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 ......
Images in Neurology |

Creutzfeldt-Jakob Disease With Focal Electroencephalographic and Magnetic Resonance Imaging Findings

K. Meng Tan, MD; Gregory A. Worrell, MD, PhD; Joseph E. Parisi, MD; Daniel H. Lachance, MD
Arch Neurol. 2007;64(4):600-601. doi:10.1001/archneur.64.4.600.
Text Size: A A A
Published online


A 77-year-old right-handed woman with no prior neurologic abnormalities presented with 2 months of progressive memory loss, aphasia, and upper limb clumsiness. Examination confirmed a severe global cognitive impairment, a predominantly expressive aphasia, bilateral ideomotor apraxia worse on the right side than the left, and an alien right upper limb with myoclonic jerks. Video electroencephalographic recording showed severe delta frequency slowing and periodic sharp waves over the left parietal head region (Figure 1A). The sharp waves were not synchronized with the myoclonic jerks. Brain magnetic resonance imaging demonstrated high-intensity signal within the left parietotemporal cortex on diffusion-weighted and fluid-attenuated inversion recovery sequences, with much less prominent but similar changes contralaterally (Figure 1B). The cerebrospinal fluid neuron-specific enolase level was markedly elevated at 112 ng/mL (reference range, <20 ng/mL). The patient's health continued to deteriorate rapidly and she died 4 weeks later. Autopsy revealed moderate generalized cerebral atrophy and histopathologic findings typical of sporadic Creutzfeldt-Jakob disease (Figure 2). Prion protein Western blot testing at the National Prion Disease Pathology Surveillance Center, Cleveland, Ohio, confirmed the diagnosis.

Figures in this Article

Sign in

Purchase Options

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

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption
Figure 1

Electroencephalographic and magnetic resonance imaging findings. A, Electroencephalography in longitudinal Laplacian montage (left-sided channels above, right-sided channels below) showing periodic left hemispheric sharp waves and delta frequency slowing, maximal in the parietal region. B, Diffusion-weighted magnetic resonance images showing bilateral parietotemporal cortical signal abnormality, maximal on the left (arrows).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2

Histopathological analysis of the cerebral cortex showing spongiform change (arrows) on hematoxylin-eosin staining (A) and positive prion protein immunostaining (arrows) (B) (original magnification ×100).

Graphic Jump Location




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.

Articles Related By Topic
Related Collections
PubMed Articles

The Rational Clinical Examination: Evidence-Based Clinical Diagnosis
Original Article: Does This Patient Have a Torn Meniscus or Ligament of the Knee?

The Rational Clinical Examination: Evidence-Based Clinical Diagnosis