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Central Nervous System Vasculitis

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Neuroimmunology

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Abstract

Central nervous system (CNS) vasculitis may occur in isolation (primary angiitis of the CNS or PACNS) or in the setting of systemic vasculitis, connective tissue disease, infection, or other miscellaneous conditions (secondary CNS vasculitis). PACNS remains a diagnostic challenge for physicians, owing to protean clinical manifestations and lack of specific diagnostic tests for PACNS. Evaluation of suspected PACNS must therefore be focused on excluding causes of secondary CNS vasculitis and non-vasculitic conditions mimicking PACNS. Treatment of PACNS involves long-term immunosuppression. Most show a favorable response to treatment; however, relapses are not uncommon. Historically, PACNS was felt to be universally fatal, but increased awareness and advances in diagnostic testing have seen a decrease in mortality from PACNS in more recent years. Physicians must maintain a high level of clinical suspicion in order to promptly detect and evaluate cases of possible CNS vasculitis, as delays in diagnosis and treatment may result in significant morbidity and mortality.

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Authors and Affiliations

  1. Department of Neurology, University of Iowa Carver College of Medicine, University of Iowa Hospitals and Clinics, Iowa City, IA, USA

    Christine M. Gill & Tracey A. Cho

  2. Department of Neurology, Neuroimmunology, Neuroinfectious disease and Neurohospitalist Sections, Neuro-Infectious Diseases Group, University of Colorado School of Medicine, Aurora, CO, USA

    Amanda L. Piquet

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  1. Christine M. Gill
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  2. Amanda L. Piquet
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  3. Tracey A. Cho
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Correspondence to Tracey A. Cho .

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Editors and Affiliations

  1. University of Colorado Anschutz Medical Campus, Aurora, CO, USA

    Amanda L. Piquet

  2. University of Colorado Anschutz Medical Campus, Aurora, CO, USA

    Enrique Alvarez

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Gill, C.M., Piquet, A.L., Cho, T.A. (2021). Central Nervous System Vasculitis. In: Piquet, A.L., Alvarez, E. (eds) Neuroimmunology. Springer, Cham. https://doi.org/10.1007/978-3-030-61883-4_24

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