Monday, April 16, 2012

Best Post of October 2011: Treatable Neurologic Disorders Misdiagnosed as Creutzfeldt-Jakob Disease

The next in our "Best of the Month" series is from October 17, 2011:

A case of primary CNS angiitis thought to be sCJD
Dr. Mark Cohen and a team of workers at Case Medical Center in Cleveland, Ohio have published an important article in the Annals of Neurology entitled Treatable Neurological Disorders Misdiagnosed as Creutzfeldt-Jakob Disease (Ann Neurol 2011;70:437–444). Why is this article important? Well, because mistaking a survivable, treatable disorder for a fatal, non-treatable disorder is not optimal. Cohen's team reviewed the pathologic diagnoses of 1,106 patients who were referred for potential prion disease to the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University from 2006 to 2009. About one-third of the cases did not have prion disease, with Alzheimer disease and vascular disease being the most common conditions accounting for dementia. Further, about one-quarter of the non-prion cases had treatable diseases, including immune-mediated disorders, neoplastic disorders such as lymphoma, as well as infectious and metabolic disorders. The immune-mediated disorders included primary angiitis of the CNS, acute disseminated encephalomyelitis, limbic encephalitis, neurosarcoidosis, paraneoplastic cerebellar degeneration, and one case of Wegener granulomatosis. Of note, more than half of patients with a treatable dementia had a positive CSF 14-3-3 protein test, highlighting the danger of relying too heavily on this test in making a diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD). It turns out that the most specific test for distinguishing CJD from other diseases was magnetic resonance imaging. Drs. David Perry and Michael Geschwind, in a review of this study in the September 2011 issue of Nature Reviews Neurology, (Perry, D. C. & Geschwind, M. D. Nat. Rev. Neurol. 2011:7, 479–480) write: "The fact that many of the non-prion diagnoses in the present study were potentially treatable RPDs [rapidly progressive dementias] should prompt thorough diagnostic testing in patients who are suspected of having sCJD, in order to rule out mimics. The use of CSF testing, contrast-enhanced MRI, and autoimmune antibody screening is supported by this study."


Theresa Matthews said...

CJD victims at the 2011 CJD Family Conference listened to a presentation on the progress of the development of the RT QUIC screening test. When asked about the potential for when this test would be available for screening. The answer offered up was a weak mumble about the socioeconomic issues. (I'm sorry, I didn't quite understand that comment. What I do understand is a medical community that keeps plugging genetic testing for some and early MRI's for all. If we get into a nitty gritty discussion about the success of current dementia treatment with the drug pipeline emptied and absolutely no drugs in the pipeline for CJD coupled with the recent plug for nicotene patches(tachyarrhythmic) when "insiders" know the best sellers are causing bradycardia and heart block then you can understand how disappointing it is to see this article. With U.S. autopsy rates historically low and the majority of dementia cases never definitively diagnosed, let's hear some honest discussion from top prion experts and AD road mappers.)

The NPDPSC has been government funded for 15 years during which time CWD invaded 20 states and the loopholes in the Feed Ban remained virtually wide open with the USDA testing less than 1% of cattle for BSE and the National Animal Identification System scrapped. The need for robust standardized sterilization techniques of neurosurgical equipment and use of disposable set ups in certain risk settings were never once proposed in the literature by this group...unlike the National CJD Surveillance Unit in the UK which has provided much formal guidance to their practitioners.

To date there have been 5 transfusion transmissions of vCJD. vCJD screening tests are now available. Victims in the UK suspect that screening is already taking place. The U.S. is supposed to be the control group. If this is the case, the debate is about the right to know and we can't even debate it. (A battle reminiscent of early HIV/AIDS victims.)

So in this era of biotech gone bad with drug, biologicals and vaccine production global, I see risk with the ongoing debates that never seem to really define those socioeconomic issues or include those that are paying the price. Should we talk about the world-wide economic collapse and state of public health globally and then review the quality and safety of those products in the 21st century. The farm has not left pharmaceuticals and the human stock is not all that healthy in most areas either.

The NPDPSC was wanting brain only specimens in 2005 in a living study participant. They had the opportunity to study any body fluid or tissues to advance their understanding or prospect of discovering a definitive biological marker and yet they wanted the brain only. In fact, they did not even review or request the MRI or PET scans on this willing participant.

My book review is too little leadership too late with too little published. Even with that critical review offered, if they were sent a temporary lifeline, rest assured it was due to critics as well as supporters.

We can keep focusing on the MRI value or we can get a definitive diagnostic screening tool in place. I can tell you which is cost effective and which is not when Italian prion experts share with me their concern that the illegal growth hormone market is NOT synthetic.

