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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.
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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.

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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.

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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).

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