We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Editorial |

Cerebrospinal Fluid Total Prion Protein A Potential In Vivo Marker of Cerebral Prion Pathology

Henrik Zetterberg, MD, PhD1,2
[+] Author Affiliations
1Clinical Neurochemistry Laboratory, Institute of Neuroscience and Physiology, the Sahlgrenska Academy at the University of Gothenburg, Mölndal, Sweden
2UCL Institute of Neurology, Queen Square, London, England
JAMA Neurol. 2015;72(3):261-263. doi:10.1001/jamaneurol.2014.4078.
Text Size: A A A
Published online


Several human degenerative diseases appear as a result of the misfolding and aggregation of proteins.1 The prototype central nervous system proteinopathy is Creutzfeldt-Jakob disease (CJD), in which neuronal prion protein (PrP) with high α-helical content switches into a stable structure rich in β-pleated sheets in a self-catalyzing process that eventually, after a prolonged build-up phase, turns neurotoxic, causing a plethora of neurological and psychiatric symptoms.2 Although human prion diseases are rare, affecting around 1 to 2 people per million individuals each year,3 they are frequent differential diagnoses when patients present with clinical signs of rapidly progressive central nervous system disease. Reliable tools for diagnosis are needed because prion diseases are transmissible, which became clear in the 1930s when healthy sheep were inoculated with brain tissue from sheep affected by scrapie (a sheep prion disease) and fell ill following an incubation period of a few years.4 The transmissibility was further emphasized during the 1980s epidemic of bovine spongiform encephalopathy, a prion disease of cattle in the United Kingdom,5 which eventually also made it clear that bovine spongiform encephalopathy could be transmitted to humans in the form of variant CJD, typically affecting younger individuals showing prion aggregation in peripheral tissues as well as in the central nervous system.68 Clinical, epidemiological, and experimental research has demonstrated that prions are transmissible by multiple routes, may have long incubation periods, and are resistant to conditions that would inactivate most previously recognized pathogens, such as formalin treatment and heat denaturation.

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

First Page Preview

View Large
First page PDF preview





Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.


Some tools below are only available to our subscribers or users with an online account.

0 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

Customize your page view by dragging & repositioning the boxes below.

See Also...
Articles Related By Topic
Related Collections
PubMed Articles

Care at the Close of Life: Evidence and Experience
Alzheimer Disease: "It's OK, Mama, If You Want to Go, It's OK"

The Rational Clinical Examination: Evidence-Based Clinical Diagnosis
Supplemental Content