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Case Reports in Neurological Medicine
Volume 2011, Article ID 495201, 4 pages
http://dx.doi.org/10.1155/2011/495201
Case Report

Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody

1Baton Rouge Neurology Associates, Baton Rouge General Medical Center, Baton Rouge, LA 70806, USA
2Department of Neurology, University of Texas Southwestern Medical Center, Dallas, TX 75390-7208, USA

Received 23 May 2011; Accepted 20 June 2011

Academic Editors: A. Atri, T. K. Banerjee, and Y. Narita

Copyright © 2011 Amer M. Awad and Mathew Stevenson. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

We describe the case of a young woman who was referred to a tertiary care center with unexplained subacute progressive encephalopathy preceded by long-standing severe headaches. Her extensive workup was remarkable for abnormal intracranial angiography suggestive of small- and medium-vessel vasculitis, persistently elevated protein in the cerebrospinal fluid and persistently high titers of antiribonuclear protein antibody. The patient showed a modest response to intravenous high-dose steroids. We propose that the patient's neurologic disease is secondary to immune-mediated central nervous system vasculitis, possibly as an initial manifestation of mixed connective tissue disease.