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 ......
Clinical Pathologic Conference |

Progressive Neuropsychiatric Symptoms and Motor Impairment

Mahtab Ghadiri, MBBS, BMedSc1; Michael E. Buckland, MBBS, PhD, FRCPA1,2; Ian J. Sutton, PhD, FRACP3; Suad Al Jahdhami, BSc, MD, FRCPA1,2; Simon Flanagan, BSc, MSc2,4; Robert Heard, MD, PhD, FRCP, FRACP5; Yael Barnett, MBBS1; Jeffrey Brennan, BSc, MBBS, FRACS6; Michael H. Barnett, MBBS, PhD, FRACP1,7
[+] Author Affiliations
1Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia
2Discipline of Neuropathology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
3Department of Neurology, St Vincent’s Hospital, Sydney, New South Wales, Australia
4Department of Pathology, University of Sydney, Sydney, New South Wales, Australia
5Westmead Millennium Institute, University of Sydney, Sydney, New South Wales, Australia
6Department of Neurosurgery, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
7Institute of Clinical Neurosciences, Department of Medicine, University of Sydney, Sydney, New South Wales, Australia
JAMA Neurol. 2014;71(6):794-798. doi:10.1001/jamaneurol.2013.6308.
Text Size: A A A
Published online

A 42-year-old white man presented with cognitive impairment and behavioral changes followed by rapidly progressive motor and gait impairment. Magnetic resonance imaging revealed striking multifocal white matter signal change, areas of restricted diffusion, diffuse callosal signal change, and atrophy and hyperintensity of the corticospinal tracts. A broad range of etiologies warrant consideration in this case, including degenerative, vascular, inflammatory, metabolic, and neoplastic diseases.

Figures in this Article

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

First Page Preview

View Large
First page PDF preview

Figures

Place holder to copy figure label and caption
Figure 1.
Magnetic Resonance Imaging Findings

An axial fluid-attenuated inversion recovery (FLAIR; [A]) image, a diffusion-weighted imaging (DWI; [B]) scan, and an apparent diffusion coefficient (ADC) map (C) at the level of the centrum semiovale are shown. A, The FLAIR image demonstrates extensive subcortical white matter hyperintensities (circle). B, Many of these areas show a high signal on the DWI scan (circle). C, The ADC map shows corresponding areas that are predominantly of high signal representing facilitated diffusion, but it also reveals islands of hypointensity signifying true restricted diffusion (circle). D, An axial FLAIR image through the lateral ventricles reveals confluent involvement of the genu and splenium of the corpus callosum and generalized cerebral atrophy. E, A sagittal FLAIR image reveals hyperintensity of the corpus callosum and marked callosal atrophy. F, An axial FLAIR image at the level of the midbrain reveals hyperintensity of the corticospinal tracts bilaterally (arrowheads).

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

A, Deep white matter showing rarefaction, macrophage infiltration, and gliosis (hematoxylin-eosin, original magnification ×400). The pigmented macrophages are indicated by arrowheads. B, Luxol fast blue staining for myelin reveals marked myelin loss (original magnification ×400). C, Axonal loss and spheroid formation are revealed by immunohistochemistry for a low-molecular-weight neurofilament (original magnification ×630). D, Immunohistochemistry for CD68 reveals the macrophage infiltration (original magnification ×400). Many macrophages stain positively with the Perls iron stain (left inset) and/or the prolonged Ziehl-Neelsen stain (right inset).

Graphic Jump Location

Tables

References

Correspondence

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.
Submit a Comment

Multimedia

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

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

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

Articles Related By Topic
Related Collections
Jobs
JAMAevidence.com
brightcove.createExperiences();