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 ......
Images in Neurology |

Primary Cerebral Angiitis Associated With Amyloid Angiopathy

Amer A. Ghavanini, MD, PhD; David G. Munoz, MD, FRCPC
Arch Neurol. 2011;68(9):1202-1203. doi:10.1001/archneurol.2011.199.
Text Size: A A A
Published online

Extract

A 74-year-old woman had seizure and postictal right-sided weakness. She had a history of hypertension and a remote (>30 years) stroke with complete recovery. Family members reported cognitive changes during the preceding months including aggression, which was unusual for her calm personality. She was disoriented and refused to follow commands. Activities of daily living were severely impaired. Magnetic resonance imaging showed subcortical and cortical edema in the left frontal lobe on T2–fluid-attenuated inversion recovery images and gadolinium enhancement of the covering leptomeninges (Figure 1A and B). Gradient-echo magnetic resonance imaging showed foci of microbleedings limited to the area of edema, sparing the rest of the brain (Figure 1C). Extensive inflammatory investigations including erythrocyte sedimentation rate, antinuclear antibody, and antineutrophil cytoplasmic antibodies yielded negative findings. A biopsy specimen of the affected brain and adjacent leptomeninges (Figure 2) showed mural and perivascular infiltration by lymphocytes, eosinophils, epithelioid macrophages, and multinucleated giant cells associated with nuclear dust and fibrinoid necrosis consistent with primary cerebral angiitis. Congo-red staining demonstrated deposition of amyloid in the vessel walls. Immunostaining with β-amyloid antibody confirmed the β-amyloid nature of deposits in inflamed leptomeningeal and cortical vessels and the presence of numerous cortical amyloid plaques. She received intravenous corticosteroid therapy followed by a combination of oral prednisone and intermittent intravenous cyclophosphamide. Seizure and aggressive behavior were treated with phenytoin and quetiapine, respectively. At 1 month, she had marked radiological improvement (Figure 1D and E). At 6 months, she was calm, oriented, and able to follow commands, read, write, and perform simple activities of daily livings. Relapse following discontinuation of prednisone therapy at 6 months required its reinstatement.

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

Figures

Place holder to copy figure label and caption
Graphic Jump Location

Figure 1. Magnetic resonance images. A, T2–fluid-attenuated inversion recovery (T2-FLAIR) image shows subcortical and cortical hyperintensity in the left frontal lobe. B, Gadolinium-enhanced T1 image shows abnormal enhancement of the leptomeninges covering the areas of T2-FLAIR abnormality (arrowheads). C, Gradient-echo images show multiple foci of microbleeding limited to the areas of T2-FLAIR hyperintensity and sparing the rest of the brain (arrows). Follow-up images 1 month after treatment with high-dose corticosteroids show markedly reduced T2-FLAIR hyperintensity (D), resolved leptomeningeal T1-gadolinium enhancement (E), and unchanged foci of microbleed gradient echo (F).

Place holder to copy figure label and caption
Graphic Jump Location

Figure 2. Brain biopsy. A, Thickening of vessel walls with perivascular and mural infiltration of inflammatory cells and deposition of amorphous eosinophilic material of unknown nature (asterisks) (hematoxylin phloxine saffron). Bar indicates 100 μm. B, The perivascular and mural infiltrate consists of lymphocytes, eosinophils (arrows), and epithelioid macrophages. Deposition of amorphous eosinophilic material (asterisk) is seen (hematoxylin phloxine saffron). Bar indicates 25 μm. C, Hemosiderin-laden macrophages in the perivascular areas are indicative of microbleedings (arrowhead). Bar indicates 25 μm. D, Multinucleated giant-cells (double arrowheads) along with eosinophils (seen in B) are consistent with granulomatous inflammation. Bar indicates 25 μm. E, Martius-scarlet-blue staining shows fibrinoid necrosis of the vascular wall (arrow). Bar indicates 50 μm. F, Congo-red staining (polarized light) demonstrates amyloid deposits (arrows). Bar indicates 50 μm. G, Immunostaining with β-amyloid antibody shows vascular deposits (arrows), as well as plaques within the cortex (arrowhead). Bar indicates 50 μm. H, Higher-powered image shows co-localization of β-amyloid deposits (arrow) and vasculitis. Bar indicates 50 μm.

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.

Multimedia

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

480 Views
3 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
Jobs
brightcove.createExperiences();