0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
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 ......
Comment & Response |

Creutzfeldt-Jakob Disease

Felix F. Benninger, MD, PhD1; Israel Steiner, MD1
[+] Author Affiliations
1Department of Neurology, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
JAMA Neurol. 2013;70(12):1588-1589. doi:10.1001/jamaneurol.2013.4412.
Text Size: A A A
Published online

Extract

To the Editor In their article, Angus-Leppan et al1 presented a comprehensive and highly interesting report of a 68-year-old patient exhibiting a rapidly progressing array of symptoms including insomnia, personality change, myokymia, and eventually cognitive decline. As concluded by the authors, Morvan syndrome2 was suspected, and indeed, serum antibodies to the voltage-gated potassium channel complex were found. Brain biopsy findings were conclusive, with definite Creutzfeldt-Jakob disease (CJD). The combination of CJD and antibodies indicating a possible treatable immune-mediated encephalopathy is intriguing and raises concern of how to proceed with patients considered to have CJD especially as this combination does not seem to be a singularity. In 2012, we cared for a 67-year-old patient presenting with a 3-week rapidly progressive personality change and cognitive decline, as well as gait instability. Initially talkative, mildly disoriented, and ataxic, his condition deteriorated rapidly and he became bed bound, dysarthric, and dysphatic after 2 weeks; as time went on, he became mute and without any reaction to outside stimulus. No metabolic disturbances, besides known chronic renal failure, were detected. Repeated magnetic resonance imaging scans using diffusion-weighted imaging showed progressive extension of cortical hyperintensity bilateral parietooccipital with cortical ribbon sign highly compatible with the diagnosis of CJD.3 On electroencephalography, diffuse slowing without periodicity was seen. Results from repeated lumbar punctures did not show pleocytosis or elevated protein. Findings from neoplastic screening, including full-body positron emission tomography–computed tomography, were negative. Serum and cerebrospinal fluid antibody testing results were positive in 2 separate laboratories for Caspr-2 at a titer of 1:32. A course of high-dose steroids and plasmapharesis did not change the progression of the disease, and the patient died 4 weeks after hospital admission. The family refused brain biopsy or postmortem analysis. In concert with Angus-Leppan et al, the probability of 2 rare diseases in our patient was extremely low and magnetic resonance imaging did not support a diagnosis other than CJD. Unmasking of epitopes due to rapid neurodegeneration and secondary antibody appearance might explain the concurrent findings, but we argue that the low titer in both cases make it more probable that the antibodies were false positive and not involved in the disease pathophysiology in accord with their detection in control populations.4 Future research is urgently needed to clarify the relationship between the occurrences of antineural antibodies in CJD, especially to formulate screening and treatment guidelines and define cutoffs for antibody titers. Thus, more patients with rapid progressive dementia could be helped effectively, while others are spared from strenuous and highly expensive, yet ineffective, treatment.

Topics

Sign in

Purchase Options

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

First Page Preview

View Large
First page PDF preview

Figures

Tables

References

Correspondence

December 1, 2013
Heather Angus-Leppan, MD, FRCP; Peter Rudge, FRCP; Simon Mead, PhD; John Collinge, MD, FRCP, FRS; Angela Vincent, FRCPath
1Department of Clinical Neurosciences, Royal Free Hospital, London, England
2MRC Prion Unit, University College London Institute of Neurology, London, England
3University of Oxford, Nuffield Department of Clinical Neurosciences, Oxford, England
JAMA Neurol. 2013;70(12):1589. doi:10.1001/jamaneurol.2013.4777.
CME
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.

Multimedia

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

718 Views
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...
Jobs
brightcove.createExperiences();