(Press-News.org) CHICAGO – A medical record review study of 97 patients with the fatal, degenerative brain disorder sporadic Jakob-Creutzfeldt disease (sCJD) suggests that a correct diagnosis of the disease was often delayed by a variety of misdiagnoses, according to a report published Online First by Archives of Neurology, a JAMA Network publication.
The disease is often misdiagnosed because of a variability of early symptoms and signs, a variability in disease duration and a lack of recognition of the condition in the medical community. Often, sCJD is mistaken for other neurodegenerative conditions such as Alzheimer disease and dementia with Lewy bodies, according to the study background.
Ross W. Paterson, M.R.C.P., and colleagues from the University of California, San Francisco, retrospectively reviewed all cases referred to the UCSF Memory and Aging Center rapidly progressing dementia and CJD clinical research program between August 2001 and February 2007. They identified 97 patients with pathology-proven sCJD for whom they had sufficient medical records (40 women and 57 men who ranged in age from 26 to 83 years).
The 97 patients had received a combined total of 373 alternative diagnoses prior to their diagnosis of likely CJD, with an average of 3.8 misdiagnoses per patient. The physicians who most commonly made the misdiagnoses were primary care physicians and neurologists. In the 18 percent of patients (17 patients) who were correctly diagnosed at their first assessment, the diagnosis was almost always made by a neurologist. The average time from onset to diagnosis was almost eight months, an average of two-thirds the way through the disease course, according to the study results.
"In any patient with a rapidly progressive dementia who has been given multiple potential diagnoses, sCJD must be considered," the authors comment.
Researchers note that "early and accurate" diagnosis of sCJD is valuable for public health reasons and to allow for potential treatments to be tested as early as possible in the disease course.
"It would therefore be valuable to improve early and accurate diagnosis of sCJD premortem to identify at-risk persons, allowing for public health measures that would prevent transmission to healthy individuals through blood donation, infected surgical equipment and or other medical procedures," the authors conclude.
###
(Arch Neurol. Published online September 24, 2012. doi:10.1001/2013.jamaneurol.79. Available pre-embargo to the media at http://media.jamanetwork.com.)
Editor's Note: An author made financial disclosures. The study was supported by the National Institute on Aging, the National Institutes of Health National Institute of Neurological Disorders and Stroke, the Michael J. Homer Family Fund, the National Institutes of Health National Center for Research Resources University of California, San Francisco Clinical and Translational Science Institute and the John Douglas French Alzheimer's Foundation. Please see the article for additional information, including other authors, author contributions and affiliations, financial disclosures, funding and support, etc.
Editorial: (Mis)diagnosis of Disease
In an editorial, Richard J. Caselli, M.D., of the Mayo Clinic Arizona, Scottsdale, writes: "Unquestionably, when confronted with the syndrome of rapidly progressive dementia, our first concern must be the search for reversible causes, a point that Paterson et al discuss in this issue of Archives, but their main point is to illustrate the diagnostic journey patients with CJD travel on their way to a final and very unfortunate diagnosis."
"There is also a societal dimension to the timely diagnosis of CJD. At this time of global economic crisis, national health care reform, escalating medical expenditures and an aging population (creating projections of accelerating health care-driven financial gloom and doom), the financial burden posed by prolonged and duplicative testing that results from diagnostically ambiguous cases of rapidly progressive dementia must also be considered," Caselli continues.
"As we attempt to rein in health care costs while sacrificing no degree of medical accuracy or compassion, we owe it to our patients and to society to have the necessary knowledge to consider the diagnosis, establish the diagnosis as efficiently as possible, and then work with patients, families and palliative care providers to maximize the quality of life for our dying patients," Caselli concludes.
(Arch Neurol. Published online September 24, 2012. doi:10.1001/2013.jamaneurol.1. Available pre-embargo to the media at http://media.jamanetwork.com.)
Editor's Note: Please see the article for additional information, including other authors, author contributions and affiliations, financial disclosures, funding and support, etc.
To contact corresponding author Michael D. Geschwind, M.D., Ph.D., call Jason Bardi at 415-502-4608 or email jason.bardi@ucsf.edu. To contact editorial author Richard J. Caselli, M.D., call Jim McVeigh at 480-301-4222 or email mcveigh.jim@mayo.edu.
Study examines delayed, misdiagnosis of sporadic Jakob-Creutzfeldt disease
2012-09-25
ELSE PRESS RELEASES FROM THIS DATE:
Education, psychological support key for defibrillator patients
2012-09-25
Improved patient education and ongoing psychological support will help people cope with the psychological distress of having an implanted defibrillator, according to a scientific statement from the American Heart Association.
The statement, published in the American Heart Association journal Circulation, is a comprehensive review of the psychosocial and quality of life for people who receive an implantable cardioverter defibrillator (ICD) to restore normal heart rhythm and prevent sudden cardiac death. It includes recommendations for improved patient care and identifies ...
