Editorial |

The (Mis)diagnosis of Creutzfeldt-Jakob Disease

Richard J. Caselli, MD
Arch Neurol. 2012;69(12):1554-1555. doi:10.1001/2013.jamaneurol.1.
Text Size: A A A
Published online


I recall, as a young resident in the 1980s, that Creutzfeldt-Jakob disease (CJD) seemed much easier to diagnose than it seems to me now. How can that be? Graying synapses aside, I suspect it reflects the progress that has been made in the past 30 years, particularly in the area of autoimmune encephalopathy. Although CJD at one time seemed to have a monopoly on rapidly progressive dementia, the increasing recognition of autoimmune encephalopathy has made us less confidant about pronouncing the “death sentence.” Patients with autoimmune encephalopathy can be highly responsive to steroids; however, that is not always the case. Paraneoplastic cases typically are not at all responsive and usually end in death. New antibodies continue to be found, making us further wary of the CJD pronouncement only to find, weeks later, that a new antibody not previously sought has been identified. So, increasingly, our attention is focused on not missing reversible causes with somewhat reduced concern for the timely diagnosis of an invariably fatal disease. In this issue of Archives of Neurology, however, Paterson and colleagues1 remind us of perhaps a more traditional perspective.

Figures in this Article

Sign In to Access Full Content

Don't have Access?

Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more

Subscribe for full-text access to content from 1998 forward and a host of useful features

Activate your current subscription (AMA members and current subscribers)

Purchase Online Access to this article for 24 hours

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption
Graphic Jump Location

Figure. Diffusion-weighted images of anoxic-ischemic encephalopathy in a 17-year-old girl demonstrating a cortical ribbon pattern most prominently in occipital-temporal and paracentral cortices.




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.
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.
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment


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

Sign In to Access Full Content

Related Content

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

See Also...
Articles Related By Topic
Related Topics
PubMed Articles
[Doctor Francoise Cathala and history of prions diseases.] Rev Neurol (Paris) Published online Apr 1, 2014.;