroimaging, and EEG features of a 51-year-old man with progressive visual symptoms that were most likely due to HvCJD. Migraine and epilepsy were initial diagnostic considerations. RESULTS Our patient presented with progressive visual decline and superimposed spells of visual dysfunction that had complex characteristics. Evolution of an abnormal signal in the parieto-occipital cortex on diffusion weighted MRI, changes on EEG, and clinical course were consistent with HvCJD. CONCLUSIONS HvCJD should be considered in all patients who present with unexplained visual phenomena. A remarkable spectrum of visual disturbances can be seen. Close follow-up as well as serial MRI and EEG can help clarify the underlying disease process. Diffusion-weighted and FLAIR sequences should be included in the MRI protocol. Author Affiliation: (1)From the Department of Neurology, Mayo Clinic, Rochester, Minnesota (JRC, CJB, NK); Department of Neurology, University of Pittsburg, Pittsburg, Pennsylvania (GG); and Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota (JAL).[Correction added after online publication 10-June-2009: Received date has been corrected.] Article History: Acceptance: Received March 31, 2008, and in revised form June 30, 2008.Accepted for publication July 9, 2008. Article note: Correspondence: Address correspondence to Neeraj Kumar, MD, Department of Neurology, Mayo Clinic Building E-8 A, 200 First Street SW, Rochester, MN 55905. E-mail: kumar.neeraj@mayo.edu.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.socscimed.2006.08.023 Byline: Kumanan Wilson (a), Ian Graham (b), Maura Ricketts (c), Christopher Dornan (c), Andreas Laupacis (a), Paul Hebert (b) Keywords: Creutzfeldt-Jakob disease; Blood safety; Policy analysis; Risk decision-making; Precautionary principle; Cost-effectiveness; Canada Abstract: The transfusion transmission of hepatitis C and HIV to thousands of Canadian blood recipients was one of this country's largest public health catastrophes. In response to this crisis, and in an effort to prevent such a tragedy from occurring again, the Canadian blood system has undergone substantial reform. Variant Creutzfeldt-Jakob (vCJD) disease was the first infectious threat faced by the blood system since undergoing reform. The response at the time to this risk provides insights into the Canadian blood system's new approach to infectious threats. Our analysis of the decision-making concerning vCJD identifies two dominant themes characterizing the new blood system's approach to safety: (1) the adoption of a precautionary approach to new risks which involves taking action in advance of definitive evidence, and (2) risk aversion amongst policy makers, which has contributed to the adoption of safety measures with comparatively high cost-effectiveness ratios. Overall the principles governing the new blood system have contributed to the system both providing protection against emerging infectious risks and regaining the confidence of the public and recipients. However, the current set of policy factors will likely contribute to increasingly risk-averse policy making that will contribute to continued increases in the cost of the blood system. The challenge the blood system now faces is to find the appropriate balance between maximizing safety and ensuring the system remains affordable. Author Affiliation: (a) University of Toronto, Toronto, Ont., Canada M5G 2C4 (b) University of Ottawa, Canada (c) Carleton University, Canada

The transfusion transmission of hepatitis C and HIV to thousands of Canadian blood recipients was one of this country's largest public health catastrophes. In response to this crisis, and in an effort to prevent such a tragedy from occurring again, the Canadian blood system has undergone substantial reform. Variant Creutzfeldt-Jakob (vCJD) disease was the first infectious threat faced by the blood system since undergoing reform. The response at the time to this risk provides insights into the Canadian blood system's new approach to infectious threats. Our analysis of the decision-making concerning vCJD identifies two dominant themes characterizing the new blood system's approach to safety: (1) the adoption of a precautionary approach to new risks which involves taking action in advance of definitive evidence, and (2) risk aversion amongst policy makers, which has contributed to the adoption of safety measures with comparatively high cost-effectiveness ratios. Overall the principles governing the new blood system have contributed to the system both providing protection against emerging infectious risks and regaining the confidence of the public and recipients. However, the current set of policy factors will likely contribute to increasingly risk-averse policy making that will contribute to continued increases in the cost of the blood system. The challenge the blood system now faces is to find the appropriate balance between maximizing safety and ensuring the system remains affordable. Keywords: Creutzfeldt-Jakob disease; Blood safety; Policy analysis: Risk decision-making; Precautionary principle: Cost-effectiveness; Canada

There is no clear cause of sporadic Creutzfeldt-Jakob disease despite intensive research. This is a neurologic disease that can ultimately lead to dementia. It belongs to a class of diseases called spongiform encephalopathies because they cause small holes in the brain, making it look like a sponge. They are also called prion diseases, because this brain damage is believed to be caused by a natural brain protein that somehow becomes abnormal and is then called a prion. Although bovine spongiform encephalopathy, or mad cow disease, may be transmitted through meat consumption, there is no evidence that Creutzfeldt-Jakob disease is transmitted in this way.