Obesity promotes prostate cancer by altering gene regulation
2012-09-25
Prostate cancer is one of the most common cancers in men and early treatment is usually very successful. However, like other cancers, obesity increases the risk of aggressive prostate disease. New research, published in BioMed Central's open access journal BMC Medicine, finds that the fat surrounding the prostate of overweight or obese men with prostate cancer provides a favorable environment to promote cancer growth.
Fat is a generally underrated organ. Not only is it an energy store but it secretes a wide range of growth factors, cytokines and hormones, including leptin ...
Primate study adds to evidence of BPA harming human reproduction
2012-09-25
PULLMAN, Wash.—A Washington State University researcher has found new evidence that the plastic additive BPA can disrupt women's reproductive systems, causing chromosome damage, miscarriages and birth defects.
Writing in the journal Proceedings of the National Academy of Sciences, WSU geneticist Patricia Hunt and colleagues at WSU and the University of California, Davis, report seeing reproductive abnormalities in rhesus monkeys with BPA levels similar to those of humans. By using an animal with the most human-like reproductive system, the research bolsters earlier work ...
Life in the extreme
2012-09-25
Life in extreme environments – hot acids and heavy metals, for example – can apparently make very similar organisms deal with stress in very different ways, according to new research from North Carolina State University.
One single-celled organism from a hot spring near Mount Vesuvius in Italy fights uranium toxicity directly – by eating the heavy metal and acquiring energy from it. Another single-celled organism that lives on a "smoldering heap" near an abandoned uranium mine in Germany overcomes uranium toxicity indirectly – essentially shutting down its cellular ...
Like prostate cancer, bladder cancer patients may benefit from anti-androgen therapy
2012-09-25
Bladder cancer patients whose tumors express high levels of the protein CD24 have worse prognoses than patients with lower CD24. A University of Colorado Cancer Center study published today in the Proceedings of the National Academy of Sciences shows that CD24 expression may depend on androgens – and that anti-androgen therapies like those currently used to treat prostate cancer may benefit bladder cancer patients.
"This is a major finding – bladder cancer development and spread to other organs depends significantly on CD24, which in turn depends on androgens like testosterone. ...
Scientists reverse Alzheimer's-like memory loss in animal models by blocking EGFR signaling
2012-09-25
Cold Spring Harbor, NY – A team of neuroscientists and chemists from the U.S. and China today publish research suggesting that a class of currently used anti-cancer drugs as well as several previously untested synthetic compounds show effectiveness in reversing memory loss in two animal models of Alzheimer's' disease.
CSHL Professor Yi Zhong, Ph.D., who led the research conducted in fruit flies and mice, says he and his colleagues were surprised with their results, which, he stressed, used two independent experimental approaches "the results of which clearly converged." ...
Most biofuels are not 'green'
2012-09-25
This press release is available in German and French.
In recent years, the demand for supposedly environmentally friendly biofuels has increased significantly worldwide; on the one hand, this has resulted in the increased cultivation of so-called energy plants and, on the other hand, innovative production methods for the second generation of biofuels have been developed. Parallel to this, ecobalance experts have refined and developed methods for environmental assessment. Since biofuels stem predominantly from agricultural products, the, in part, controversial discussion ...
Prostate cancer diagnosis and surgery can lead to anxiety, depression and reduced quality of life
2012-09-25
JACKSONVILLE, Fla. — Men who undergo surgical removal of prostate cancer can experience significant levels of anxiety one year after surgery, and higher levels of anxiety appear to be linked to poor sexual satisfaction and depression, say researchers at Mayo Clinic's campus in Florida. Their recent study, published in the online edition of Psycho-Oncology, suggests that men who experience high levels of "cancer-specific anxiety" following surgery for prostate cancer could likely benefit from counseling designed to address their worries and improve their quality of life.
"The ...
New research shows bees decrease their food intake when given compound found in red wine
2012-09-25
TEMPE, Ariz. – The idea that drinking red wine may provide health benefits – or possibly even extend your life — is an appealing thought for many people. Now, there may be added attraction. Researchers have found that when given resveratrol, a compound found in red wine, bees consume less food.
Previous scientific studies on resveratrol show that it lengthens the lifespan of diverse organisms ranging from unicellular yeast, to fruit flies and mice. Since bees are social animals like humans, a team of scientists from Arizona State University, the Norwegian University ...
Study finds germ-killing power in the eyes
2012-09-25
Berkeley — When it comes to germ-busting power, the eyes have it, according to a discovery by University of California, Berkeley, researchers that could lead to new, inexpensive antimicrobial drugs.
A team of UC Berkeley vision scientists has found that small fragments of keratin protein in the eye play a key role in warding off pathogens. The researchers also put synthetic versions of these keratin fragments to the test against an array of nasty pathogens. These synthetic molecules effectively zapped bacteria that can lead to flesh-eating disease and strep throat (Streptococcus ...