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 ......
Research Letter |

Atypical Lymphocytic Angiitis of the Optic Nerve and Central Nervous System

Eleonora M. Lad, MD, PhD1; Christine M. Hulette, MD2; Alan D. Proia, MD, PhD2
[+] Author Affiliations
1Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina
2Department of Pathology, Duke University Medical Center, Durham, North Carolina
JAMA Ophthalmol. 2014;132(11):1373-1375. doi:10.1001/jamaophthalmol.2014.2786.
Text Size: A A A
Published online


Primary angiitis of the central nervous system (PACNS), also known as primary vasculitis of the central nervous system, is a rare, poorly understood, and often fatal disorder.1,2 Although the association of PACNS with optic neuropathy has been documented,35 this is the first report to our knowledge of visual loss with histopathologic involvement of the optic nerves.

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.
Histopathologic Findings in the Brain

A, Section of the right occipital lobe shows well-circumscribed, eosinophilic nodules with demyelination indicated by loss of blue staining (hematoxylin-eosin and Luxol fast blue, scale bar = 500 µm). B, The nodules were fibrotic as highlighted by the green staining using Masson trichrome stain (scale bar = 500 µm). The nodules had a central, cellular core containing a mixture of macrophages and CD3+ T lymphocytes (C) and CD20+ B lymphocytes (D) (scale bars = 10 µm), with T cells predominating. The lymphocytes surrounded and infiltrated the wall of an arteriole.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.
Histopathologic Findings in the Optic Nerves

A, Cross section of the right optic nerve shows demyelination (loss of blue stain) affecting approximately 40% of the nerve (hematoxylin-eosin and Luxol fast blue, scale bar = 1 mm). B, Lymphocytes were most prominent in and around the wall of blood vessels (hematoxylin-eosin, scale bar = 100 µm). C, Higher-magnification image shows lymphocytes infiltrating the walls of blood vessels. The smaller vessel at the top left is fibrotic (hematoxylin-eosin, scale bar = 10 µm). D, Masson trichrome stain demonstrated fibrosis (dark green staining) of the inflamed blood vessels in the nerve (scale bar = 100 µm).

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.
Submit a Comment


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