Hope floats with the younger practitioners that realize as important as it is to get the diagnosis right for treatable conditions, it is more of a Global Public Health imperative to be correctly classifying and capturing the rapid, progressive untreatable one that can be comfortably hiding at a rate of 2 to 13% in the other groups.

Terry S. Singeltary Sr. said...

Continuing Enhanced National Surveillance for Prion Diseases in the United States – The CDC announces the availability of fiscal year (FY) 2012 funds for a cooperative agreement program to continue enhanced national surveillance for prion diseases in the United States. The purpose of the funding is to continue an active surveillance program similar to that conducted by the National Prion Disease Pathology Surveillance Center since 1997 to monitor the occurrence of potentially emerging human TSEs in the United States. This program addresses the ?Healthy People 2020? focus area(s) of Immunizations and Infectious Diseases. In 1997, in collaboration with the American Association of Neuropathologists (AANP), the National Prion Disease Pathology Surveillance Center was established to enhance national CJD surveillance and to make possible laboratory investigation of newly emerging prion diseases. This pathology center, located at Case Western Reserve University in Cleveland, Ohio, provided the services of a cutting edge prion disease laboratory, filling a critical public health gap for monitoring prion diseases in humans. Neuropathology reports and submitted brain tissues have been evaluated at the NPDPSC to determine the occurrence of variant CJD and other emerging prion diseases in the United States for approximately 15 years. Eligible applicants should have experience in conducting prion disease surveillance and must be capable of fulfilling the ongoing needs for enhancing such surveillance at the national level. Measurable outcomes of the program will be in alignment with one (or more) of the following performance goal(s) for the National Center for Emerging and Zoonotic Infectious Diseases. This announcement is only for non-research activities supported by CDC. LOI: April 27, 2012. Application: June 11, 2012.

Approximate Current Fiscal Year Funding: $2.55M. Approximate Total Project Period Funding: $12.75M (This amount is an estimate, and is subject to availability of funds.) This includes direct and indirect cost. Approximate Number of Awards: One.

Eligibility: Universities/Colleges, Non-profits

Solicitation #: CDC-RFA-CK12-1208 Issue Date: 4/13/12

?Healthy People 2020? $$$

Figure 1 .


Bovine Spongiform Encephalopathy (BSE) emerged in the 1980s as a disease of cows produced by an aberrant protein (prions). This disease is a food-borne human pathogen producing new variant Creutzfeld-Jakob Disease in people. This event has changed how cattle are fed and the standards of global agricultural trade. The investigation of this disease, and its ultimate solution required an integrated approach by scientists and health policymakers across multiple disciplines. A similar disease, chronic wasting disease (CWD) of elk, deer and moose is spreading in North America. The mechanism of transmission is not understood. There is a risk of spread to cattle and other food animals and ultimately to humans, since the prion protein of CWD can be efficiently converted to a form that apparently overcomes the structural barriers between more distant species. A One Health approach to CWD envisions the convergence of human, veterinary, wildlife disease and research scientists to establish improved surveillance and diagnostic methods, define the transmission chain, risk of cross-species spread, and control strategies.



A budget of $2,978,682 is requested to fund the Initiative for a period of 3 years.

The majority of the budget is for salaries and benefits (45%), contracts (30%) and travel (22%).

Monday, April 16, 2012

Continuing Enhanced National Surveillance for Prion Diseases in the United States

MOM DOD 12/14/97 hvCJD 'confirmed'


kind regards, terry

Terry S. Singeltary Sr. said...

Thursday, April 12, 2012

Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010

Eurosurveillance, Volume 17, Issue 15, 12 April 2012

Research articles

kind regards,

JOHN SMITH said...

Could someone in the Medical Profession please contact me with a opinion on this man's neurological condition

The Harsh Reality of Drug Addiction richardmclaughlin007 — January 18, 2009 — after 11 months of sobriety from drug addiction, in 7 short days this man hits the depths of despair and insanity.

This video’s was shot in Vancouver's downtown eastside by the narrator and is quite extreme, It shows how common place and and readily available drugs are and how people can succumb to a extreme physical reaction from lack of sleep, nutrition and dehydration. This video was made for many different reasons, one being educational the other as mentioned earlier it’s common place here in Vancouver, in any other city or town in North America this man would have received immediate medical attention but here in Vancouver both the police and ambulance just drive by.

This man was spotted two hours later sleeping on a concrete curb as his pillow.

Both the narrator and producer of this video have had spent many years struggling with addiction and have spent hard time in Vancouver's “NOTORIOUS” downtown eastside.

Today they have escaped and are clean and sober and now dedicate there lives to those who still suffer from “THE HARSH REALITY OF ADDICTION”