Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody

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 vasculiti...

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Main Authors: Amer M. Awad, Mathew Stevenson
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2011/495201
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author Amer M. Awad
Mathew Stevenson
author_facet Amer M. Awad
Mathew Stevenson
author_sort Amer M. Awad
collection DOAJ
description 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.
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spelling doaj-art-24af6157512e4fb09942122153891a8a2025-02-03T06:05:00ZengWileyCase Reports in Neurological Medicine2090-66682090-66762011-01-01201110.1155/2011/495201495201Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein AntibodyAmer M. Awad0Mathew Stevenson1Baton Rouge Neurology Associates, Baton Rouge General Medical Center, Baton Rouge, LA 70806, USADepartment of Neurology, University of Texas Southwestern Medical Center, Dallas, TX 75390-7208, USAWe 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.http://dx.doi.org/10.1155/2011/495201
spellingShingle Amer M. Awad
Mathew Stevenson
Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody
Case Reports in Neurological Medicine
title Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody
title_full Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody
title_fullStr Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody
title_full_unstemmed Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody
title_short Isolated Central Nervous System Vasculitis Associated with Antiribonuclear Protein Antibody
title_sort isolated central nervous system vasculitis associated with antiribonuclear protein antibody
url http://dx.doi.org/10.1155/2011/495201
work_keys_str_mv AT amermawad isolatedcentralnervoussystemvasculitisassociatedwithantiribonuclearproteinantibody
AT mathewstevenson isolatedcentralnervoussystemvasculitisassociatedwithantiribonuclearproteinantibody