29. Primary Central Nervous System Vasculitis

  1. Gary S. Hoffman MD, MS3,
  2. Cornelia M. Weyand MD, PhD4,
  3. Carol A. Langford MD, MHS3 and
  4. Jörg J. Goronzy MD, PhD4
  1. Rula A. Hajj-Ali MD1 and
  2. Leonard H. Calabrese DO2

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch29

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Hajj-Ali, R. A. and Calabrese, L. H. (2012) Primary Central Nervous System Vasculitis, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch29

Editor Information

  1. 3

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 4

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. 1

    Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, USA

  2. 2

    Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, USA

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244

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Keywords:

  • Central nervous system;
  • vasculitis;
  • reversible cerebral vasoconstriction syndrome;
  • stroke;
  • transient ischemic attack;
  • primary angiitis of the central nervous system

Summary

Primary central nervous system vasculitis (PACNS) is a vasculitis confined to the CNS which affects small and medium-sized cerebral blood vessels. Recently, major advancements have been attained in understanding PACNS and differentiating it from its mimics. The diagnosis of PACNS remains a challenging task given the lack of specific diagnostic tests and the diversity of other diseases that masquerade its presentation.